Neurodevelopmental Implications of head positioning

in term and preterm infants Stacey Belliard DPT NTMTC

Disclosures NO affiliations with or involvement in any organization or entity with

any financial interest (such as honoraria educational grants

participation in speakersrsquo bureaus membership employment

consultancies stock ownership or other equity interest and expert

testimony or patent-licensing arrangements) or non-financial interest

(such as personal or professional relationships affiliations

knowledge or beliefs) in the subject matter or materials discussed in

this presentation

Learning Objectives By completion of this session participants will be able to

raquo1) Describe neurodevelopmental implications of poor head positioning in

preterm and term infants

raquo2) Demonstrate neuroprotective head positioning techniques for

premature infants under 32 weeks gestation

raquo3) Identify characteristics of deformational plagiocephaly brachycephaly

and dolichocephaly

raquo4) Demonstrate neuroprotective strategies for preventing and treating

various cranial deformations

Head Positioning and IVH

Positioning and handling to prevent IVH in preterm infants raquo 2003 Vermont Oxford Network focus group developed an evidence based protocol for potentially

best practices to prevent IVH in VLBW infants which includes

raquo Midline head (supine or sidelying) first 72 hours of life

raquo Head of bed elevated to 30 degrees

raquo No lifting of legs

raquo 2017 Systematic review and meta-analysis concluded there is insufficient evidence regarding the

effect of head positioning and tilting on the incidence of GMH-IVH and cerebral hemodynamics and

oxygenation in preterm infants Neither recommend nor refute the use of a neutral head position

andor head tilting in order to prevent GMH-IVH

McLendon et al 2003 de Bijl-

Marcus 2017

CRANIAL DEFORMATIONS

Brain Growth and Development raquoThe rapid growth in neuronal cell number during the 10th through

18th weeks of gestation achieves near adult cell numbers

raquo this is followed by dramatic increases in dendritic growth and

arborization then myelinization

raquoAt 15 months of age the brain is roughly 65 adult size while the

cerebellum has achieved adult proportion

raquoThe majority of myelinization is complete by 2 years of age

Bronfin 2001

Brain Growth raquo A newborns head circumference is larger than the chest circumference at

birth

raquo Average occipital-frontal circumference (OFC) is 35 cm in the term

newborn 45 cm at 1 year and 55 cm in an adult

raquo The OFC increases by 2 cm per month for the first 3 months of life 1 cm

per month for the second 3 months of life and 05 cm per month from 6ndash12

months

raquo The volume of the cranial vault is 65 of adult size at birth and 95 of the

adult size at age 10 years In contrast facial size is 40 of adult size at

birth and 65 at 10 years

Bronfin 2001

Anatomy of the Skull

Sutures raquo ―syndesmotic joints

raquo permit growth

raquo Sagittal

raquo Coronal

raquo Lambdoidal

raquo Metopic

Bronfin 2001

Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that

interferes with cell migration and differentiation through genetically

programmed biochemical processes or through extrinsic chemical

interference (teratogens) In essence this process represents an error in

the normal development of a part

raquo Deformation an alteration of a body part that is developing normally until

a mechanical force is applied

Bronfin 2001

Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures

Cranial Deformations raquo Deformational Brachycephaly

raquo Deformational Plagiocephaly

raquo Deformational Dolichocephaly

raquo True Scaphocephaly (Not deformational)

Differentiation between synostotic and non-synostotic head shapes

Deformational Brachycephaly

bull Occipital flattening

bull Increased CI (wide head)

bull Often have some

asymmetryplagiocephaly

bull May have turricephaly

(increased cranial height

limited to posterior cranium)

Synostotic Brachycephaly

bull Bilateral coronal sutures

fused

bull Very rare

bull Congenital not developed

bull Severe forehead retrusion

bull Anterior turricephaly

Differentiation between synostotic and non-synostotic head shapes

Deformational

(Scapho)Dolichocephaly

bull Long slender head ―toaster

bull Usually result of extreme head

rotation to one side

bull Premature infants positioned side-

to-side in NICU

bull Normal increase in width from

anterior to posterior

Synostotic Scaphocephaly

(Sagittal Synostosis)

bull ―Boat shaped head

bull Frontal bossing

bull Bilateral occipitalparietal narrowing

posterior to anterior fontanelle

bull Decreased vertical height posterior

cranium

Differentiation between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

bull Frontal bossing ipsilateral

bull Often associated with head

preference or torticollis

bull Anterior shift of ipsilateral forehead

ear and cheek

bull Eye may appear more open where

forehead shows increased bossing

Unilateral Coronal Synostosis

bull Flattening of forehead ipsilateral

bull Nasal root and midfacial angulation

bull Anteror displacement of ipsilateral

ear

bull Eye appears more open where

forehead is flattened contralateral

eyelid ptosis

Differentiaion between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

(Positional Plagiocephaly)

bull Unilateral occipital flattening

bull Ipsilateral frontal bossing

bull Contralateral occipital bossing

bull Parallelogram (sort of)

bull Ipsilateral ear shift anterior DP LS

Lambdoidal Synostosis

(Synostotic Posterior Plagiocephaly)

bull Unilateral occipital flattening

bull Contralateral parietal bossing

bull Minimal contralateral frontal bossing

bull Trapezoid windswept

bull Ipsilateral ear shift posterior

DP vs LS

Assessment raquo No standards

raquo Visual Assessment

raquo Anthropometric Caliper measurement

raquo Flexicurve

raquo Plagiocephalometry

raquo 3D Photography

raquo Radiological imaging

raquo In the near future app under development in Germany

Measurements From To

Length Glabella Opisthocranium

Width Eurion (1 cm above the

otobasiun superious)

Eurion (1 cm above the

otobasiun superious)

Oblique Frontotemporal point (lateral

point of the ipsilateral eyebrow)

Lamboidal point

Circumference Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Disclosures NO affiliations with or involvement in any organization or entity with

any financial interest (such as honoraria educational grants

participation in speakersrsquo bureaus membership employment

consultancies stock ownership or other equity interest and expert

testimony or patent-licensing arrangements) or non-financial interest

(such as personal or professional relationships affiliations

knowledge or beliefs) in the subject matter or materials discussed in

this presentation

Learning Objectives By completion of this session participants will be able to

raquo1) Describe neurodevelopmental implications of poor head positioning in

preterm and term infants

raquo2) Demonstrate neuroprotective head positioning techniques for

premature infants under 32 weeks gestation

raquo3) Identify characteristics of deformational plagiocephaly brachycephaly

and dolichocephaly

raquo4) Demonstrate neuroprotective strategies for preventing and treating

various cranial deformations

Head Positioning and IVH

Positioning and handling to prevent IVH in preterm infants raquo 2003 Vermont Oxford Network focus group developed an evidence based protocol for potentially

best practices to prevent IVH in VLBW infants which includes

raquo Midline head (supine or sidelying) first 72 hours of life

raquo Head of bed elevated to 30 degrees

raquo No lifting of legs

raquo 2017 Systematic review and meta-analysis concluded there is insufficient evidence regarding the

effect of head positioning and tilting on the incidence of GMH-IVH and cerebral hemodynamics and

oxygenation in preterm infants Neither recommend nor refute the use of a neutral head position

andor head tilting in order to prevent GMH-IVH

McLendon et al 2003 de Bijl-

Marcus 2017

CRANIAL DEFORMATIONS

Brain Growth and Development raquoThe rapid growth in neuronal cell number during the 10th through

18th weeks of gestation achieves near adult cell numbers

raquo this is followed by dramatic increases in dendritic growth and

arborization then myelinization

raquoAt 15 months of age the brain is roughly 65 adult size while the

cerebellum has achieved adult proportion

raquoThe majority of myelinization is complete by 2 years of age

Bronfin 2001

Brain Growth raquo A newborns head circumference is larger than the chest circumference at

birth

raquo Average occipital-frontal circumference (OFC) is 35 cm in the term

newborn 45 cm at 1 year and 55 cm in an adult

raquo The OFC increases by 2 cm per month for the first 3 months of life 1 cm

per month for the second 3 months of life and 05 cm per month from 6ndash12

months

raquo The volume of the cranial vault is 65 of adult size at birth and 95 of the

adult size at age 10 years In contrast facial size is 40 of adult size at

birth and 65 at 10 years

Bronfin 2001

Anatomy of the Skull

Sutures raquo ―syndesmotic joints

raquo permit growth

raquo Sagittal

raquo Coronal

raquo Lambdoidal

raquo Metopic

Bronfin 2001

Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that

interferes with cell migration and differentiation through genetically

programmed biochemical processes or through extrinsic chemical

interference (teratogens) In essence this process represents an error in

the normal development of a part

raquo Deformation an alteration of a body part that is developing normally until

a mechanical force is applied

Bronfin 2001

Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures

Cranial Deformations raquo Deformational Brachycephaly

raquo Deformational Plagiocephaly

raquo Deformational Dolichocephaly

raquo True Scaphocephaly (Not deformational)

Differentiation between synostotic and non-synostotic head shapes

Deformational Brachycephaly

bull Occipital flattening

bull Increased CI (wide head)

bull Often have some

asymmetryplagiocephaly

bull May have turricephaly

(increased cranial height

limited to posterior cranium)

