Dandy-Walker syndrome

Definition:

Dandy-Walker syndrome (DWS) is a congenital brain malformation, characterized by posterior fossa cyst, cystic dilatation of the fourth ventricle, cerebellar vermis dysgenesis, and an upwardly displaced tentorium. Patients often have motor deficits such as delayed motor development, hypotonia and ataxia. About half have mental retardation, and some have hydrocephalus. It has been suggested that heterozygous loss of ZIC1 and ZIC4 is the cause of this disease. Various managements of DWS have been reported from open excision of the cysts to CSF diversion. Ventriculoperitoneal (VP) and cystoperitoneal (CP) shunt insertion are the most common choices in the treatment of DWS.

References:

[1]. Inessa Grinberg, et al. Heterozygous deletion of the linked genes ZIC1 and ZIC4 is involved in Dandy-Walker malformation. Nat Genet. 2004 Oct;36(10):1053-5. [Content Brief]

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