congenital hydrocephalus


con·gen·i·tal hy·dro·ceph·a·lus

hydrocephalus due to a developmental defect of the brain. Synonym(s): primary hydrocephalus

congenital hydrocephalus

Hydrocephalus occurring in newborns, typically caused by birth defects such as spina bifida, aqueductal stenosis, or birth trauma with ventricular hemorrhage.

In congenital hydrocephalus, the faulty drainage of CSF from the ventricles of the brain often results in rapidly increasing head circumference, malformation of the skull (thin bone with widened fontanels and separated sutures), distended scalp veins, thin, shiny scalp skin, weak neck muscles incapable of supporting the head, and abnormal development of psychomotor and cognitive or language skills. In untreated cases of congenital hydrocephalus, the outcome is fatal in about half of the patients due to infection, malnutrition, or increased intracranial pressure. The parents of infants treated neurosurgically for congenital hydrocephalus are instructed in signs and symptoms that may indicate surgical complications: fever and headache, irritability, poor feeding, inconsolability.

See also: hydrocephalus