Synostotic Brachycephaly

bull Bilateral coronal sutures

fused

bull Very rare

bull Congenital not developed

bull Severe forehead retrusion

bull Anterior turricephaly

Differentiation between synostotic and non-synostotic head shapes

Deformational

(Scapho)Dolichocephaly

bull Long slender head ―toaster

bull Usually result of extreme head

rotation to one side

bull Premature infants positioned side-

to-side in NICU

bull Normal increase in width from

anterior to posterior

Synostotic Scaphocephaly

(Sagittal Synostosis)

bull ―Boat shaped head

bull Frontal bossing

bull Bilateral occipitalparietal narrowing

posterior to anterior fontanelle

bull Decreased vertical height posterior

cranium

Differentiation between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

bull Frontal bossing ipsilateral

bull Often associated with head

preference or torticollis

bull Anterior shift of ipsilateral forehead

ear and cheek

bull Eye may appear more open where

forehead shows increased bossing

Unilateral Coronal Synostosis

bull Flattening of forehead ipsilateral

bull Nasal root and midfacial angulation

bull Anteror displacement of ipsilateral

ear

bull Eye appears more open where

forehead is flattened contralateral

eyelid ptosis

Differentiaion between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

(Positional Plagiocephaly)

bull Unilateral occipital flattening

bull Ipsilateral frontal bossing

bull Contralateral occipital bossing

bull Parallelogram (sort of)

bull Ipsilateral ear shift anterior DP LS

Lambdoidal Synostosis

(Synostotic Posterior Plagiocephaly)

bull Unilateral occipital flattening

bull Contralateral parietal bossing

bull Minimal contralateral frontal bossing

bull Trapezoid windswept

bull Ipsilateral ear shift posterior

DP vs LS

Assessment raquo No standards

raquo Visual Assessment

raquo Anthropometric Caliper measurement

raquo Flexicurve

raquo Plagiocephalometry

raquo 3D Photography

raquo Radiological imaging

raquo In the near future app under development in Germany

Measurements From To

Length Glabella Opisthocranium

Width Eurion (1 cm above the

otobasiun superious)

Eurion (1 cm above the

otobasiun superious)

Oblique Frontotemporal point (lateral

point of the ipsilateral eyebrow)

Lamboidal point

Circumference Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Learning Objectives By completion of this session participants will be able to

raquo1) Describe neurodevelopmental implications of poor head positioning in

preterm and term infants

raquo2) Demonstrate neuroprotective head positioning techniques for

premature infants under 32 weeks gestation

raquo3) Identify characteristics of deformational plagiocephaly brachycephaly

and dolichocephaly

raquo4) Demonstrate neuroprotective strategies for preventing and treating

various cranial deformations

Head Positioning and IVH

Positioning and handling to prevent IVH in preterm infants raquo 2003 Vermont Oxford Network focus group developed an evidence based protocol for potentially

best practices to prevent IVH in VLBW infants which includes

raquo Midline head (supine or sidelying) first 72 hours of life

raquo Head of bed elevated to 30 degrees

raquo No lifting of legs

raquo 2017 Systematic review and meta-analysis concluded there is insufficient evidence regarding the

effect of head positioning and tilting on the incidence of GMH-IVH and cerebral hemodynamics and

oxygenation in preterm infants Neither recommend nor refute the use of a neutral head position

andor head tilting in order to prevent GMH-IVH

McLendon et al 2003 de Bijl-

Marcus 2017

CRANIAL DEFORMATIONS

Brain Growth and Development raquoThe rapid growth in neuronal cell number during the 10th through

18th weeks of gestation achieves near adult cell numbers

raquo this is followed by dramatic increases in dendritic growth and

arborization then myelinization

raquoAt 15 months of age the brain is roughly 65 adult size while the

cerebellum has achieved adult proportion

raquoThe majority of myelinization is complete by 2 years of age

Bronfin 2001

Brain Growth raquo A newborns head circumference is larger than the chest circumference at

birth

raquo Average occipital-frontal circumference (OFC) is 35 cm in the term

newborn 45 cm at 1 year and 55 cm in an adult

raquo The OFC increases by 2 cm per month for the first 3 months of life 1 cm

per month for the second 3 months of life and 05 cm per month from 6ndash12

months

raquo The volume of the cranial vault is 65 of adult size at birth and 95 of the

adult size at age 10 years In contrast facial size is 40 of adult size at

birth and 65 at 10 years

Bronfin 2001

Anatomy of the Skull

Sutures raquo ―syndesmotic joints

raquo permit growth

raquo Sagittal

raquo Coronal

raquo Lambdoidal

raquo Metopic

Bronfin 2001

Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that

interferes with cell migration and differentiation through genetically

programmed biochemical processes or through extrinsic chemical

interference (teratogens) In essence this process represents an error in

the normal development of a part

raquo Deformation an alteration of a body part that is developing normally until

a mechanical force is applied

Bronfin 2001

Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures

Cranial Deformations raquo Deformational Brachycephaly

raquo Deformational Plagiocephaly

raquo Deformational Dolichocephaly

raquo True Scaphocephaly (Not deformational)

Differentiation between synostotic and non-synostotic head shapes

Deformational Brachycephaly

bull Occipital flattening

bull Increased CI (wide head)

bull Often have some

asymmetryplagiocephaly

bull May have turricephaly

(increased cranial height

limited to posterior cranium)

Synostotic Brachycephaly

bull Bilateral coronal sutures

fused

bull Very rare

bull Congenital not developed

bull Severe forehead retrusion

bull Anterior turricephaly

Differentiation between synostotic and non-synostotic head shapes

Deformational

(Scapho)Dolichocephaly

bull Long slender head ―toaster

bull Usually result of extreme head

rotation to one side

bull Premature infants positioned side-

to-side in NICU

bull Normal increase in width from

anterior to posterior

Synostotic Scaphocephaly

(Sagittal Synostosis)

bull ―Boat shaped head

bull Frontal bossing

bull Bilateral occipitalparietal narrowing

posterior to anterior fontanelle

bull Decreased vertical height posterior

cranium

Differentiation between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

bull Frontal bossing ipsilateral

bull Often associated with head

preference or torticollis

bull Anterior shift of ipsilateral forehead

ear and cheek

bull Eye may appear more open where

forehead shows increased bossing

Unilateral Coronal Synostosis

bull Flattening of forehead ipsilateral

bull Nasal root and midfacial angulation

bull Anteror displacement of ipsilateral

ear

bull Eye appears more open where

forehead is flattened contralateral

eyelid ptosis

Differentiaion between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

(Positional Plagiocephaly)

bull Unilateral occipital flattening

bull Ipsilateral frontal bossing

bull Contralateral occipital bossing

bull Parallelogram (sort of)

bull Ipsilateral ear shift anterior DP LS

Lambdoidal Synostosis

(Synostotic Posterior Plagiocephaly)

bull Unilateral occipital flattening

bull Contralateral parietal bossing

bull Minimal contralateral frontal bossing

bull Trapezoid windswept

bull Ipsilateral ear shift posterior

DP vs LS

Assessment raquo No standards

raquo Visual Assessment

raquo Anthropometric Caliper measurement

raquo Flexicurve

raquo Plagiocephalometry

raquo 3D Photography

raquo Radiological imaging

raquo In the near future app under development in Germany

Measurements From To

Length Glabella Opisthocranium

Width Eurion (1 cm above the

otobasiun superious)

Eurion (1 cm above the

otobasiun superious)

Oblique Frontotemporal point (lateral

point of the ipsilateral eyebrow)

Lamboidal point

Circumference Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Head Positioning and IVH

Positioning and handling to prevent IVH in preterm infants raquo 2003 Vermont Oxford Network focus group developed an evidence based protocol for potentially

best practices to prevent IVH in VLBW infants which includes

raquo Midline head (supine or sidelying) first 72 hours of life

raquo Head of bed elevated to 30 degrees

raquo No lifting of legs

raquo 2017 Systematic review and meta-analysis concluded there is insufficient evidence regarding the

effect of head positioning and tilting on the incidence of GMH-IVH and cerebral hemodynamics and

oxygenation in preterm infants Neither recommend nor refute the use of a neutral head position

andor head tilting in order to prevent GMH-IVH

McLendon et al 2003 de Bijl-

Marcus 2017

CRANIAL DEFORMATIONS

Brain Growth and Development raquoThe rapid growth in neuronal cell number during the 10th through

