[The significance of associated malformations of the central nervous system in myelomeningocele]

Abstract

Neural tube defects are the most frequent congenital structural malformations in Norway. Approximately half of these are myelomeningocele. Infants with myelomeningocele frequently have hydrocephalus at birth. Problems with intracranial pressure may as well develop later. There are several reports on pathological corpus callosum and an increasing number of reports on cognitive problems in patients with myelomeningocele. Most patients with myelomeningocele have a tethered spinal cord, and some have syringomyelia. Chiari malformation type II is a malformation of the skull and brainstem which is frequently observed in individuals with myelomeningocele. Chiari malformation may cause severe respiratory problems in infants. Chiari malformation, tethered cord as well as syringomyelia are associated with a range of neurological problems which may progress in adulthood. Surgical intervention may improve the situation. Anaesthesia may induce neurological complications in individuals with Chiari malformation or syringomyelia. Since pregnancy and childbirth are associated with complications, women with myelomeningocele should be examined before they become pregnant. Children and adults with myelomeningocele should routinely undergo MRI examinations of caput and the spinal cord to clarify their anatomical situation.