Cleft lip and palate

CCLEFT LIP AND

PALATEISA

Introduction

• Cleft lip and palate are the most common congenital craniofacial anomalies treated by plastic surgeons.

• Cleft care requires that the plastic surgeon be a member of a collaborative multidisciplinary team.

• Successful treatment of these children requires technical skill, in-depth knowledge of the abnormal anatomy, and appreciation of three-dimensional facial aesthetics.

EPIDEMIOLOGY • the most common diagnosis is cleft lip and palate at 46%,

• Isolated cleft palate at 33%

• isolated cleft lip at 21%.

• Majority of bilateral cleft lips (86%) and unilateral cleft lips (68%) are associated with a cleft palate.

• In the white population, cleft lip with or without cleft palate occurs in approximately 1 in 1,000 live births.

• twice as common in the Asian population

• half as common in African Americans

ETIOPATHOGENESIS• Both environmental teratogens and genetic factors are implicated in the genesis of

cleft lip and palate

• Intrauterine exposure to the anticonvulsant phenytoin is associated with a 10-fold increase in the incidence of cleft lip.

• Maternal smoking during pregnancy doubles the incidence of cleft lip.

• Genetic abnormalities can result in syndromes that include clefts of the primary or secondary palates among the developmental fields affected.

• The most common syndrome associated with cleft lip and palate is van derWoude syndrome

• DiGeorge, or conotruncal anomaly syndromes are the most common diagnoses associated with isolated cleft palate.

MULTIDISCIPLINARY CLEFT CARE• cleft lip and or palate require coordinated care from multiple

specialties to optimize treatment outcome.

• Typical members of a cleft team:• audiologist, dentist, geneticist, nurse, nutritionist/dietitian, oral surgeon,

orthodontist, otolaryngologist, pediatrician, plastic surgeon, psychologist, social worker, and speech pathologist.

• The goal of cleft care is to eliminate as many steps in the treatment plan as possible by optimizing the outcome and benefit of each essential intervention.

• a child with a cleft requires a complex lengthy surgical treatment plan

Head and Neck of 4-Week Old Embryo

Trigeminal nerve

Facial nerve

Glossopharyngealnerve

Vagus nerve

Embryological Background

Embryological Background

• Development of the Lip:• Unpaired Frontonasal Prominence• Medial and Lateral Nasal

prominences • 2 maxillary prominences• 2 mandibular prominences

Embryological Background• Fusion defects can occur anywhere between these

prominences

• The defect in the fusion between the frontonasal and maxillary will lead to cleft lip

Embryological Background Development of Palate:

We have two parts of two different embryonic origins:

1 ) primary palate : the triangular part of hard palate anterior to incisor foramen which originate from the premaxilla ( frontonasal prominences).

develop between 4th and 8th week of gestation 2 ) secondary palate : remaining part of the hard palate and all soft palate posterior to

incisor foramen which comes from palatine shelves of the maxillary prominences

develop between 8th and 12th week of gestation

Embryological Background

•Various theories have been given for its development. 1. Alteration in intrinsic palatal shelf force 2. Failure of tongue to drop down 3. Non fusion of shelves4. Rupture of cyst formed at the site of fusion

Classification

• We classify as the follows:• its combined (cl+cp) or isolated cleft(cl or cp)?

• is it unilateral or bilateral?

• is it complete (if it cross the nasal philtrum) or incomplete ( if it doesn’t cross the nasal philtrum.

Classification Systems

Kernahan “Striped Y”

Millard Modification of Kernahan “Striped Y”

Kriens “LAHSHAL”

Karnahan’s Classification Millard’s Modification of Karnahan’s Classifcation

Lip

Alveolus

Primary PalateHard Palate (Maxillary)Hard Palate (Palatine)Soft Palate

Kriens “LAHSHAL”L = Lip (right)A = Alveolus (right)H = Hard Palate (right)S = Soft Palate (median)H = Hard Palate (left)A = Alveolus (left)L = Lip (left)

Capital letter = complete cleftLowercase letter = incomplete cleft“.” or “-” = normal

ExamplesLA….l = complete right cleft lip and alveolus, incomplete left cleft lipLAHS = complete right unilateral cleft lip, alveolus, hard, and soft palate

Other Types of Clefts• Microform Cleft:

• May look like• a little dent in the red part of the lip • a scar from the lip up to the nostril.