18th weeks of gestation achieves near adult cell numbers

raquo this is followed by dramatic increases in dendritic growth and

arborization then myelinization

raquoAt 15 months of age the brain is roughly 65 adult size while the

cerebellum has achieved adult proportion

raquoThe majority of myelinization is complete by 2 years of age

Bronfin 2001

Brain Growth raquo A newborns head circumference is larger than the chest circumference at

birth

raquo Average occipital-frontal circumference (OFC) is 35 cm in the term

newborn 45 cm at 1 year and 55 cm in an adult

raquo The OFC increases by 2 cm per month for the first 3 months of life 1 cm

per month for the second 3 months of life and 05 cm per month from 6ndash12

months

raquo The volume of the cranial vault is 65 of adult size at birth and 95 of the

adult size at age 10 years In contrast facial size is 40 of adult size at

birth and 65 at 10 years

Bronfin 2001

Anatomy of the Skull

Sutures raquo ―syndesmotic joints

raquo permit growth

raquo Sagittal

raquo Coronal

raquo Lambdoidal

raquo Metopic

Bronfin 2001

Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that

interferes with cell migration and differentiation through genetically

programmed biochemical processes or through extrinsic chemical

interference (teratogens) In essence this process represents an error in

the normal development of a part

raquo Deformation an alteration of a body part that is developing normally until

a mechanical force is applied

Bronfin 2001

Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures

Cranial Deformations raquo Deformational Brachycephaly

raquo Deformational Plagiocephaly

raquo Deformational Dolichocephaly

raquo True Scaphocephaly (Not deformational)

Differentiation between synostotic and non-synostotic head shapes

Deformational Brachycephaly

bull Occipital flattening

bull Increased CI (wide head)

bull Often have some

asymmetryplagiocephaly

bull May have turricephaly

(increased cranial height

limited to posterior cranium)

Synostotic Brachycephaly

bull Bilateral coronal sutures

fused

bull Very rare

bull Congenital not developed

bull Severe forehead retrusion

bull Anterior turricephaly

Differentiation between synostotic and non-synostotic head shapes

Deformational

(Scapho)Dolichocephaly

bull Long slender head ―toaster

bull Usually result of extreme head

rotation to one side

bull Premature infants positioned side-

to-side in NICU

bull Normal increase in width from

anterior to posterior

Synostotic Scaphocephaly

(Sagittal Synostosis)

bull ―Boat shaped head

bull Frontal bossing

bull Bilateral occipitalparietal narrowing

posterior to anterior fontanelle

bull Decreased vertical height posterior

cranium

Differentiation between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

bull Frontal bossing ipsilateral

bull Often associated with head

preference or torticollis

bull Anterior shift of ipsilateral forehead

ear and cheek

bull Eye may appear more open where

forehead shows increased bossing

Unilateral Coronal Synostosis

bull Flattening of forehead ipsilateral

bull Nasal root and midfacial angulation

bull Anteror displacement of ipsilateral

ear

bull Eye appears more open where

forehead is flattened contralateral

eyelid ptosis

Differentiaion between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

(Positional Plagiocephaly)

bull Unilateral occipital flattening

bull Ipsilateral frontal bossing

bull Contralateral occipital bossing

bull Parallelogram (sort of)

bull Ipsilateral ear shift anterior DP LS

Lambdoidal Synostosis

(Synostotic Posterior Plagiocephaly)

bull Unilateral occipital flattening

bull Contralateral parietal bossing

bull Minimal contralateral frontal bossing

bull Trapezoid windswept

bull Ipsilateral ear shift posterior

DP vs LS

Assessment raquo No standards

raquo Visual Assessment

raquo Anthropometric Caliper measurement

raquo Flexicurve

raquo Plagiocephalometry

raquo 3D Photography

raquo Radiological imaging

raquo In the near future app under development in Germany

Measurements From To

Length Glabella Opisthocranium

Width Eurion (1 cm above the

otobasiun superious)

Eurion (1 cm above the

otobasiun superious)

Oblique Frontotemporal point (lateral

point of the ipsilateral eyebrow)

Lamboidal point

Circumference Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Positioning and handling to prevent IVH in preterm infants raquo 2003 Vermont Oxford Network focus group developed an evidence based protocol for potentially

best practices to prevent IVH in VLBW infants which includes

raquo Midline head (supine or sidelying) first 72 hours of life

raquo Head of bed elevated to 30 degrees

raquo No lifting of legs

raquo 2017 Systematic review and meta-analysis concluded there is insufficient evidence regarding the

effect of head positioning and tilting on the incidence of GMH-IVH and cerebral hemodynamics and

oxygenation in preterm infants Neither recommend nor refute the use of a neutral head position

andor head tilting in order to prevent GMH-IVH

McLendon et al 2003 de Bijl-

Marcus 2017

CRANIAL DEFORMATIONS

Brain Growth and Development raquoThe rapid growth in neuronal cell number during the 10th through

18th weeks of gestation achieves near adult cell numbers

raquo this is followed by dramatic increases in dendritic growth and

arborization then myelinization

raquoAt 15 months of age the brain is roughly 65 adult size while the

cerebellum has achieved adult proportion

raquoThe majority of myelinization is complete by 2 years of age

Bronfin 2001

Brain Growth raquo A newborns head circumference is larger than the chest circumference at

birth

raquo Average occipital-frontal circumference (OFC) is 35 cm in the term

newborn 45 cm at 1 year and 55 cm in an adult

raquo The OFC increases by 2 cm per month for the first 3 months of life 1 cm

per month for the second 3 months of life and 05 cm per month from 6ndash12

months

raquo The volume of the cranial vault is 65 of adult size at birth and 95 of the

adult size at age 10 years In contrast facial size is 40 of adult size at

birth and 65 at 10 years

Bronfin 2001

Anatomy of the Skull

Sutures raquo ―syndesmotic joints

raquo permit growth

raquo Sagittal

raquo Coronal

raquo Lambdoidal

raquo Metopic

Bronfin 2001

Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that

interferes with cell migration and differentiation through genetically

programmed biochemical processes or through extrinsic chemical

interference (teratogens) In essence this process represents an error in

the normal development of a part

raquo Deformation an alteration of a body part that is developing normally until

a mechanical force is applied

Bronfin 2001

Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures

Cranial Deformations raquo Deformational Brachycephaly

raquo Deformational Plagiocephaly

raquo Deformational Dolichocephaly

raquo True Scaphocephaly (Not deformational)

Differentiation between synostotic and non-synostotic head shapes

Deformational Brachycephaly

bull Occipital flattening

bull Increased CI (wide head)

bull Often have some

asymmetryplagiocephaly

bull May have turricephaly

(increased cranial height

limited to posterior cranium)

Synostotic Brachycephaly

bull Bilateral coronal sutures

fused

bull Very rare

bull Congenital not developed

bull Severe forehead retrusion

bull Anterior turricephaly

Differentiation between synostotic and non-synostotic head shapes

Deformational

(Scapho)Dolichocephaly

bull Long slender head ―toaster

bull Usually result of extreme head

rotation to one side

bull Premature infants positioned side-

to-side in NICU

bull Normal increase in width from

anterior to posterior

Synostotic Scaphocephaly

(Sagittal Synostosis)

bull ―Boat shaped head

bull Frontal bossing

bull Bilateral occipitalparietal narrowing

posterior to anterior fontanelle

bull Decreased vertical height posterior

cranium

Differentiation between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

bull Frontal bossing ipsilateral

bull Often associated with head

preference or torticollis

bull Anterior shift of ipsilateral forehead

ear and cheek

bull Eye may appear more open where

forehead shows increased bossing

Unilateral Coronal Synostosis

bull Flattening of forehead ipsilateral

bull Nasal root and midfacial angulation

bull Anteror displacement of ipsilateral

ear

bull Eye appears more open where

forehead is flattened contralateral

eyelid ptosis

Differentiaion between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

(Positional Plagiocephaly)

bull Unilateral occipital flattening

bull Ipsilateral frontal bossing

bull Contralateral occipital bossing

bull Parallelogram (sort of)

bull Ipsilateral ear shift anterior DP LS

Lambdoidal Synostosis

(Synostotic Posterior Plagiocephaly)

bull Unilateral occipital flattening

bull Contralateral parietal bossing

bull Minimal contralateral frontal bossing

bull Trapezoid windswept

bull Ipsilateral ear shift posterior

DP vs LS

Assessment raquo No standards

raquo Visual Assessment

raquo Anthropometric Caliper measurement

raquo Flexicurve

raquo Plagiocephalometry

raquo 3D Photography

raquo Radiological imaging

raquo In the near future app under development in Germany

Measurements From To

Length Glabella Opisthocranium

Width Eurion (1 cm above the

otobasiun superious)

Eurion (1 cm above the

otobasiun superious)

Oblique Frontotemporal point (lateral

point of the ipsilateral eyebrow)

Lamboidal point

Circumference Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

CRANIAL DEFORMATIONS

Brain Growth and Development raquoThe rapid growth in neuronal cell number during the 10th through

18th weeks of gestation achieves near adult cell numbers

raquo this is followed by dramatic increases in dendritic growth and

arborization then myelinization

raquoAt 15 months of age the brain is roughly 65 adult size while the

cerebellum has achieved adult proportion

raquoThe majority of myelinization is complete by 2 years of age

Bronfin 2001

Brain Growth raquo A newborns head circumference is larger than the chest circumference at

birth

raquo Average occipital-frontal circumference (OFC) is 35 cm in the term

newborn 45 cm at 1 year and 55 cm in an adult

raquo The OFC increases by 2 cm per month for the first 3 months of life 1 cm

per month for the second 3 months of life and 05 cm per month from 6ndash12

months

raquo The volume of the cranial vault is 65 of adult size at birth and 95 of the

adult size at age 10 years In contrast facial size is 40 of adult size at

birth and 65 at 10 years

Bronfin 2001

Anatomy of the Skull

Sutures raquo ―syndesmotic joints

raquo permit growth

raquo Sagittal

raquo Coronal

raquo Lambdoidal

raquo Metopic

Bronfin 2001

Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that

interferes with cell migration and differentiation through genetically

programmed biochemical processes or through extrinsic chemical

interference (teratogens) In essence this process represents an error in

the normal development of a part

raquo Deformation an alteration of a body part that is developing normally until

a mechanical force is applied

Bronfin 2001

Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures

Cranial Deformations raquo Deformational Brachycephaly

raquo Deformational Plagiocephaly

raquo Deformational Dolichocephaly

raquo True Scaphocephaly (Not deformational)

