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