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Craniofacial Conditions: Encephalocoele

Sometimes, the brain forces an opening through a weak or defective portion of the skull. Then, the brain and the membranes covering it protrude from the skull in a sac-like form, known as an encephalocoele (en=in; ceph=head; coele=opening). Also referred to by its Latin name, cranium bifidum, encephaloceles are rare, occurring at a rate of one per 5,000 live births.

Usually diagnosed immediately after birth, occasionally a small encephalocele may go undetected for a period of time, particularly in the nasal or forehead area. Encephaloceles may be found anywhere in the head and face region, frequently occurring in the eye region or the back of the head. Sometimes they create a cleft in the roof of the mouth (palate).

Encephaloceles are frequently accompanied by other head and face anomalies or brain malformations. Neurological problems, vision problems, and various physical and mental developmental delays are some of the other symptoms that may be experienced.

Encephaloceles are caused by the failure of the neural tube to close completely when the fetus is developing.

Typically, surgery is performed on young patients during infancy or their toddler years. The encephalocoele is corrected by gently pushing the brain tissue back into place, surgically closing the area of bony weakness from which the brain tissue has come through, and correcting any associated head or face abnormalities.