Differentiation between synostotic and non-synostotic head shapes

Deformational Brachycephaly

bull Occipital flattening

bull Increased CI (wide head)

bull Often have some

asymmetryplagiocephaly

bull May have turricephaly

(increased cranial height

limited to posterior cranium)

Synostotic Brachycephaly

bull Bilateral coronal sutures

fused

bull Very rare

bull Congenital not developed

bull Severe forehead retrusion

bull Anterior turricephaly

Differentiation between synostotic and non-synostotic head shapes

Deformational

(Scapho)Dolichocephaly

bull Long slender head ―toaster

bull Usually result of extreme head

rotation to one side

bull Premature infants positioned side-

to-side in NICU

bull Normal increase in width from

anterior to posterior

Synostotic Scaphocephaly

(Sagittal Synostosis)

bull ―Boat shaped head

bull Frontal bossing

bull Bilateral occipitalparietal narrowing

posterior to anterior fontanelle

bull Decreased vertical height posterior

cranium

Differentiation between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

bull Frontal bossing ipsilateral

bull Often associated with head

preference or torticollis

bull Anterior shift of ipsilateral forehead

ear and cheek

bull Eye may appear more open where

forehead shows increased bossing

Unilateral Coronal Synostosis

bull Flattening of forehead ipsilateral

bull Nasal root and midfacial angulation

bull Anteror displacement of ipsilateral

ear

bull Eye appears more open where

forehead is flattened contralateral

eyelid ptosis

Differentiaion between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

(Positional Plagiocephaly)

bull Unilateral occipital flattening

bull Ipsilateral frontal bossing

bull Contralateral occipital bossing

bull Parallelogram (sort of)

bull Ipsilateral ear shift anterior DP LS

Lambdoidal Synostosis

(Synostotic Posterior Plagiocephaly)

bull Unilateral occipital flattening

bull Contralateral parietal bossing

bull Minimal contralateral frontal bossing

bull Trapezoid windswept

bull Ipsilateral ear shift posterior

DP vs LS

Assessment raquo No standards

raquo Visual Assessment

raquo Anthropometric Caliper measurement

raquo Flexicurve

raquo Plagiocephalometry

raquo 3D Photography

raquo Radiological imaging

raquo In the near future app under development in Germany

Measurements From To

Length Glabella Opisthocranium

Width Eurion (1 cm above the

otobasiun superious)

Eurion (1 cm above the

otobasiun superious)

Oblique Frontotemporal point (lateral

point of the ipsilateral eyebrow)

Lamboidal point

Circumference Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Brain Growth and Development raquoThe rapid growth in neuronal cell number during the 10th through

18th weeks of gestation achieves near adult cell numbers

raquo this is followed by dramatic increases in dendritic growth and

arborization then myelinization

raquoAt 15 months of age the brain is roughly 65 adult size while the

cerebellum has achieved adult proportion

raquoThe majority of myelinization is complete by 2 years of age

Bronfin 2001

Brain Growth raquo A newborns head circumference is larger than the chest circumference at

birth

raquo Average occipital-frontal circumference (OFC) is 35 cm in the term

newborn 45 cm at 1 year and 55 cm in an adult

raquo The OFC increases by 2 cm per month for the first 3 months of life 1 cm

per month for the second 3 months of life and 05 cm per month from 6ndash12

months

raquo The volume of the cranial vault is 65 of adult size at birth and 95 of the

adult size at age 10 years In contrast facial size is 40 of adult size at

birth and 65 at 10 years

Bronfin 2001

Anatomy of the Skull

Sutures raquo ―syndesmotic joints

raquo permit growth

raquo Sagittal

raquo Coronal

raquo Lambdoidal

raquo Metopic

Bronfin 2001

Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that

interferes with cell migration and differentiation through genetically

programmed biochemical processes or through extrinsic chemical

interference (teratogens) In essence this process represents an error in

the normal development of a part

raquo Deformation an alteration of a body part that is developing normally until

a mechanical force is applied

Bronfin 2001

Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures

Cranial Deformations raquo Deformational Brachycephaly

raquo Deformational Plagiocephaly

raquo Deformational Dolichocephaly

raquo True Scaphocephaly (Not deformational)

Differentiation between synostotic and non-synostotic head shapes

Deformational Brachycephaly

bull Occipital flattening

bull Increased CI (wide head)

bull Often have some

asymmetryplagiocephaly

bull May have turricephaly

(increased cranial height

limited to posterior cranium)

Synostotic Brachycephaly

bull Bilateral coronal sutures

fused

bull Very rare

bull Congenital not developed

bull Severe forehead retrusion

bull Anterior turricephaly

Differentiation between synostotic and non-synostotic head shapes

Deformational

(Scapho)Dolichocephaly

bull Long slender head ―toaster

bull Usually result of extreme head

rotation to one side

bull Premature infants positioned side-

to-side in NICU

bull Normal increase in width from

anterior to posterior

Synostotic Scaphocephaly

(Sagittal Synostosis)

bull ―Boat shaped head

bull Frontal bossing

bull Bilateral occipitalparietal narrowing

posterior to anterior fontanelle

bull Decreased vertical height posterior

cranium

Differentiation between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

bull Frontal bossing ipsilateral

bull Often associated with head

preference or torticollis

bull Anterior shift of ipsilateral forehead

ear and cheek

bull Eye may appear more open where

forehead shows increased bossing

Unilateral Coronal Synostosis

bull Flattening of forehead ipsilateral

bull Nasal root and midfacial angulation

bull Anteror displacement of ipsilateral

ear

bull Eye appears more open where

forehead is flattened contralateral

eyelid ptosis

Differentiaion between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

(Positional Plagiocephaly)

bull Unilateral occipital flattening

bull Ipsilateral frontal bossing

bull Contralateral occipital bossing

bull Parallelogram (sort of)

bull Ipsilateral ear shift anterior DP LS

Lambdoidal Synostosis

(Synostotic Posterior Plagiocephaly)

bull Unilateral occipital flattening

bull Contralateral parietal bossing

bull Minimal contralateral frontal bossing

bull Trapezoid windswept

bull Ipsilateral ear shift posterior

DP vs LS

Assessment raquo No standards

raquo Visual Assessment

raquo Anthropometric Caliper measurement

raquo Flexicurve

raquo Plagiocephalometry

raquo 3D Photography

raquo Radiological imaging

raquo In the near future app under development in Germany

Measurements From To

Length Glabella Opisthocranium

Width Eurion (1 cm above the

otobasiun superious)

Eurion (1 cm above the

otobasiun superious)

Oblique Frontotemporal point (lateral

point of the ipsilateral eyebrow)

Lamboidal point

Circumference Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Brain Growth raquo A newborns head circumference is larger than the chest circumference at

birth

raquo Average occipital-frontal circumference (OFC) is 35 cm in the term

newborn 45 cm at 1 year and 55 cm in an adult

raquo The OFC increases by 2 cm per month for the first 3 months of life 1 cm

per month for the second 3 months of life and 05 cm per month from 6ndash12

months

raquo The volume of the cranial vault is 65 of adult size at birth and 95 of the

adult size at age 10 years In contrast facial size is 40 of adult size at

birth and 65 at 10 years

Bronfin 2001

Anatomy of the Skull

Sutures raquo ―syndesmotic joints

raquo permit growth

raquo Sagittal

raquo Coronal

raquo Lambdoidal

raquo Metopic

Bronfin 2001

Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that

interferes with cell migration and differentiation through genetically

programmed biochemical processes or through extrinsic chemical

interference (teratogens) In essence this process represents an error in

the normal development of a part

raquo Deformation an alteration of a body part that is developing normally until

a mechanical force is applied

Bronfin 2001

Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures

Cranial Deformations raquo Deformational Brachycephaly

raquo Deformational Plagiocephaly

raquo Deformational Dolichocephaly

raquo True Scaphocephaly (Not deformational)

Differentiation between synostotic and non-synostotic head shapes

Deformational Brachycephaly

bull Occipital flattening

bull Increased CI (wide head)

bull Often have some

asymmetryplagiocephaly

bull May have turricephaly

(increased cranial height

limited to posterior cranium)