• Muscle tissue underneath the cleft can be affected and may require surgery

• Submucous Cleft Palate:• Midline deficiency or lack of

muscular tissue• Often a submucous cleft palate is

associated with a bifid or cleft uvula

• Posterior nasal spine is almost always missing

• Speech Problems are common

Prenatal Diagnosis• Cleft lip can be easily

diagnosed by performing ultrasonography in the second trimester

• Diagnosing a cleft palate with ultrasonography is very difficult

• Three-dimensional imaging has been introduced to prenatal ultrasonography diagnostics of cleft anomalies

Diagnosis• Advantages of Prenatal

Diagnosis:1. Time for parental education2. Time for parental

psychological preparation3. Opportunity to investigate

other associated anomalies4. Gives parents the choice of

continuing the pregnancy5. Opportunity for fetal surgery

Etiology• “Actually no one knows exactly what causes clefts”

• Multiple factors may be involved, like:• Genetics (inherited characteristic) from one or both

parents .• Environmental factors• Drugs: corticosteroids (anti-inflammatory),

phenytoin (anticonvulsant), retinoid.• Infections: like rubella during pregnancy. • Alcohol consumption, smoking, hypoxia during

pregnancy, some of dietary and vitamins deficiencies (like folic acid and vitamin A deficiency)

• Maternal Age

GENETICSCL/P• Normal parents, one child with CL/P 4% risk CL/P in next child• Normal parents, two kids with CL/P 9% risk CL/P in next child• One parent CL/P, no affected kids 4% risk CL/P in next child• One parent CL/P, one child CL/P 17% risk CL/P in next child• Risk of CL/P in siblings increases with severity of deformity• - child with unilateral CL risk CL/P next child 2.5%• - child with bilateral CL/P risk CL/P next child 5.7%

CP• Normal parents, one child with CP 2% risk of CP in next child• Normal parents, 2 children with CP 7% risk of CP in next child• Parent with CP, no affected children 6% risk for next child• Parent with CP, one child with CP 15% risk for next child

• All infants with clefs must be evaluated for presence of other anomalies• Associated nomalies are more common in infants with isolated CP

Nonsyndromic Cleft

Pierre Robin Sequence is the most common associated nonsyndromic anomaly

• is a relative term describing the small size of the lower jaw ) and Glossoptosis (is a medical condition and abnormality which refers to the downward displacement or retraction of the tongue)

Syndromic CleftCommon Syndromes

Stickler (25% of syndromic CP) –

• autosomal dominant type 2 collagen gene

• Pierre Robin, ocular/hearing/joint malformations

Velocardiofacial (Shprintzen’s) – 15% of syndromic CP

• Autosomal dominant, variable expressivity, CATCH 22 – Deletions at 22q11,

• Facial, cardiovascular, immunologic, developmental anomalies

Van der Woude’s (19% of syndromic CL/P and CP)

• Autosomal dominant

• CL/P or CP with bilateral lower lip pitsShprintzen’

Problems Associated With Cleft Lip and Palate

•Feeding•Dental problems•Nasal Deformity and Esthetic Problems•Ear Problems•Speech Difficulties•Associated Anomalies

Feeding Difficulties

• Cleft lip= makes it more difficult for an infant to suck on a nipple

• Cleft Palate= may cause formula or breast milk to be accidently taken up into the nasal cavity

• Inability to create negative pressure inside oral cavity

• Frequent regurgitations

• Upper respiratory tract infections

Dental Problems• Local Dental Problems:

• Congenitally Missing teeth, Hypodontia, Hyperdontia, Oligodontia• Presence of natal and neonatal teeth• Anamalies of tooth morphology like microdontia, macrodontia etc• Fused teeth• Enamel Hypoplasia• Poor periodontal support, early loss of teeth• Gemination, Dilacerations

• Orthodontics Problems:• Class III tendency• Anterior and Posterior Cross bite• Spacing and crowding

Nasal Deformity and Esthetic Problems

• Facial Disfigurements

• Poor nasal shape

• Scar marks of surgeries

• Poor lip function during speech

• Poor dental alignment and smile

Ear Problems

• Middle ear disease - 22% to 88%

• Conductive hearing loss and chronic suppurative otitis media may result

• Repeated tympanostomy tube placement

Why do cleft kids have eustachian tube dysfunction? •Abnormal curvature of the eustachian tube lumen

