Meningomyelocoele is the most severe type of spina bifida where there is a cystic swelling over the spinal defect containing meninges, nerve roots, and spinal cord outside the vertebral canal. It occurs due to failure of closure of the neural tube during development. Clinical features include lower limb paralysis, bladder and bowel dysfunction, hydrocephalus, and Arnold-Chiari malformation in 95% of cases. Treatment involves closure of the defect soon after birth along with lifelong multidisciplinary care. One study found that children with meningomyelocoele who could walk, even with aids, performed better cognitively than those who used wheelchairs, suggesting locomotion may positively impact development.

1. Meningomyelocoele
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2. Introduction
• Meningomyelocoele is the most severe type of SPINA
BIFIDA.
• Spina Bifida means "split spine”
• 4 types of Spina Bifida are:
– Spina bifida occulta,
– Meningocele
– Spina bifida cystica (Myelomeningocele) and
– Lipomeningocele
• Meningomyelocoele
– occurs due to Failure of closure of the neural tube
during the third week of gestation
– abnormal differentiation of the embryonic neural tube
• In Meningomyelocoele, a cystic swelling occurs over
the site of the spinal defect which contains meninges,
nerve roots, and the spinal cord itself which has left
the vertebral canal

3. Spina bifida

4. Spinal Dysraphism
Spina bifida occulta Meningocele Myelomeningocele

5. Statistics Spina Bifida

6. Incidence - Meningomyelocoele
• 1-2/1000 live birth
• Neural tube defects are
second most common type
of birth defect
• Myelomeningocele is the
most common form of neural
tube defect.
• Slightly higher in females
than in males (1.2:1)
• 85% occur in lumbosacral
• region

7. Aetiology
• Poor nutrition - Folic acid deficiency
• Genetics (People of Northern European and Hungarian
ancestry have the highest rates of the disease)
• Chromosome abnormalities:
– Trisomies 13 and 18
– Triploidy
– Single gene mutations
• Maternal obesity, hyperthermia
• pregnancies complicated by diabetes at conception
• Drugs: valproate, carbamazepine and drugs to induce
ovulation
• Maternal exposures to fumonisins, electromagnetic fields,
hazardous waste sites, disinfection by-products found in
drinking water and pesticides

8. Meningomyelocele – Clinical
Features
• Both meninges and spinal cord protrude
into the skin of the back
• Leak of cerebrospinal fluid (CSF) is common
• Severe neurological deficits are common
• Risk for bacterial meningitis
• Paraplegia
• Diminished control of lower limbs, bladder Hydrocephalus
and bowel
• Hydrocephalus often accompanies
• Short stature and precocious puberty
Paraplegia

9. Primary Functional System Congenital
deficits Anomalies
• Lower limb paralysis
• Facial clefts
• Sensory loss
• Heart
• Bladder and bowel malformations, and
dysfunction
• Genitourinary tract
• Cognitive dysfunction anomalies
• Cranial nerve • Urinary tract
dysfunction anomalies
– Ocular muscle palsies,
– solitary kidney or
– Swallowing andeating
– malformed ureters
problems and
– Abnormal phonation

10. Arnold Chiari II in 95% of
myelomeningocoele
• Herniation of cerebella vermis through
foramen Magnum (cerebella tonsils extend
down into the spinal cord, lower CN run up
instead of down)
• Dislocation of the 4th ventricle towards
neural canal
• Associated with hydrocephalus 85%
• Developmental abnormalities in CV, GI,
GU systems 10%

11. DIAGNOSIS
• Ultrasound during the second trimester
• The diagnosis of myelomeningocele is certain when 3 classic
central findings are present in ultrasonography
– concavity of the frontal bones,
– ventriculomegaly, and
– Chiari II malformation.
• Positive screening for maternal serum alpha-fetoprotein (AFP)
• Screening of the amniotic fluid for AFP, as well as for the
presence of acetylcholinesterase
Ultra Sound
Coronal view of spine Lemon" shaped skull
Sagittal view of spine

12. Treatment
• Intrauterine surgery
• Post-natal surgery:- Closure of the Myelomeningocele is performed
immediately after birth if external CSF leakage is present and
typically within the first 24-48 hours in the absence of CSF leakage
• Multidisciplinary interventions needed to prevent progressive
deterioration of multiple body systems
• Treatment team consists of pediatric specialists in
– Physical medicine and rehabilitation
– Neurosurgery
– Urology
– Orthopedics along with
– Pediatric nursing
– Physical therapy
– occupational and recreational therapy
– psychology and
– medical social work

13. Medication
• Anticholinergics: - to suppress detrusor
overactivity eg. Oxybutynin chloride
• Tricyclic antidepressants, eg.
Imipramine hydrochloride
• Alpha-adrenergic antagonists: -
decrease bladder outlet resistance,
increase urinary flow rate, and improve
bladder emptying eg. Terazosin

14. Locomotion improves the
cognitive profile of children
with Meningomyelocele?
• Twenty-nine children with MMC and shunted hydrocephalus
were studied.
• All had motor impairment, but after physiotherapy and training,
walking was possible in 23 of them (5 autonomously and 18
with an aid), while 6 had recourse to a wheelchair.
• Even though global I.Q. in these MMC children was within the
normal range, the characteristic splitting between verbal I.Q.
(VIQ) and performance I.Q. (PIQ) was observed, but the most
interesting observation was a significant performance-related
difference (P=0.044 and P=0.012) between ambulatory patients
(both with and without aids) and those who were dependent on
wheelchairs (PIQ: 83-85 vs 63).
Reference: Rendeli C. et.al; Does locomotion improve the cognitive profile of children with
meningomyelocele?; Child’s Nerv Syst (2002) 18:231–234