An encephalocele is a herniation of brain tissue through the skull, which occurs during pregnancy due to incomplete neural tube closure. Although the etiology of encephalocele is not well understood, it is thought to be a genetically inherited condition.
An infant born with encephalocele will have a sac-like projection of brain and associated membranes protruding from between the forehead and nose or at the back of the head. Treatment of encephalocele takes place early in life and involves craniofacial and neurosurgical specialists in order to maximize both facial form and neurological function. The procedure involves reduction of herniated brain tissue and repair of any associated bony and skin defects. Prognosis depends upon the amount of tissue within the mass that needs to be removed and the nature of the brain tissue that remains intact.