•Cephalometric data - width and angulation of the skull base with respect to the eustachian tube are different

•Abnormal insertions of the tensor and levator veli palatini muscles into the cartilages and skull base

•Palatal muscle dysfunction

Speech Problems:

• Hearing loss hampers proper development of speech

• Velopharyngeal Insufficiency (VPI)

• Abnormal air

• Poor pronunciation of • Bilabial, • Labiodental, • Linguoalveolar sounds

C

MANAGEMENT OF CLEFT LIP AND

PALATE

Schedule of TreatmentBirth:

Initial AssessmentPre-surgical assessment

3 Month:Primary Lip repair

9-18 month:Palate Repair

2 Year:Speech assessment

3-5 Year:Lip Revision Surgery

8-9 Year: Initial interventional

OrthodonticsPreparation for alveolar bone

grafting

10 Year:Alveolar Bone Grafts

12-14 Year:Definite Orthodontics

16 Year:Nasal Revision Surgery

17-20 Year:Orthognathic Surgery

Multidisciplinary Cleft Lip And Palate Team• Genetic Scientist

• Pediatrician

• Pedodontist

• Orthodontist

• Oral and Maxillofacial Surgeon

• Prosthodontist

• ENT Surgeon

• Plastic Surgeon

• Psychiatrist

• Speech Therapist

• Social Worker

Feeding• Cleft lip = makes it more difficult for an infant to suck on a nipple• use special nipples to allow the baby to latch properly (either pump or use formula)

• Cleft Palate = may cause formula or breast milk to be accidentally taken up into the nasal cavity• don’t feed baby without palatal obturator (prosthetic palate)

• feed in an upright position to keep milk from coming out of the nose

Mead Johnson/Enfamil Cleft Feeder Special Needs Feeder / Haberman Feeder

Pigeon Feeder Dr. Brown’s Natural Flow to relieve gas

Presurgical Orthopeadics:1.Reduces the size of cleft; Aids in Surgery

2.Partial obturation aids in feeding

3.Parental Reassurance at a crucial time

• Maxillary Strapping

• Nasoalveolar Moulding Appliances (NAM)

Require orthopedic repositioning of the nasal cartilages, columella, nasal tip, and lateral wall of the vestibule

Presurgical infant nasal remodelling nasal molding

Nasal molding by post surgical nasal stenting

Facial taping 2 to 3 months

BILATERAL CLEFT LIPThe most challenging condition1. The premaxilla is extremely

protrusive

2. The premaxilla and prolabium can be of variable size

3. The columella is deficient/almost nonexistent

4. The palatal shelves are collapsed

• Protrusive maxilla imperative to be repositioned

• Premaxillary orthopedics with inraoral aplliance

• Denture adhesive

• Elastic strap

•Latham Appliance

Rule of TenPrimary repair- repaired at approximately 10 weeks • The surgeon usually uses the “Rule of Ten”• The child weighs 10 pounds • The child has a hemoglobin of at least 10 grams • The child has a white count of no higher than 10,000 • The child is at least 10 weeks of age

Surgical Techniques

• Cleft Lip Repair

•unilateral• rotation-advancement

flap developed by Millard

• complications

•dehiscence • infection• excess tension

Surgical Techniques

•Cleft Lip Repair•bilateral

• bilateral rotation advancement with attachment to premaxilla mucosa

Cleft Palate Repair - Timing

• Dorf and Curtin• 10% occurrence of articulation errors when palatoplasty was completed by 1 year

• 86% incidence of articulation errors when repair was complete after 1 year

• Haapanen and Rantala – • Significantly fewer children in the groups repaired before 18 months had hypernasal speech, articulation errors, or required secondary surgery to correct speech

Cleft Palate Repair•Schweckendick’s Primary Veloplasty

•V-Y Pushback•Von Langenbeck Palatal Repair•Furlow Palatoplasty

Cleft Palate RepairSchweckendick’s Primary Veloplasty

• Incisions made in soft palate

• Muscle bundles released from the posterior hard palate and rotated

• Reconstruction of levator sling

• Closure of mucosal layers separately

Cleft Palate RepairV-Y Pushback (WARDILL OPERATION)

