Treatment of myelomeningocele: a review of outcomes and continuing neurosurgical considerations among adults.

OBJECT: Myelomeningocele is the most severe congenital malformation of the CNS that is compatible with survival. From the time of the development of practical treatment for hydrocephalus in the late 1950s, affected individuals began to survive into adulthood in substantial numbers. Data on the neurological status of these individuals are sparse, as are descriptions of their continuing requirements for neurosurgical care.
METHODS: A review of the literature was undertaken using the PubMed database maintained by the National Library of Medicine. Formal grading of the quality of evidence was not attempted, but methodological issues affecting validity or generalizability were noted.
RESULTS: Observations from 2 major longitudinal studies of cohorts of patients treated without selection using contemporary neurosurgical techniques have been published at intervals beginning in the mid-1970s. Numerous cross-sectional, institutional reviews have focused on neurosurgical issues in adulthood: hydrocephalus, Chiari malformation Type II and syringomyelia, and secondary spinal cord tethering. The organization of medical services for adults with myelomeningocele has received limited study.
CONCLUSIONS: Surviving adults with myelomeningocele achieve a wide range of neurological and functional outcomes, the most critical and adverse determinant of which is symptomatic CSF shunt failure. From a neurosurgical standpoint, adults with myelomeningocele remain clinically active indefinitely, and they deserve periodic neurosurgical surveillance.