Acrania: report of the first surviving case.

The first known surviving case of acrania is presented. The patient was the first child of a 29-year-old Japanese woman. Fetal ventriculomegaly was documented in the 35th gestational week. Prenatal sonography and magnetic resonance imaging suggested hydrocephalus with a wide encephalomeningocele. The baby was born at 38 weeks of gestational age by vaginal delivery. The patient had no calvarium, but did have a complete skull base with a partial defect in the occipital scalp and an underlying dural defect. Subsequently, the patient underwent repair of the scalp defect. At 3 months of age, after hydrocephalus developed, a subduro-peritoneal shunt was placed because of cosmetic and nursing problems. His developmental quotient was 10 at 3 years.