Spina Bifida: Physiotherapy in the management of meningomyelocele

01/05/2023 1Ayodele Ayobami Emmanuel

SPINA BIFIDA: PHYSIOTHERAPY IN THE MANAGEMENT OF MENINGOMYELOCELE

AN END OF PAEDIATRIC POSTING PRESENTATION

BY

AYODELE, AYOBAMI EMMANUEL

Presented at the Department of PhysiotherapyUniversity of Abuja Teaching Hospital

Gwagwalada, Abuja(October, 2016)

01/05/2023 Ayodele Ayobami Emmanuel 2

CONTENTS

• INTRODUCTION• EMBRYOLOGY/PATHOPHYSIOLOGY• EPIDEMIOLOGY• AETIOLOGY• DIAGNOSIS• MANAGEMENT• COMPLICATIONS• PHYSIOTHERAPY• REFERENCES


INTRODUCTION

• Spina bifida is a variable defect in which the vertebral arch of the spinal column is either incompletely formed or absent. The term bifida is from the Latin bifidus, or "left in 2 parts."1

• Classified as a defect of the neural tube (i.e. the embryonic structure that develops into the spinal cord and brain).1

• Neural tube defects have a range of presentations, from stillbirth to incidental radiographic findings of spina bifida occulta .2

• The term myelodysplasia has been used as a synonym for spina bifida. 1

• Lesions most commonly occur in the lumbar and sacral regions, but can be found anywhere along the entire length of the spine. 1


TYPES OF SPINA BIFIDA• Spina bifida is a treatable spinal cord malformation that occurs in

varying degrees of severity 2.

• Spina bifida occulta: It can occur without neurologic defects.

• Meningocele: A cystic swelling of the dura and arachnoid, protrudes through the spina bifida defect in the vertebral arch.

• Meningomyelocele: when cord tissue extends into the meningocele.

• If the spinal cord is exposed on the surface of the back, the condition is called myeloschisis 3.


Types of Spina Bifida

Retrieved from www.schn.health.nsw.gov.au


Types of Spina Bifida


EMBRYOLOGY/PATHOPHYSIOLOGY• Neural tube defects occur between the 17th and 30th day of

gestation 4.

• This defect then disrupts all of the overlying tissues, preventing the vertebral arch from closing 4.

• If the posterior vertebral arch and overlying tissues do not form normally, the normal spinal cord and meninges may then herniate out through the defect and cause a meningomyelocele (MMC) 2.

• If the vertebral arch fails to grow and fuse normally and the spinal cord and meninges are not disturbed, spina bifida occulta results 2.


MENINGOMYELOCELE• MMC is associated with abnormal development of the cranial neural

tube, which results in several characteristic CNS anomalies 2.

• The Chiari type II malformation is characterized by cerebellar hypoplasia and varying degrees of caudal displacement of the lower brainstem into the upper cervical canal through the foramen magnum 2.

• This deformity impedes the flow and absorption of cerebrospinal fluid (CSF) and causes hydrocephalus, which occurs in more than 90% of infants with MMC 2.

• Numerous other associated nervous system malformations include syringomyelia, diastematomyelia, and agenesis of the corpus callosum 2.

• Non-neurologic associations include spine malformations, hydronephrosis, cardiac defects, and gastrointestinal anomalies 2.


HYDROCEPHALUS

Retrieved from: www.mskcc.org


A Lumbar Myelomeningocele

Retrieved from: emedicine.Medscape.com


EPIDEMIOLOGY• Variations in incidence between some racial populations. • Incidence of MMC in America: 1.1 in 1000 births 5.

• The current incidence in America is about 0.6 per 1000, and there is good evidence that this has been steadily declining.

• African-American cases are often a third of those found for white Americans, while those for Hispanic-Americans are two to three times greater 2.

• 1 in 10,000 in Finland; 5 in 1000 in Northern Ireland 5. • At least 2000 cases/year in the US 5.• Nigeria??


STATISTICS

YEAR MALE FEMALE2011 0 02012 1* 02013 0 02014 0 02015 1 02016 (1st January- 24th October)

1 3

TOTAL 3 3

TABLE 1: Meningomeylocele cases at Physiotherapy Department, UATH (2011- 24th October, 2016)

*Spina bifida


AETIOLOGY• The risk of an adult with MMC having a child with a neural tube

defect is 5% 6.

