What is congenital hydrocephalus?

Updated: Jun 04, 2018
  • Author: Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP; Chief Editor: Jasvinder Chawla, MD, MBA  more...
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Congenital hydrocephalus applies to the ventriculomegaly that develops in the fetal and infancy periods, often associated with macrocephaly. [8] The most common causes of congenital hydrocephalus are obstruction of the cerebral aqueduct flow, Arnold-Chiari malformation or Dandy–Walker malformation. [9]  These patients may stabilize in later years due to compensatory mechanisms but may decompensate, especially following minor head injuries. During these decompensations, determining the extent to which any new neurological deficits may be due to the new acute event, compared with hydrocephalus that may have gone unnoticed for many years, is difficult. An extremely severe variant of congenital hydrocephalus is hydranencephaly, where the brain's cerebral hemispheres are absent to varying degrees and the remaining cranial cavity is filled with cerebrospinal fluid.

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