Historically, high mortality rate: In 2001, a mortality rate of 24% was reported in a large series of patients with 20-25 year follow-up, with the majority dying in infancy and preschool years. A rising death rate continued in adulthood after a temporary slowing down in school-aged children and teens. Interestingly, while childhood deaths were primarily related to hindbrain herniation, deaths in adulthood were related to renal failure (43).
Ambulation expected: 89% of preteen children with myelomeningocele are “community ambulators” when patients with severe developmental delay (~13% of patients) are excluded; about 50% of patients after adolescence are “community ambulators” (1, 42).
Independence expected: About 82% of adults are independent in ADLs; 32% are gainfully employed (1, 60).
Hydrocephalus a major health concern: 80% of children require treatment for hydrocephalus (1). This appears to be lower after in utero myelomeningocele repair. 85% of shunts placed at birth will malfunction within 10 years; 40% of shunts placed at birth malfunction within the first year (45). Postoperative shunt infection rates in children with myelomeningocele are approximately 11–18% (1, 43, 46).