Encephalocele is a neural defect of development which creates a defect in the skull through which the brain covering, and sometimes the brain substance, emerges. It is often combined with other malformations, including hydrocephaly and microcephaly. Small and moderate encephaloceles can be operated on successfully and survival is good if no other serious malformation jeopardizes the child's life. However, if the brain is involved in the malformation, neurological or metal deficit may occur.
This malformation is relative infrequent (usually less than 2 per 10,000 births). Relatively high rate are seen, however, in Sichuan, China, Atlanta, USA and Mexico (where affected infants also had cataracts). The recorded rates for England and Wales have declined markedly over the past 20 years.