Colpocephaly

Colpocephaly is a disorder in which there is evidence of abnormal growth of the occipital horns —the posterior portion of the lateral ventricles (cavities or compartments) of the brain. This abnormal growth occurs when there is underdevelopment or a lack of thickening in the white matter of the hindbrain. Microcephaly (abnormally small head) and mental retardation are characteristic of colpocephaly. Other conditions include motor abnormalities, muscle spasms, and seizures.

Although the cause is unknown, researchers believe the disorder results from an intrauterine problem that occurs between the second and sixth months of pregnancy. Colpocephaly can be diagnosed late in pregnancy, although it is often misdiagnosed as hydrocephalus (an excessive buildup of cerebrospinal fluid in the brain). It can be more accurately diagnosed after birth when signs of mental retardation, microcephaly, and seizures are evident.

There is no definitive treatment for colpocephaly. Anticonvulsant drugs may be given to prevent seizures, and doctors often prevent contractures (the contraction or shortening of muscles). The prognosis for individuals with colpocephaly depends on the severity of the associated conditions and the degree of abnormal brain development. Special education can benefit some children.

Some of the central nervous system disorders that are associated with colpocephaly are:

• polimicrogiria
• Perventricular leucomacy (PVL)
• intraventricular bleeding
• hydrocephalus
• esquizencefalia
• microgiria
• microcephaly
• Pierre-Robin Syndrome
• neurofibromatosis