In the US, the leading identified cause of infant death is birth defects, and myelomeningocele is one of the most common birth defects. Mortality rates reported for infants who are untreated for myelomeningocele range from 90-100% based on several series of studies dating from the turn of the century through recent years. Most untreated infants die within the first year of life. Death in the first 2 years of life for those untreated usually results from hydrocephalus or intracranial infection. The likelihood that a 2-month-old infant untreated for myelomeningocele lives to be 7 years is only 28%. (Facts about Spina Bifida)

Survival rates for infants born with myelomeningocele have improved dramatically with the introduction of antibiotics and developments in the neurosurgical treatment of hydrocephalus. Early death in both treated and untreated patients is associated with advanced hydrocephalus and multiple system congenital anomalies. In the US, antibiotics, sac closure, and ventriculoperitoneal shunt placement are the standard of care and are implemented in the perinatal period in 93-95% of patients. Supportive care only may be recommended in cases where there is an irreparable sac; active gross CNS infection or bleeding; and/or other gross congenital organ anomalies causing life-threatening problems (Facts about Spina Bifida).

Paraplegia from the myelodysplasia typically causes some impairment of mobility along with neurogenic bowel and bladder. Other neurological deficits may present acutely or chronically at birth or later. These deficits can be related to hydrocephalus, Arnold Chiari II malformation, or a variety of intraspinal pathologies. Seizures occur in 10-30% of affected children and adolescents. These seizures can be related to brain malformation, or they may be a sign of shunt malfunction or infection (Shure, 1993).

Musculoskeletal deterioration may be caused by progressive bony and joint deformities, pathologic fractures, and muscle deterioration. Renal compromise occurs because of problems related to neurogenic bladder. Despite advances in the management of neurogenic bladder, renal failure is still the leading cause of death in patients with myelomeningocele after the first year of life.