Spina Bifida: Physiotherapy in the management of meningomyelocele

06/12/2016 Ayodele Ayobami Emmanuel 1
SPINA BIFIDA: PHYSIOTHERAPY IN THE
MANAGEMENT OF MENINGOMYELOCELE
AN END OF PAEDIATRIC POSTING PRESENTATION
BY
AYODELE, AYOBAMI EMMANUEL
Presented at the Department of Physiotherapy
University of Abuja Teaching Hospital
Gwagwalada, Abuja
(October, 2016)

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 FEMALE
2011 0 0
2012 1* 0
2013 0 0
2014 0 0
2015 1 0
2016 (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 study8.

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 fat2.
• In children with L1–L3 lesions, the effect of increasing obesity is a
critical factor in the loss of ambulation2.
• 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

FUNCTIONAL INDPENDENCE MEASURE

Spina Bifida: Physiotherapy in the management of meningomyelocele

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