Neuro

Myelomeningocele

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Myelomeningocele, Severe Spina Bifida

  • Epidemiology
  1. Occurs in 1 per 1000 live births
  • Risk Factors
  1. Maternal Folate Deficiency
  2. Prior history of child with Myelomeningocele (4% risk)
  3. Valproic Acid (1-2% risk if taken during pregnancy)
  • Pathophysiology
  1. Severe form of Spinal Dysraphism
  2. Affects lumbosacral region in 75% of cases
  3. Associated with dysfunction of multiple organ systems
  • Signs
  1. Flaccid Paralysis of lower extremities
  2. Deep Tendon Reflexes absent
  • Associated Conditions
  1. Hydrocephalus (80% of cases)
  2. Arnold Chiari II Malformation
  3. Neurogenic Bladder
  4. Urinary Incontinence
  5. Stool Incontinence
  • Management
  1. Supportive care may be appropriate in severe cases
  2. Surgery in first few days of life
    1. Surgical repair of Myelomeningocele
    2. CSF Shunt for Hydrocephalus
  3. Frequent Bladder Catheterization for neurogenic Bladder
    1. Reduces risk of Pyelonephritis and Hydronephrosis
  • Prognosis
  1. Mortality with aggressive treatment: 10-15% by age 4
  2. Normal intelligence in 70% of survivors
  • Complications
  1. See Ventriculoperitoneal Shunt Malfunction
  2. See Arnold-Chiari II Malformation
  3. Urinary Tract Infections
    1. Related to neurogenic Bladder
    2. Urine may be difficult to interpret (due to chronically dirty urine, especially if prior intestinal graft)
    3. Symptoms may be difficult to assess (due to cognition, differential diagnosis including shunt malfunction)
    4. Treat symptomatic Urinary Tract Infections
  4. Skin Infections
    1. Sensory deficits below Myelomeningocele allow for skin breakdown and infection
    2. Undress and fully examine all Spina bifida patients for fever source
  • Prevention
  1. Folic Acid supplementation started before conception
  • References
  1. Majoewsky (2012) EM:Rap-C3 2(9): 5
  2. Behrman (2000) Nelson Pediatrics, Saunders, p. 1804
  3. Goetz (1999) Neurology, Saunders, p. 515-16