• Women with low RBC cell folate levels during early pregnancy have up to a 6x greater risk of having a child with a neural tube defect 2.

• Intrauterine exposure to antiepileptic drugs, particularly valproate and carbamazepine, and to drugs used to induce ovulation 2.

• Maternal exposures to fumonisins, EM fields, hazardous waste sites, disinfection by-products found in drinking water, and pesticides 2.

• Other risk factors for MMC include maternal obesity, hyperthermia (as a result of maternal fever or febrile illness or the use of saunas, hot tubs, or tanning beds), and maternal diarrhoea 6.


DIAGNOSIS• Measurement of maternal serum α-fetoprotein (MSAFP) levels is a

common screening test.

• If the level is elevated, indicating that any portion of the foetus is not covered by skin, this screening test is then followed by detailed ultrasonography.

• Ultrasound will diagnose 92% of neural tube defects 2.

• Mothers with elevated MSAFP levels and a normal appearing ultrasound scan may be evaluated by amniocentesis for the presence of elevated acetylcholinesterase levels in the amniotic fluid 2.


MANAGEMENT OF MMC• Generally surgery follows within the first few days of life to close the

spinal cord defect 2.

• It is also important to prevent infection and additional trauma to the exposed tissues.

• Additional surgeries may be required to manage other problems in the feet, hips, or spine.

• The individuals with hydrocephalus will also require subsequent surgeries due to the shunt needing to be replaced.

• Due to the bowel and bladder problems that are often caused by the neural tube defect, catheterization may be necessary.

• The MOMS study 8.


Retrieved from: hydrocephalus.cfsites.org


COMPLICATIONS• Common complications of MMC include the following 2.• Reproductive organs impairment

• Neurogenic Bladder: The vast majority of children with MMC have a neurogenic bladder.

• Only 5.0% to 7.5% of the MMC population have normal urologic function.

• Neurogenic Bowel: Traditional bowel continence is present in approximately 10% of children with MMC.

• Musculoskeletal complications.

• Psychosocial issues: Vulnerable child syndrome

• Pressure sores, Latex allergy, Learning disabilities


NEUROSURGICAL COMPLICATIONS• Wound infection rates range from 7% to 12% 2

• Hydrocephalus; visual impairment

• Ventriculitis: low subsequent IQ

• Shunt failure

• 5% - 32% of infants with MMC will present with signs of Chiari compression, making it the most common cause of death in patients with MMC 9.

• Chiari compression can occur at any time, presentation in the first year of life is associated with up to 50% mortality 9.

• Chronic headaches are the most frequently reported symptom 9.


OBESITY• Obesity is prevalent in children with MMC. The higher the level of

lesion along the spine the higher her percentage of body fat 2.

• In children with L1–L3 lesions, the effect of increasing obesity is a critical factor in the loss of ambulation 2.

• Typically, children with MMC reach their peak ambulatory abilities around the age of 10 years. They then experience a slow decline in function over the next 10 years 2.

• The children who ambulate more have a lower percentage of body fat.


PHYSIOTHERAPY INTERVENTION

• A multidisciplinary approach towards managing patients with MMC is essential for successful outcomes.

• Patient should be assessed as soon after birth as possible.

• At different stages the focus of physiotherapy will change with changing needs of the patient.

• Regular review is essential to meet up with patient needs.

• Parents and care-givers should be involved in patient care.


CLINICAL PRESENTATION• The following can be observed in a case of MMC 2.

• Flaccid or spastic paralysis of the lower limbs

• Urinary and or faecal incontinence

• Hydrocephalus

• Poor trunk control

• Musculoskeletal complications• Scoliosis• Hip dysplasia • Hip dislocation• Hip/knee contracture • Club foot• Muscle atrophy


PHYSICAL ASSESSMENT

• Open wound• Deformities• Skin abnormalities• Sensation• Muscle tone• Muscle strength• Range of Motion• Contractures• Dislocation• Developmental Milestones


PLAN OF TREATMENT

• Prevent/correct deformity

• Maintain/improve physiological properties of joints and muscles

• Monitor normal motor development

• Educate parent(s), caregivers

• Encourage and maximise independent mobility

• Encourage participation in regular physical activity.


MEANS OF TREATMENT• Serial casting (CTEV)

• Passive mobilization, AAEX, FAEX, RAEX, stretches.