Synostotic Brachycephaly

bull Bilateral coronal sutures

fused

bull Very rare

bull Congenital not developed

bull Severe forehead retrusion

bull Anterior turricephaly

Differentiation between synostotic and non-synostotic head shapes

Deformational

(Scapho)Dolichocephaly

bull Long slender head ―toaster

bull Usually result of extreme head

rotation to one side

bull Premature infants positioned side-

to-side in NICU

bull Normal increase in width from

anterior to posterior

Synostotic Scaphocephaly

(Sagittal Synostosis)

bull ―Boat shaped head

bull Frontal bossing

bull Bilateral occipitalparietal narrowing

posterior to anterior fontanelle

bull Decreased vertical height posterior

cranium

Differentiation between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

bull Frontal bossing ipsilateral

bull Often associated with head

preference or torticollis

bull Anterior shift of ipsilateral forehead

ear and cheek

bull Eye may appear more open where

forehead shows increased bossing

Unilateral Coronal Synostosis

bull Flattening of forehead ipsilateral

bull Nasal root and midfacial angulation

bull Anteror displacement of ipsilateral

ear

bull Eye appears more open where

forehead is flattened contralateral

eyelid ptosis

Differentiaion between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

(Positional Plagiocephaly)

bull Unilateral occipital flattening

bull Ipsilateral frontal bossing

bull Contralateral occipital bossing

bull Parallelogram (sort of)

bull Ipsilateral ear shift anterior DP LS

Lambdoidal Synostosis

(Synostotic Posterior Plagiocephaly)

bull Unilateral occipital flattening

bull Contralateral parietal bossing

bull Minimal contralateral frontal bossing

bull Trapezoid windswept

bull Ipsilateral ear shift posterior

DP vs LS

Assessment raquo No standards

raquo Visual Assessment

raquo Anthropometric Caliper measurement

raquo Flexicurve

raquo Plagiocephalometry

raquo 3D Photography

raquo Radiological imaging

raquo In the near future app under development in Germany

Measurements From To

Length Glabella Opisthocranium

Width Eurion (1 cm above the

otobasiun superious)

Eurion (1 cm above the

otobasiun superious)

Oblique Frontotemporal point (lateral

point of the ipsilateral eyebrow)

Lamboidal point

Circumference Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Anatomy of the Skull

Sutures raquo ―syndesmotic joints

raquo permit growth

raquo Sagittal

raquo Coronal

raquo Lambdoidal

raquo Metopic

Bronfin 2001

Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that

interferes with cell migration and differentiation through genetically

programmed biochemical processes or through extrinsic chemical

interference (teratogens) In essence this process represents an error in

the normal development of a part

raquo Deformation an alteration of a body part that is developing normally until

a mechanical force is applied

Bronfin 2001

Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures

Cranial Deformations raquo Deformational Brachycephaly

raquo Deformational Plagiocephaly

raquo Deformational Dolichocephaly

raquo True Scaphocephaly (Not deformational)

Differentiation between synostotic and non-synostotic head shapes

Deformational Brachycephaly

bull Occipital flattening

bull Increased CI (wide head)

bull Often have some

asymmetryplagiocephaly

bull May have turricephaly

(increased cranial height

limited to posterior cranium)

Synostotic Brachycephaly

bull Bilateral coronal sutures

fused

bull Very rare

bull Congenital not developed

bull Severe forehead retrusion

bull Anterior turricephaly

Differentiation between synostotic and non-synostotic head shapes

Deformational

(Scapho)Dolichocephaly

bull Long slender head ―toaster

bull Usually result of extreme head

rotation to one side

bull Premature infants positioned side-

to-side in NICU

bull Normal increase in width from

anterior to posterior

Synostotic Scaphocephaly

(Sagittal Synostosis)

bull ―Boat shaped head

bull Frontal bossing

bull Bilateral occipitalparietal narrowing

posterior to anterior fontanelle

bull Decreased vertical height posterior

cranium

Differentiation between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

bull Frontal bossing ipsilateral

bull Often associated with head

preference or torticollis

bull Anterior shift of ipsilateral forehead

ear and cheek

bull Eye may appear more open where

forehead shows increased bossing

Unilateral Coronal Synostosis

bull Flattening of forehead ipsilateral

bull Nasal root and midfacial angulation

bull Anteror displacement of ipsilateral

ear

bull Eye appears more open where

forehead is flattened contralateral

eyelid ptosis

Differentiaion between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

(Positional Plagiocephaly)

bull Unilateral occipital flattening

bull Ipsilateral frontal bossing

bull Contralateral occipital bossing

bull Parallelogram (sort of)

bull Ipsilateral ear shift anterior DP LS

Lambdoidal Synostosis

(Synostotic Posterior Plagiocephaly)

bull Unilateral occipital flattening

bull Contralateral parietal bossing

bull Minimal contralateral frontal bossing

bull Trapezoid windswept

bull Ipsilateral ear shift posterior

DP vs LS

Assessment raquo No standards

raquo Visual Assessment

raquo Anthropometric Caliper measurement

raquo Flexicurve

raquo Plagiocephalometry

raquo 3D Photography

raquo Radiological imaging

raquo In the near future app under development in Germany

Measurements From To

Length Glabella Opisthocranium

Width Eurion (1 cm above the

otobasiun superious)

Eurion (1 cm above the

otobasiun superious)

Oblique Frontotemporal point (lateral

point of the ipsilateral eyebrow)

Lamboidal point

Circumference Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Sutures raquo ―syndesmotic joints

raquo permit growth

raquo Sagittal

raquo Coronal

raquo Lambdoidal

raquo Metopic

Bronfin 2001

Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that

interferes with cell migration and differentiation through genetically

programmed biochemical processes or through extrinsic chemical

interference (teratogens) In essence this process represents an error in

the normal development of a part

raquo Deformation an alteration of a body part that is developing normally until

a mechanical force is applied

Bronfin 2001

Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures

Cranial Deformations raquo Deformational Brachycephaly

raquo Deformational Plagiocephaly

raquo Deformational Dolichocephaly

raquo True Scaphocephaly (Not deformational)

Differentiation between synostotic and non-synostotic head shapes

Deformational Brachycephaly

bull Occipital flattening

bull Increased CI (wide head)

bull Often have some

asymmetryplagiocephaly

bull May have turricephaly

(increased cranial height

limited to posterior cranium)

Synostotic Brachycephaly

bull Bilateral coronal sutures

fused

bull Very rare

bull Congenital not developed

bull Severe forehead retrusion

bull Anterior turricephaly

Differentiation between synostotic and non-synostotic head shapes

Deformational

(Scapho)Dolichocephaly

bull Long slender head ―toaster

bull Usually result of extreme head

rotation to one side

bull Premature infants positioned side-

to-side in NICU

bull Normal increase in width from

anterior to posterior

Synostotic Scaphocephaly

(Sagittal Synostosis)

bull ―Boat shaped head

bull Frontal bossing

bull Bilateral occipitalparietal narrowing

posterior to anterior fontanelle

bull Decreased vertical height posterior

cranium

Differentiation between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

bull Frontal bossing ipsilateral

bull Often associated with head

preference or torticollis

bull Anterior shift of ipsilateral forehead

ear and cheek

bull Eye may appear more open where

forehead shows increased bossing

Unilateral Coronal Synostosis

bull Flattening of forehead ipsilateral

bull Nasal root and midfacial angulation

bull Anteror displacement of ipsilateral

ear

bull Eye appears more open where

forehead is flattened contralateral

eyelid ptosis

Differentiaion between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

(Positional Plagiocephaly)

bull Unilateral occipital flattening

bull Ipsilateral frontal bossing

bull Contralateral occipital bossing

bull Parallelogram (sort of)

bull Ipsilateral ear shift anterior DP LS

Lambdoidal Synostosis

(Synostotic Posterior Plagiocephaly)

bull Unilateral occipital flattening

bull Contralateral parietal bossing

bull Minimal contralateral frontal bossing

bull Trapezoid windswept

bull Ipsilateral ear shift posterior

DP vs LS

Assessment raquo No standards

raquo Visual Assessment

raquo Anthropometric Caliper measurement

raquo Flexicurve

raquo Plagiocephalometry

raquo 3D Photography

raquo Radiological imaging

raquo In the near future app under development in Germany

Measurements From To

Length Glabella Opisthocranium

Width Eurion (1 cm above the

otobasiun superious)

Eurion (1 cm above the

otobasiun superious)

Oblique Frontotemporal point (lateral

point of the ipsilateral eyebrow)

Lamboidal point

Circumference Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that

interferes with cell migration and differentiation through genetically

programmed biochemical processes or through extrinsic chemical

interference (teratogens) In essence this process represents an error in

the normal development of a part

raquo Deformation an alteration of a body part that is developing normally until

a mechanical force is applied

Bronfin 2001

Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures

Cranial Deformations raquo Deformational Brachycephaly

raquo Deformational Plagiocephaly

raquo Deformational Dolichocephaly

raquo True Scaphocephaly (Not deformational)

Differentiation between synostotic and non-synostotic head shapes

Deformational Brachycephaly

bull Occipital flattening

bull Increased CI (wide head)

bull Often have some

asymmetryplagiocephaly

bull May have turricephaly

(increased cranial height

limited to posterior cranium)

