Dandy-Walker syndrome associated with congenital heart defects: report of three cases.

Three cases of Dandy-Walker syndrome associated with congenital heart defects are reported, and their management is described. The various theories concerning the aetiology of the Dandy-Walker syndrome are discussed and it is suggested that the theory of primary developmental atresia of the foramina of Magendie and Luschka is still acceptable. If the child with Dandy-Walker syndrome requires treatment by extracranial CSF drainage it is proposed that a ventriculo-peritoneal shunt is preferable when there is any suspicion of cardiac anomaly. The proximal catheter should be placed in the posterior fossa cyst (dilated fourth ventricle) rather than in the lateral ventricle.