• Tactile stimulation

• Balance & Trunk control exercises

• Positioning

• Orthosis & Assistive devices

• Parent education: Parents should be educated on the child’s condition,

progress and prognosis and involved in treatment planning and home

programmes 10.


Serial casting for CTEV

Retrieved from: www.texasfootdoctor.org


Reciprocating gait orthosis (RGO)

Retrieved from: 2.bp.blogspot.com


Dennis Brown brace

Retrieved from: www.wheelsonline.com


OUTCOME MEASURE

• The functional independence measure (FIM) is the most widely

accepted functional measure.11

• FIM consists of 18 scales scored from 1 to 7; higher numbers mean

greater ability.

• Others include: CHQ, BI.


CASE PRESENTATION

• A 2 year old male who presented at the Physiotherapy department on account of inability to stand and walk since birth.

• Patient had an excision of lumbosacral MMC detected in utero on the day of delivery.• Patient had slowly evolving hydrocephalus: OFC at 2months - 42cm; 3months - 49cm; 1

year - 53.5cm; 2years - 54cm • Penile retraction and rectal prolapse on defecation• Bilateral CTEV: Had weekly serial casting for 6 weeks . Presently on Dennis Brown Splint

which is applied nightly.• No skin abnormalities observed.• Sensation absent at legs and feet.• Muscle tone: hypotonia at both lower limbs.• ROM: PROM is full and pain-free in all joints except the club feet which are plantigrade.

AROM is limited at ankles and feet.• Nil contractures.• Functional limitations: Pt can’t stand unaided; can’t walk.


Patient Update

• Currently being managed as a physiotherapy, neurosurgery, urology

and orthopaedic outpatient.

• Patient has good control of both upper limbs and trunk.

• Patient can crawl but is yet to stand or walk independently.

• CTEV: Feet plantigrade but mother not compliant with the use of

abduction brace.


CONCLUSION

• About 90% of babies born with Spina Bifida now live to be adults,

about 80% have normal intelligence and about 75% play sports and

do other fun activities 2.

• Most do well in school, and many play in sports 2.


REFERENCES

• Lundy-Ekman L (2007). Neuroscience: Fundamentals for Rehabilitation. 3rd edition. St. Louis: Saunders, 2007.

• Foster, MR (2016). Medscape Drugs & Diseases - Medscape Reference. Retrieved October 24, 2016, from http://emedicine.medscape.com/article/311113-overview

• Burke R, Liptak G (2011). Providing a Primary Care Medical Home for Children and Youth with Spina Bifida. American Academy of Pediatrics. 128:1645-1657

• Fletcher JM, Copeland K, Frederick JA (2005). Spinal lesion level in spina bifida: a source of neural and cognitive heterogeneity. Journal of Neurosurgery. 102(3 Suppl):268-79.

• Shin M, Besser LM, Siffel C, Kucik JE, Shaw GM, Lu C, Correa A, and the Congenital Anomaly Multistate Prevalence and Collaborative (2010). Prevalence of Spina Bifida Among Children and Adolescents in 10 Regions in the United States. Pediatrics.

• Canfield MA, Ramadhani TA, Shaw GM (2009). Anencephaly and spina bifida among Hispanics: maternal, sociodemographic, and acculturation factors in the National Birth Defects Prevention Study. Birth Defects Res A Clin Mol Teratol. Jul. 85(7):637-46


REFERENCES

• Goodman C, Fuller K (2009). Pathology: Implications for Physical Therapy. 3rd edition. St. Louis: Saunders, Tubbs, RS; Chambers, MR; Smyth, MD; Bartolucci, AA; Bruner, JP; Tulipan, N; Oakes, WJ (2003). "Late gestational intrauterine myelomeningocele repair does not improve lower extremity function". Pediatric Neurosurgery. 38 (3): 128–32.

• McLone DG, Knepper PA (1989). The cause of Chiari II malformations: a unified theory. Pediatric Neuroscience. 15:1-12.

• Campbell, SK, Linden, DW, Palisano RJ (2000). Physical Therapy for Children (2nd Edition). Philadelphia, PA: W.B. Saunders.

• McDonnell GV & McCann JP (2000). Issues of medical management in adults with spina bifida. Child's Nerv Syst; 16: 222−227.


THANK YOU


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Spina Bifida: Physiotherapy in the management of meningomyelocele