Staged Neuroendoscopic Management of a Dandy Walker Malformation Case Presenting with Complex Hydrocephalus

Abstract

Backgroud: Dandy-Walker malformation is a rare congenital condition characterized by alteration of posterior fossa anatomy. Hydrocephalus is a common finding in these patients. VP-shunt placement remains the treatment of choice. Endoscopic Third Ventriculostomy (ETV) is an acceptable alternative for older children. Other techniques have also been proposed.
 Case presentation: A full-term infant presented with signs of hydrocephalus. An endoscopic foraminoplasty of the posterior fossa cyst was conducted as a first stage. The cyst’s volume was reduced, without any changes of the supratentorial ventricular dimensions. A month post-surgery signs of hydrocephalus reoccurred. An ETV was performed alongside a fenestration of the tentorium. The toddler was discharged in good condition with amelioration of her symptoms. One month later she presented with recurrent hydrocephalus and subdural hygromas. A shunt was placed in the posterior fossa under endoscopic guidance, and an additional one was introduced in the subdural space. The patient was followed-up for 18 months without any further need for treatment. VP-shunt placement is the treatment of choice.
 Discussion: Lushka and Magendie foraminoplasty seems to be ineffective. ETV should be reserved for older children. The ventriculoscope can be employed for positioning the VP-shunt in a specific anatomical region. Subdural hygromas may present after neuroendoscopic treatment. A staged approach may be mandatory for complex hydrocephalus treatment.