A Case of Holoprosencephaly Associated with Dandy-Walker Cyst: A long-term follow-up

Abstract

A rare case of holoprosencephaly associated with Dandy-Walker cyst was reported. A two-day-old male baby was admitted to our clinic because of a large head. On admission, his head circumference was 39.5 cm, and there was no facial anomaly. CT showed an irregular monoventricle and a large cystic cavity in the posterior cranial fossa. Cerebral angiography demonstrated an azygos anterior cerebral artery. Confluence of sinuses and transverse sinus were markedly elevated. Deep cerebral veins were not apparent. Ventriculo-peritoneal shunt was performed. At the age of three years, he received a Dandy-Walker cyst-peritoneal shunt because of increased intracranial pressure. He became well and was discharged. At the age of nine years, he vomitted and his consciousness was disturbed. Shunt revision did not improve his conditions. The wall between the monoventricle and Dandy-Walker cyst was partially removed, and his conditions improved. In such a case like this, it is necessary to remove the wall between the ventricle and Dandy-Walker cyst.