Synostotic Brachycephaly

bull Bilateral coronal sutures

fused

bull Very rare

bull Congenital not developed

bull Severe forehead retrusion

bull Anterior turricephaly

Differentiation between synostotic and non-synostotic head shapes

Deformational

(Scapho)Dolichocephaly

bull Long slender head ―toaster

bull Usually result of extreme head

rotation to one side

bull Premature infants positioned side-

to-side in NICU

bull Normal increase in width from

anterior to posterior

Synostotic Scaphocephaly

(Sagittal Synostosis)

bull ―Boat shaped head

bull Frontal bossing

bull Bilateral occipitalparietal narrowing

posterior to anterior fontanelle

bull Decreased vertical height posterior

cranium

Differentiation between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

bull Frontal bossing ipsilateral

bull Often associated with head

preference or torticollis

bull Anterior shift of ipsilateral forehead

ear and cheek

bull Eye may appear more open where

forehead shows increased bossing

Unilateral Coronal Synostosis

bull Flattening of forehead ipsilateral

bull Nasal root and midfacial angulation

bull Anteror displacement of ipsilateral

ear

bull Eye appears more open where

forehead is flattened contralateral

eyelid ptosis

Differentiaion between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

(Positional Plagiocephaly)

bull Unilateral occipital flattening

bull Ipsilateral frontal bossing

bull Contralateral occipital bossing

bull Parallelogram (sort of)

bull Ipsilateral ear shift anterior DP LS

Lambdoidal Synostosis

(Synostotic Posterior Plagiocephaly)

bull Unilateral occipital flattening

bull Contralateral parietal bossing

bull Minimal contralateral frontal bossing

bull Trapezoid windswept

bull Ipsilateral ear shift posterior

DP vs LS

Assessment raquo No standards

raquo Visual Assessment

raquo Anthropometric Caliper measurement

raquo Flexicurve

raquo Plagiocephalometry

raquo 3D Photography

raquo Radiological imaging

raquo In the near future app under development in Germany

Measurements From To

Length Glabella Opisthocranium

Width Eurion (1 cm above the

otobasiun superious)

Eurion (1 cm above the

otobasiun superious)

Oblique Frontotemporal point (lateral

point of the ipsilateral eyebrow)

Lamboidal point

Circumference Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures

Cranial Deformations raquo Deformational Brachycephaly

raquo Deformational Plagiocephaly

raquo Deformational Dolichocephaly

raquo True Scaphocephaly (Not deformational)

Differentiation between synostotic and non-synostotic head shapes

Deformational Brachycephaly

bull Occipital flattening

bull Increased CI (wide head)

bull Often have some

asymmetryplagiocephaly

bull May have turricephaly

(increased cranial height

limited to posterior cranium)

Synostotic Brachycephaly

bull Bilateral coronal sutures

fused

bull Very rare

bull Congenital not developed

bull Severe forehead retrusion

bull Anterior turricephaly

Differentiation between synostotic and non-synostotic head shapes

Deformational

(Scapho)Dolichocephaly

bull Long slender head ―toaster

bull Usually result of extreme head

rotation to one side

bull Premature infants positioned side-

to-side in NICU

bull Normal increase in width from

anterior to posterior

Synostotic Scaphocephaly

(Sagittal Synostosis)

bull ―Boat shaped head

bull Frontal bossing

bull Bilateral occipitalparietal narrowing

posterior to anterior fontanelle

bull Decreased vertical height posterior

cranium

Differentiation between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

bull Frontal bossing ipsilateral

bull Often associated with head

preference or torticollis

bull Anterior shift of ipsilateral forehead

ear and cheek

bull Eye may appear more open where

forehead shows increased bossing

Unilateral Coronal Synostosis

bull Flattening of forehead ipsilateral

bull Nasal root and midfacial angulation

bull Anteror displacement of ipsilateral

ear

bull Eye appears more open where

forehead is flattened contralateral

eyelid ptosis

Differentiaion between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

(Positional Plagiocephaly)

bull Unilateral occipital flattening

bull Ipsilateral frontal bossing

bull Contralateral occipital bossing

bull Parallelogram (sort of)

bull Ipsilateral ear shift anterior DP LS

Lambdoidal Synostosis

(Synostotic Posterior Plagiocephaly)

bull Unilateral occipital flattening

bull Contralateral parietal bossing

bull Minimal contralateral frontal bossing

bull Trapezoid windswept

bull Ipsilateral ear shift posterior

DP vs LS

Assessment raquo No standards

raquo Visual Assessment

raquo Anthropometric Caliper measurement

raquo Flexicurve

raquo Plagiocephalometry

raquo 3D Photography

raquo Radiological imaging

raquo In the near future app under development in Germany

Measurements From To

Length Glabella Opisthocranium

Width Eurion (1 cm above the

otobasiun superious)

Eurion (1 cm above the

otobasiun superious)

Oblique Frontotemporal point (lateral

point of the ipsilateral eyebrow)

Lamboidal point

Circumference Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Cranial Deformations raquo Deformational Brachycephaly

raquo Deformational Plagiocephaly

raquo Deformational Dolichocephaly

raquo True Scaphocephaly (Not deformational)

Differentiation between synostotic and non-synostotic head shapes

Deformational Brachycephaly

bull Occipital flattening

bull Increased CI (wide head)

bull Often have some

asymmetryplagiocephaly

bull May have turricephaly

(increased cranial height

limited to posterior cranium)

Synostotic Brachycephaly

bull Bilateral coronal sutures

fused

bull Very rare

bull Congenital not developed

bull Severe forehead retrusion

bull Anterior turricephaly

Differentiation between synostotic and non-synostotic head shapes

Deformational

(Scapho)Dolichocephaly

bull Long slender head ―toaster

bull Usually result of extreme head

rotation to one side

bull Premature infants positioned side-

to-side in NICU

bull Normal increase in width from

anterior to posterior

Synostotic Scaphocephaly

(Sagittal Synostosis)

bull ―Boat shaped head

bull Frontal bossing

bull Bilateral occipitalparietal narrowing

posterior to anterior fontanelle

bull Decreased vertical height posterior

cranium

Differentiation between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

bull Frontal bossing ipsilateral

bull Often associated with head

preference or torticollis

bull Anterior shift of ipsilateral forehead

ear and cheek

bull Eye may appear more open where

forehead shows increased bossing

Unilateral Coronal Synostosis

bull Flattening of forehead ipsilateral

bull Nasal root and midfacial angulation

bull Anteror displacement of ipsilateral

ear

bull Eye appears more open where

forehead is flattened contralateral

eyelid ptosis

Differentiaion between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

(Positional Plagiocephaly)

bull Unilateral occipital flattening

bull Ipsilateral frontal bossing

bull Contralateral occipital bossing

bull Parallelogram (sort of)

bull Ipsilateral ear shift anterior DP LS

Lambdoidal Synostosis

(Synostotic Posterior Plagiocephaly)

bull Unilateral occipital flattening

bull Contralateral parietal bossing

bull Minimal contralateral frontal bossing

bull Trapezoid windswept

bull Ipsilateral ear shift posterior

DP vs LS

Assessment raquo No standards

raquo Visual Assessment

raquo Anthropometric Caliper measurement

raquo Flexicurve

raquo Plagiocephalometry

raquo 3D Photography

raquo Radiological imaging

raquo In the near future app under development in Germany

Measurements From To

Length Glabella Opisthocranium

Width Eurion (1 cm above the

otobasiun superious)

Eurion (1 cm above the

otobasiun superious)

Oblique Frontotemporal point (lateral

point of the ipsilateral eyebrow)

Lamboidal point

Circumference Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Differentiation between synostotic and non-synostotic head shapes

Deformational Brachycephaly

bull Occipital flattening

bull Increased CI (wide head)

bull Often have some

asymmetryplagiocephaly

bull May have turricephaly

(increased cranial height

limited to posterior cranium)

Synostotic Brachycephaly

bull Bilateral coronal sutures

fused

bull Very rare

bull Congenital not developed

bull Severe forehead retrusion

bull Anterior turricephaly

Differentiation between synostotic and non-synostotic head shapes

Deformational

(Scapho)Dolichocephaly

bull Long slender head ―toaster

bull Usually result of extreme head

rotation to one side

bull Premature infants positioned side-

to-side in NICU

bull Normal increase in width from

anterior to posterior

Synostotic Scaphocephaly

(Sagittal Synostosis)

bull ―Boat shaped head

bull Frontal bossing

bull Bilateral occipitalparietal narrowing

posterior to anterior fontanelle

bull Decreased vertical height posterior

cranium

Differentiation between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

bull Frontal bossing ipsilateral

bull Often associated with head

preference or torticollis

bull Anterior shift of ipsilateral forehead

ear and cheek

bull Eye may appear more open where

forehead shows increased bossing

Unilateral Coronal Synostosis

bull Flattening of forehead ipsilateral

bull Nasal root and midfacial angulation

bull Anteror displacement of ipsilateral

ear

bull Eye appears more open where

forehead is flattened contralateral

eyelid ptosis

Differentiaion between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

(Positional Plagiocephaly)

bull Unilateral occipital flattening

bull Ipsilateral frontal bossing

bull Contralateral occipital bossing

bull Parallelogram (sort of)

bull Ipsilateral ear shift anterior DP LS

Lambdoidal Synostosis

(Synostotic Posterior Plagiocephaly)

bull Unilateral occipital flattening

bull Contralateral parietal bossing

bull Minimal contralateral frontal bossing

bull Trapezoid windswept

bull Ipsilateral ear shift posterior

DP vs LS

Assessment raquo No standards

raquo Visual Assessment

raquo Anthropometric Caliper measurement

raquo Flexicurve

raquo Plagiocephalometry

raquo 3D Photography

raquo Radiological imaging

raquo In the near future app under development in Germany

Measurements From To

Length Glabella Opisthocranium

Width Eurion (1 cm above the

otobasiun superious)

