Dandy-Walker Malformation & Variants

Dandy-Walker malformation is characterized by agenesis or hypoplasia of the cerebellar vermis and a posterior fossa cyst, which represents cystic dilation of the fourth ventricle. It is also associated with hydrocephalus, according to specialists at the University Center for Fetal Medicine. Dandy-Walker variant consists of cerebellar dysgenesis, variable hypoplasia of the cerebellar vermis and the lack of a posterior fossa cyst or enlarged posterior fossa. Ventricular dilation may also be present.  

Treatment

There is no fetal intervention for Dandy-Walker malformation or variants. Routine pregnancy care will continue throughout the pregnancy, and genetic counseling is offered. Delivery should occur at a tertiary care center as the neonate will need a neurosurgical evaluation and an in-depth anatomical assessment.

Postnatal treatment of the Dandy-Walker malformation or variants consists mainly of placement of a ventriculoperitoneal (VP) shunt for management of symptomatic hydrocephalus. The shunt will redirect fluid to a cavity in another area of the body. 


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