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

Objects: forty-two patients with Dandy-Walker syndrome who were treated wit h different surgical modalities over a period of 8 years, from 1988 to 1996 , at the Sanjay Gandhi Post Graduate Institute of Medical Sciences were rev iewed in the present study. Methods: All the patients presented with hydrocephalus at the time of diagn osis. Association of other CNS anomalies was detected in 9 (22%) patients. Vermian hypoplasia was present in 36 (88%) cases, while cerebellar hypoplas ia was documented in 27 (59%) of these patients. The treatment modality for these cases has continued to change in our institution over the years. Ini tially the ventriculoperitoneal shunt was the treatment of choice and was p erformed in 28 (66%) patients. Subsequently shunting of the cyst to the per itoneal cavity was performed in 7 (16%) patients. More recently, 3 of our p atients were treated by fenestration of the cyst membrane and 4 others (9%) , by ventriculocystoperitoneal shunting procedures. In this study the addit ional insertion of cystsperitoneal 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 ventricu loperitoneal shunt diversion because of persistent ventricular dilatation. In the group of 4 patients with primary ventriculocystsperitoneal shunts, o nly 1 patient required shunt revision. The patients on whom cyst membrane f enestration was performed required no additional procedures, except for 1 w ho already had a ventriculoperitoneal shunt in situ. Conclusions: Therefore, 18 of the total 42 patients could ultimately be mad e asymptomatic by ventriculocystoperitoneal shunting for one or the other o f these reasons.