Eurion (1 cm above the

otobasiun superious)

Oblique Frontotemporal point (lateral

point of the ipsilateral eyebrow)

Lamboidal point

Circumference Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Differentiation between synostotic and non-synostotic head shapes

Deformational

(Scapho)Dolichocephaly

bull Long slender head ―toaster

bull Usually result of extreme head

rotation to one side

bull Premature infants positioned side-

to-side in NICU

bull Normal increase in width from

anterior to posterior

Synostotic Scaphocephaly

(Sagittal Synostosis)

bull ―Boat shaped head

bull Frontal bossing

bull Bilateral occipitalparietal narrowing

posterior to anterior fontanelle

bull Decreased vertical height posterior

cranium

Differentiation between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

bull Frontal bossing ipsilateral

bull Often associated with head

preference or torticollis

bull Anterior shift of ipsilateral forehead

ear and cheek

bull Eye may appear more open where

forehead shows increased bossing

Unilateral Coronal Synostosis

bull Flattening of forehead ipsilateral

bull Nasal root and midfacial angulation

bull Anteror displacement of ipsilateral

ear

bull Eye appears more open where

forehead is flattened contralateral

eyelid ptosis

Differentiaion between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

(Positional Plagiocephaly)

bull Unilateral occipital flattening

bull Ipsilateral frontal bossing

bull Contralateral occipital bossing

bull Parallelogram (sort of)

bull Ipsilateral ear shift anterior DP LS

Lambdoidal Synostosis

(Synostotic Posterior Plagiocephaly)

bull Unilateral occipital flattening

bull Contralateral parietal bossing

bull Minimal contralateral frontal bossing

bull Trapezoid windswept

bull Ipsilateral ear shift posterior

DP vs LS

Assessment raquo No standards

raquo Visual Assessment

raquo Anthropometric Caliper measurement

raquo Flexicurve

raquo Plagiocephalometry

raquo 3D Photography

raquo Radiological imaging

raquo In the near future app under development in Germany

Measurements From To

Length Glabella Opisthocranium

Width Eurion (1 cm above the

otobasiun superious)

Eurion (1 cm above the

otobasiun superious)

Oblique Frontotemporal point (lateral

point of the ipsilateral eyebrow)

Lamboidal point

Circumference Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Differentiation between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

bull Frontal bossing ipsilateral

bull Often associated with head

preference or torticollis

bull Anterior shift of ipsilateral forehead

ear and cheek

bull Eye may appear more open where

forehead shows increased bossing

Unilateral Coronal Synostosis

bull Flattening of forehead ipsilateral

bull Nasal root and midfacial angulation

bull Anteror displacement of ipsilateral

ear

bull Eye appears more open where

forehead is flattened contralateral

eyelid ptosis

Differentiaion between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

(Positional Plagiocephaly)

bull Unilateral occipital flattening

bull Ipsilateral frontal bossing

bull Contralateral occipital bossing

bull Parallelogram (sort of)

bull Ipsilateral ear shift anterior DP LS

Lambdoidal Synostosis

(Synostotic Posterior Plagiocephaly)

bull Unilateral occipital flattening

bull Contralateral parietal bossing

bull Minimal contralateral frontal bossing

bull Trapezoid windswept

bull Ipsilateral ear shift posterior

DP vs LS

Assessment raquo No standards

raquo Visual Assessment

raquo Anthropometric Caliper measurement

raquo Flexicurve

raquo Plagiocephalometry

raquo 3D Photography

raquo Radiological imaging

raquo In the near future app under development in Germany

Measurements From To

Length Glabella Opisthocranium

Width Eurion (1 cm above the

otobasiun superious)

Eurion (1 cm above the

otobasiun superious)

Oblique Frontotemporal point (lateral

point of the ipsilateral eyebrow)

Lamboidal point

Circumference Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Differentiaion between synostotic and non-synostotic head shapes

Deformational Plagiocephaly

(Positional Plagiocephaly)

bull Unilateral occipital flattening

bull Ipsilateral frontal bossing

bull Contralateral occipital bossing

bull Parallelogram (sort of)

bull Ipsilateral ear shift anterior DP LS

Lambdoidal Synostosis

(Synostotic Posterior Plagiocephaly)

bull Unilateral occipital flattening

bull Contralateral parietal bossing

bull Minimal contralateral frontal bossing

bull Trapezoid windswept

bull Ipsilateral ear shift posterior

DP vs LS

Assessment raquo No standards

raquo Visual Assessment

raquo Anthropometric Caliper measurement

raquo Flexicurve

raquo Plagiocephalometry

raquo 3D Photography

raquo Radiological imaging

raquo In the near future app under development in Germany

Measurements From To

Length Glabella Opisthocranium

Width Eurion (1 cm above the

otobasiun superious)

Eurion (1 cm above the

otobasiun superious)

Oblique Frontotemporal point (lateral

point of the ipsilateral eyebrow)

Lamboidal point

Circumference Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Assessment raquo No standards

raquo Visual Assessment

raquo Anthropometric Caliper measurement

raquo Flexicurve

raquo Plagiocephalometry

raquo 3D Photography

raquo Radiological imaging

raquo In the near future app under development in Germany

Measurements From To

Length Glabella Opisthocranium

Width Eurion (1 cm above the

otobasiun superious)

Eurion (1 cm above the

otobasiun superious)

Oblique Frontotemporal point (lateral

point of the ipsilateral eyebrow)

Lamboidal point

Circumference Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Measurements From To

Length Glabella Opisthocranium

Width Eurion (1 cm above the

otobasiun superious)

Eurion (1 cm above the

otobasiun superious)

Oblique Frontotemporal point (lateral

point of the ipsilateral eyebrow)

Lamboidal point

Circumference Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Include Glabella and

Opisthocranium (lower edge of

measurement tape directly

above eyebrow)

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Measurements Cranial Index (Cephalic Index) CI

Represents the ratio of maximum cranial width to maximum cranial length

Commonly used for isolated sagittal synostosis (ISS) but also used for any

scaphocephalydolichocephaly or brachycephaly head shape

Scale for Cephalic Index (CI)

raquo Normal 75 ndash 90 mm

raquo Mild 91 ndash 93 mm

raquo Moderate 94 ndash 97 mm

raquo Severe gt97 mm

raquo Normocephaly or plagiocephaly = CI gt76-lt90

raquo Brachycephaly = CI gt90 CI=CWCL X 100

raquo Dolichocephaly = CI lt76

Ruiz-Correa 2006 Likus 2014

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Deformational Dolichocephaly

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Deformational Brachycephaly

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Measurements Cranial Vault Asymmetry Index (CVAI)

(used to measure plagiocephaly)

Level of Deformity CVAI

1 Normal lt35

2 Mild 35-625

3 Moderate 625-875

4 Severe 875-110

5 Very Severe gt110

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Deformational Plagiocephaly

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Neurodevelopmental Implications of Synostotic Cranial Malformations

bull 50 of children with nonsyndromic sagittal suture craniosynostosis had

reading andor spelling learning disability

bull 37 of children with isolated sagittal synostosis had speech andor

language impairment of whom 71 had moderate or severe impairments

bull Children with nonsyndromic craniosynostosis had significantly different

standardized distribution of BSID-II PDI=0 accelerated 43 normal 48

mild delay and 9 significant delay

bull consistently lower mean neurodevelopmental scores in children with single-

suture craniosynostosis compared with controls These results provide

further support for neurodevelopmental screening in young children with

single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic

disorder as numerous reports of neurodevelopmental outcomes are

being published Although there are many issues to be addressed and

confounding variables to take into account patients and their families

are making more complex demands not only in terms of cosmetic

appearance but also in terms of cognitive results The clinicians point

of view should be geared toward comprehensive management

embracing appearance as well as function

bull ―Overall findings suggest that isolated craniosynostosis is associated

with a 3-5 fold increase in risk for cognitive deficits or

learninglanguage disabilities

Shim 2016 Collett 2005

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media

bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can

result in an increased susceptibility to otitis media

bull A retrospective study using a parent questionnaire comparing 1259 patients with

deformational plagiocephaly to patient data from CDC Questionnaire showed a

trend directly correlating otitis media and severity level of DP (not statistically

significant)

bull In addition a subset of infants with DP were administered a tympanogram to assess

state of middle ear Tympanogrametry showed a marked percentage of infants with

DP to have eustachian tube dysfunction

bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of

otitis media than the less severe cases (types I-III)

bull The significantly high percentage of tympanogram readings that pointed to otitis

