Dandy-walker syndrome: A challenging problem

Abstract

Dandy-Walker syndrome is a challenging medical syndrome. The outcome of this syndrome is frequently disappointing, causing patient’s family distress and treating neurosurgeons unsatisfaction. There is no general consensus on the best method to manage this syndrome as it is very difficult to predict its outcome. One of the main causes of confusion is the enormous varieties of Dandy-Walker syndrome (DWS). Therefore, it is very important to tailor the management for each case according to the specific findings and conditions of that case. DWS is usually treated surgically by ventriculoperitoneal (VP) shunt or cystoperitoneal shunt, or both. There are some successful trials of endoscopic third ventriculostomy (ETV) as well. In most of the cases, the decision was made according to the surgeon’s experience. We hereby present our 30-year experience of dealing with DWS. In a period between 1987 and 2016, 87 cases of DWS were included in our study. However, only 21of these cases have been followed up for more than 2 years. These cases were thoroughly studied, and we came up with a classification and new concept for management. The cases of Dandy-Walker syndrome associated with hydrocephalus are classified into four subgroups according to intracranial pressure (ICP) gradient between the supratentorial and infratentorial compartments. These subgroups are: Group A, the ICP in posterior fossa (intra-DWS cyst pressure) is higher than the ICP in the supratentorial compartment. Group B, ICP is equal in both compartments. Group C, ICP is lower in the posterior fossa than the supratentorial compartment, and Group D are the cases of DWS variants.Surgery will be designed according to the classification. In principle, Group A should receive cystoperitoneal shunt, Group B receives VP shunt and cystoperitoneal shunt, Group C should receive VP shunt, and in Group D, the cyst should be connected and communicated to ventricular system and subarachnoid spaces and drained through a shunt to the peritoneal cavity.