Dandy-Walker malformation treatment
Treatment is generally focused on alleviating hydrocephalus and posterior fossa symptoms, often including surgical interventions like ventriculoperitoneal and cystoperitoneal shunting 1).
Early treatment for ventriculomegaly is recommended to achieve maximum cognitive development. In the absence of hydrocephalus, DWM may be followed. When treatment is necessary, the posterior fossa cyst must be shunted. Shunting the lateral ventricles alone is contraindicated because of the risk of upward herniation 2)
However, it is important to confirm the patency of the cerebral aqueduct, otherwise, the supratentorial ventricles need to be shunted concurrently. Varying reports exist regarding rates of associated aqueductal stenosis, although it is widely believed to be rare.
Another option once used commonly is excision of the obstructing membrane. This has fallen out of favor due to its associated risks of morbidity and mortality. However, it remains an option for patients with frequent shunt malfunctions
Newer treatments include endoscopic third ventriculostomy in cases where the aqueduct is patent; however, further study is necessary 3) 4).
In a North American, multicenter, prospective database review, shunt-based and ETV-based primary treatment strategies of DWSH appear similarly durable. Pediatric neurosurgeons can reasonably consider ETV-based initial treatment given the similar durability and the low complication rate. However, given the observational nature of this study, the treating surgeon might need to consider subgroups that were too small for a separate analysis. Very young children with comorbidities were more commonly treated with shunts, and older children with fewer comorbidities were offered ETV-based treatment. Future studies may determine preoperative characteristics associated with ETV treatment success in this population 5).