COLPOCEPHALY is a disorder in which there is an abnormal enlargement of the occipital horns–the posterior or rear portion of the lateral ventricles (cavities or chambers) of the brain. This enlargement occurs when there is an underdevelopment or lack of thickening of the white matter in the posterior cerebrum. Colpocephaly is characterized by microcephaly (abnormally small head) and mental retardation. Other features may include motor abnormalities, muscle spasms, and seizures.
Although the cause is unknown, researchers believe that the disorder results from an intrauterine disturbance that occurs between the second and sixth months of pregnancy.
Colpocephaly may be diagnosed late in pregnancy, although it is often misdiagnosed as hydrocephalus (excessive accumulation of cerebrospinal fluid in the brain). It may be more accurately diagnosed after birth when signs of mental retardation, microcephaly, and seizures are present.
There is no definitive treatment for colpocephaly. Anticonvulsant medications can be given to prevent seizures, and doctors try to prevent contractures (shrinkage 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. Some children benefit from special education.