mediasuggests an overall malfunction of the middle ear drainage function of the

eustachian tube in these children Purzycki 2009

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly

bull ―It is suspected that the developmental delay in school-aged children

diagnosed as infants suffering from plagiocephaly is caused by the

modification of the skull form

bull This study compared auditory ERPs in infants with DP and healthy

controls

bull ―Differences between the patients and control subjects indicate that already

at this early age the presence of the plagiocephalic skull signals

compromise of brain functioning

bull ―The present data suggest that most of the plagiocephalic infants have an

elevated risk of auditory processing disorders

bull This study demonstrated ―for the first time that the central sound

processing as reflected by ERPs is affected in children with

plagiocephaly Balan et al

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)

children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly

bull In contrast to prior study infants with deformational plagiocephaly did not

differ from controls with respect to the maximum P150 or N450 signals

bull ―This study marks the first electrophysiologic examination to demonstrate

that deformational plagiocephaly is not likely associated with significant

impariments in language function

bull This study used phonemes rather than tones as the external stimulus to

directly examine language processing not just auditory processing

bull ―The scope of this investigation was limited to language systems and other

areas of brain functioning may be at risk in infants with deformational

plagiocephaly

Hashim et al

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp

illustrating the P150 and N450 components Hashim et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull two abnormal conditions were identified hemifield asymmetries in which the breadth

of the left and right hemifields differed by more than 20 degrees and constricted

hemifields in which a given hemifield was at least 20 degrees less than those

established for normal controls of the same age

bull Interestingly there was no statistical correlation between the side of the visual-field

defects relative to the side of the occipital flatness However for the majority of the

16 infants for whom these comparisons were investigated the visual defects were

ipsilateral to the flattened occiput A correlation between the severity of the hemifield

constriction and the severity of the cranial asymmetry was also observed

bull These observations do not speak to whether these defects are a product of

plagiocephaly itself or the result of a more global developmental delay

Siatakowski et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects

bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral

astigmatism and 14 (15) had bilateral astigmatism

bull Children with deformational plagiocephaly do not have an increased

prevalence of strabismus compared with the general population but do

have an increased prevalence of astigmatism whereas children with

nonsyndromic craniosynostotic plagiocephaly have an increased

prevalence of strabismus and astigmatism

Gupta et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry

bull Observations made at the Washington University School of Medicine St Louis

Missouri in 1996 At that time CT scans were commonly obtained

bull 23 scans available to analyze the shape and volume of the mandible which

although not directly affected by positional forces appeared to be affected by the

altered shape of the cranium

bull This analysis found that the volume of the mandible on the affected side (ie the

side of the occipital flatness) was roughly 4 percent larger than on the contralateral

side

bull Similarly small but consistently significant differences were observed in several

other defined linear measurements between the affected and non-affected sides of

the mandible

bull Unfortunately given the preliminary nature of the article neither the possible effects

of these mandibular asymmetries on oral functions nor their response to

interventions such as remolding orthoses were investigated Kane et al

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations

Developmental Delay

bull Children with DP were more likely to require special education services in school than

their non-affected siblings (349 vs 66) Required services included speech therapy

occupational therapy and physical therapy

bull 36 of children with deformational plagiocephaly had delays in one or more domain on

the ASQ (Ages and Stages Questionnaire)

bull before any intervention infants with deformational plagiocephaly show significant delays

in both mental and psychomotor development Also of particular note is that no child with

deformational plagiocephaly showed accelerated development

bull Children with DP scored lower on all scales of the BSID-III than children without DP

Differences were largest in cognition language and adaptive behavior and smallest in

motor development These findings do not imply that DP causes developmental

problems but may nonetheless serve as a marker of developmental risk Clinicians

should screen children with DP for developmental concerns to facilitate early identification

and intervention Miller Hutchinson

Kordestani Collett

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay

bull Australian infants with DP displayed significantly weaker motor skills than normative

estimates malesgt females at very least a diagnostic marker of neurodev problems

bull A positive association between plagiocephaly and developmental delay was reported in

13 of 19 studies including 4 of 5 studies with ―strong methodological quality

bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays

Motor delay was the most commonly affected domain reported in high-quality papers

Clinicians should closely monitor infants with plagiocephaly

bull These studies suggest that ―infants with DP are at increased risk for developmental

delays in infancy and the level of risk is comparable to or even greater than the risk

level for infants with craniosynostosis

bull Results of these studies ―very tentatively suggest that DP is associated with increased

risk for developmental delay however a causal association should not be presumed

Knight Martinuk Collett

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of

increase in the number of referrals to specialty clinics Five studies have produced

varying results indicating that the incidence of plagiocephaly ranges from 31 to

610

bull This is the first study to estimate the incidence of positional plagiocephaly using 4

community-based data collection sites in infants ranging from 7 to 12 weeks of age

The estimated incidence of positional plagiocephaly was found to be 466

bull Of the 440 infants assessed 205 were observed to have some form of

plagiocephaly

bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to

be 466

bull Of all infants with plagiocephaly 632 were affected on the right side

bull Of all infants with plagiocephaly783 had a mild form

Mawji et al

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Risk Factors for Deformational Plagiocephaly bull Premature

bull Gender (boys)

bull Birth rank (first)

bull Only bottle feeding

bull Same side when bottle feeding

bull Infant head preference in supine sleeping

bull Prone positioning on monitors (tummy time lt 3 timesday when

awake)

bull ―Children with occipital VP shunts are at significant risk of

developing contralateral positional plagiocephaly particularly in the first

12 months of life

Vlimmeren 2007Stuart 2017Hutchison 2003

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Mechanism of Cranial Deformation Several Hypotheses

1) Infantrsquos head is ―soft or ―malleable (water balloon model)

-No immediate deformation takes place (severity peaks ~ 4 months)

-not all infants have their ―soft heads deformed

2) Inherent problem with bone mineralization

-no evidence to support this hypothesis either

3) Hereditary

-identical twins

-human cerebrum is not naturally flat or asymmetric

Rogers 2011

Rogers

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

History of Cranial Deformation raquo Intentional cranial deformation

Common in ancient cultures of Peru N American Chinook Indians

French aristocracy and others

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically

since 1996 and the introduction of safe sleep protocol

raquo Container Babies

raquo New life saving respiratory equipment

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Newtonrsquos first law for cranial deformation

raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force

from the bed to the infantrsquos head

raquo This counterforce will resist cranial growth in the area of contactand consequently

volume increases will be displaced to areas where there is no resistance

raquo The force applied by the head to the resting surface equals the weight of the

infantrsquos head multiplied by the force of gravity (F=mg)

raquo The process is fastest in early infancy and tapers dramatically even after the first

year of life

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

The pumpkin analogy

A stationary pumpkin growing against a firm planar surface

will become flat over time The degree of deformation is

proportionate to the rate of growth against a constant and

fixed external force This may explain why infants with larger

average head sizes (eg males and larger infants) and those

with rapid rates of head growth (eg premature infants) are

more likely to develop DP and DB

Rogers 2011

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Proposed Developmental Pathways for Mechanism of DP and Developmental Delay

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction

raquo 2 Changing crib position from parallel to perpendicular to headwall

raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright

side-lying

raquo 4 Providing cares from both sides of bed

raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)

raquo 6 Alternate feeding positions (use both arms)

raquo 7 Use supportive equipment for respiratory tubing as necessary

raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)

raquo 9 Weekly assessment monitoring and education by physical therapist

raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)

ldquoA 4-month standardized pediatric physical therapy program to treat positional preference

significantly reduced the prevalence of severe deformational plagiocephaly compared with usual

care ―

Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

Equipment and Positioning Aides raquo Alternative sleeping surfaces

raquo Custom fabricated foam cups

raquo Tortle hat

raquo Cuddle Cup (coming soon from DandleLion)

raquo Specialized pillows

raquo Orthotic HelmetBand

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction

in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526

raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting

on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology

2017 111267-279

raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199

raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational

Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389

raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz

Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January

2013 Volume 131 Number 1

raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing

Positional Plagiocephaly

raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark

MD PhDsect Traboulsi Elias I MD

raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532

raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel

A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in

deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e

raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study

Pediatrics Vol 112 No 4 October 2003

raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental

delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499

References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and

plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23

raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The

Journal of Carniofacial Surgery Vol 24 Number 4 July 2013

raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational

plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20

raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T

Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed

Tomography The Scientific World Journal

Volume 2014 (2014)

raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on

child development J Craniofac Surg 2002 Jan13(1) 99-104

raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study

Pediatrics Jul 2013 peds2012-3438

raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and

Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-

Issue 1-p 67-68

raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George

JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the

prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt

2)e497-503

raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly

Pediatrics February 2000 Vol 105Issue 2

References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-

suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion

1499-500

raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J

Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369

raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and

Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011

raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of

Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal

March 2006 Vol 43 No 2

raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz

PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal

Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006

Vol 43 No 2

raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean

Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10

raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in

children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43

raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal

of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78

raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of

Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med

2012 Jun 1 166(6) 536ndash542

References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki

Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv

Syst 2017 33(2) 275ndash28

raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for

deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-

18

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