• Two uni-pedicled flaps (greater palatine artery) and one or two anteriorly based pedicled flaps

• Posterior flaps rotated in a V-Y advancement technique - increasing the length of the palate

• Nasal mucosa not closed

• Improved speech results compared with bipedicled techniques

• Indicated for incomplete clefts

Von Langenbeck Operation:

Cleft Palate RepairFurlow Palatoplasty

• Lengthens the soft palate• Reconstructs the muscle sling. • Also commonly used to correct velopharyngeal insufficiency in patients with

submucous cleft palate• Speech outcomes are improved compared with other palatoplasty

techniques.

How do you manage ear disease? • 96% of children with cleft

palate required tympanostomy tube placement

• 50% of these children required repeat tympanostomy tube placement.

• Frequency of otitis media decreases as the child with CP ages

• Audiology and tympanometry as well as exams / clinical history

Orthodontic Treatment of Transitional Dentition• The purpose the dentition adjacent to cleft has to be orthodontically

repositioned to prepare the cleft side for the secondary alveolar bone graft

• Preparing the maxillary arch for a bone graft (6-12 months) :

1. Bonded edgewise appliance

2. Supported with a maxillary expander quad helix expander

Alveolar Bone Grafting

• Primary Bone Grafting• Bone graft done at the time of primary cheiloplasty• Bone graft done during the first 2 years of life• Bone graft done prior to the eruption of the primary canine

Secondary Bone Grafting

• Done before eruption of the permanent canine

• Usually when the root of the canine is 1/3 to 2/3 formed

• Usually between ages 8-10

• In CLP dental age is usually behind chronological age

Quadhelix to expand prior to ABG

angle brackets to keep roots away from cleft

Alveolar Bone Grafting1. Provide bone for the eruption and/or orthodontic repositioning of teeth

2. Closure of oro-nasal fistulas

3. Support and elevation of the alar base

4. Stabilization of the pre-maxilla in bilateral cases

5. Provide continuity of the alveolar ridge

Types of bone grafts• Autogenous

• Cancellous- iliac crest• Cortical- calvarium, mandible• Cortico-cancellous- iliac, rib, tibia, mandible

• *Allogeneic• Graft resorbs, remodels, may contribute to osteoinduction and osteoconduction

• **Alloplast• Bone grows into, around alloplast• No active osteoinduction but some osteoconduction• Teeth do not erupt through alloplast

*Nique T, Fonseca RJ, et al: Particulate allogeneic bone grafts into maxillary alveolar clefts in humans- A preliminary report. J Oral Maxillofac Surg 45: 386-392, 1987

**Horswell BB, El Deeb M: Nonporous HA in the repair of alveolar cleft defect in a primate model. J Oral Maxiilofac Surg 47:946-952, 1989

Alveolar Bone Grafting

Alveolar Bone Grafting

Preoperative Cleft Defect Postoperative Bone Graft

•Correction of anterior crossbite•Arch expansion

Quad HelixExpansion screws

Orthodontic Treatment For Permanent Dentition

Orthognathic Surgery

• Midfacial Advancement

• LeForte osteotomies• leave vascular pedicle attached in

back of maxilla - prevents necrosis

Rhinoplasty

• Rhinoplasty

• standard techniques• tip projection• alar rotation• columellar length

• Age =17-20

Surgical Management of Velopharyngeal InsufficiencyMajor Goals of Surgery

• Close the gap or hole between the roof of the mouth and the nose.

• Reconnect the muscles that normally make the palate work.

• Make the repaired palate long enough so that when the muscles are working, the palate can perform its function properly.

Velopharyngeal Insufficiency VPI

Wardill Operation: WY push back

• Dorrance and Brown’s – U shaped push back palatoplasty

Prosthetic Management of VPI

REFERENCES

1.Thorne CH, Grabb WC, M.D RWB. Grabb And Smith’s Plastic Surgery. 6th ed. Wolters Kluwer Health/Lippincott Williams & Wilkins; 2007.

2.Brunicardi F, Andersen D, Billiar T, Dunn D, Hunter J, Matthews J, et al. Schwartz’s Principles of Surgery. 9th ed. McGraw-Hill Education; 2009.

CTHANK YOU