Dandy-Walker syndrome: different modalities of treatment and outcome in 42 cases

Forty-two patients with Dandy-Walker syndrome who were treated with different surgical modalities over a period of 8 years, from 1988 to 1996, at the Sanjay Gandhi Post Graduate Institute of Medical Sciences were reviewed in the present study.

All the patients presented with hydrocephalus at the time of diagnosis. Association of other CNS anomalies was detected in 9 (22%) patients. Vermian hypoplasia was present in 36 (88%) cases, while cerebellar hypoplasia was documented in 27 (59%) of these patients. The treatment modality for these cases has continued to change in our institution over the years. Initially the ventriculoperitoneal shunt was the treatment of choice and was performed in 28 (66%) patients. Subsequently shunting of the cyst to the peritoneal cavity was performed in 7 (16%) patients. More recently, 3 of our patients were treated by fenestration of the cyst membrane and 4 others (9%), by ventriculocystoperitoneal shunting procedures. In this study the additional insertion of cystoperitoneal shunts was required in 8 (27%) of the 28 patients who had primary ventriculoperitoneal shunt procedures, because of either nonresolution or reappearance of a posterior fossa cyst. Six out of the 7 cases of primary cystoperitoneal shunts required additional ventriculoperitoneal shunt diversion because of persistent ventricular dilatation. In the group of 4 patients with primary ventriculocystoperitoneal shunts, only 1 patient required shunt revision. The patients on whom cyst membrane fenestration was performed required no additional procedures, except for 1 who already had a ventriculoperitoneal shunt in situ.

Therefore, 18 of the total 42 patients could ultimately be made asymptomatic by ventriculocystoperitoneal shunting for one or the other of these reasons.