Treatment options for Dandy-Walker malformation.

OBJECT

The aim of this study was to assess the efficacy of various treatment options available for children with Dandy-Walker malformation (DWM) and to evaluate the role of endoscopic procedures in the treatment of this disorder.

METHODS

The authors conducted a retrospective review of 72 children who underwent surgical treatment for DWM during a 16-year period. All patients underwent computed tomography scanning, and 26 underwent magnetic resonance (MR) imaging. The initial surgical treatment included ventriculoperitoneal (VP) shunt placement in 21 patients, cystoperitoneal (CP) shunt placement in 24, and combined VP and CP shunt insertion in three. Twenty-one patients underwent endoscopic procedures (endoscopic third ventriculostomy [ETV] alone in 16 patients, ETV with aqueductal stent placement in three, and ETV with fenestration of the occluding membrane in two). Three patients underwent membrane excision via a posterior fossa craniectomy. In the 26 patients who had undergone preoperative MR imaging, aqueductal patency was noted in 23 and aqueductal obstruction in three. These three patients underwent placement of a stent from the third ventricle to the posterior fossa cyst in addition to the ETV procedure. During the follow-up period, 12 patients with a CP shunt and four with a VP shunt experienced shunt malfunctions that required revision. Four patients with a CP shunt also required placement of a VP shunt. In addition, five of the 21 ETVs failed, requiring VP shunt insertion. A reduction in ventricle size noted on postoperative images occurred more frequently in patients with a VP shunt, whereas a reduction in cyst size was more appreciable in patients with a CP shunt. Successful ETV resulted in a slight decrease in ventricle size and varying degrees of reduction in cyst size.

CONCLUSIONS

Endoscopic procedures may be considered an acceptable alternative in children with DWM. The authors propose a treatment protocol based on preoperative MR imaging findings of associated aqueductal stenosis.