Dandy-Walker syndrome: an evaluation of surgical treatment.

Abstract

✓ Twenty-seven patients with Dandy-Walker syndrome surgically treated between 1947 and 1972 are reviewed. The results of treatment and the ultimate functional status of the patients are evaluated. The initial treatment in 17 cases was excision of cyst membranes; 15 of these patients required further surgery to control hydrocephalus, while the other two, one of whom had normal pressure preoperatively, still have large heads. Ten patients were treated by ventricular shunting alone. Thus far 11 patients have died; the commonest causes of death have been CSF infection, uncontrolled hydrocephalus, and acute shunt obstruction. In a 1½- to 20-year follow-up, eight patients have normal or near-normal mental function; these results seem to correlate with the absence or control of increased intracranial pressure. Six patients who are alive are clinically retarded; two of these had severe, diffuse neurological deficits before treatment, two had multiple shunt problems and revisions, and two were either retarded or had severe behavior problems before treatment.