Colpocephaly is a birth defect where the rear cavities of the brain are larger than usual. Medically the term colpocephalogy means disproportional enlargement of the occipital horns of the lateral ventricles of the cerebrum. The white matter in the cerebrum fails to develop and thicken as per normal foetal growth parameters. The disorder results in mental retardation and lack of muscular control. There is a high degree of variation in the severity of the disorder.

Causes of Colpocephaly:

This defect is thought to occur between the first and second trimesters of pregnancy due to changes in the foetal environment. Colpocephaly may be a result of genetic or metabolic disorders. Use of alcohol or prohibited drugs and maternal diabetes may also be the cause, as per available data.  This defect occurs because the white matter located in the rear portion of the cerebrum does not develop as part of regular foetal development.

Diagnosis of Colpocephaly

Colpocephaly can be diagnosed in an ultrasound scan in late pregnancy. The diagnosis is confirmed only after birth and upon manifestation of the symptoms.
Colpocephaly is indicated on an ultrasound by the appearance  of the foetal skull. The frontal skull bones appear flattened and concave. This appearance is the “lemon sign” used in ultrasonography.

Colpocephaly is often confused with hydrocephalus, another foetal brain defect. Colpocephaly is the dilation of the occipital horns while the remaining parts of the cerebrum show normal growth. This condition does not require surgical intervention nor does the condition progress. Hydrocephalus requires surgical intervention and shunting of the brain and is progressive in nature, often obstructing other brain functions.

Symptoms of Colpocephaly

Infants born with colpocephaly are likely to develop epilepsy within the first year of their life. The common symptoms of colpocephaly include:

  1. Abnormally small head
  2. Mental retardation
  3. Convulsions
  4. Excessive drooling
  5. Urinary and faecal incontinence
  6. Muscle seizures
  7. Lack of control over movement.
  8. Excessive vomiting
  9. Eye deviation


Mental retardation as a result of Colpocephaly is irreversible. The extent of retardation may range from mild to severe.

There is no standard line of treatment and the treatment is directed towards helping the affected lead as normal a life as possible. Special education may help children with developmental disabilities. Epileptic seizures affect many children affected by colpocephaly to which anti convulsing medicines bring some relief. Physical therapy is used to help in facilitating movements. Orthopaedic braces and physical therapy help children to cope with the muscle seizures and develop some degree of muscle control and provide cosmetic changes in appearance to some extent. In a few cases, surgery can help treating certain types of cephalic disorders.

Colpocephaly is a very rare syndrome and less than 50 cases have been recorded. As the syndrome causes irreversible intellectual retardation, medical research is more focussed on finding the cause of this disorder and preventing it.

Top Comments

1.hypoplastic vermis with mega cisterna magna 2.mild to moderate colpocephaly 3.subtle thickening of the cortex in bilateral posterior parietal region with ill defined gray with matter interface 4.thinning of the distal body of corpus callosum
by p.v.rao     30-Apr-2012

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