/Dandy-Walker syndrome

Dandy-Walker syndrome

Rare Disease

MONDO:0009072

Also Known As

Dandy Walker MalformationDandy-Walker syndromeDandy-Walker syndrome, Isolated casesatresia of foramina of Magendie and LuschkaDW complexDWSDandy-Walker complexDandy-Walker malformationDandy-Walker syndrome or malformation (type of DW complex)Dandy-Walker variant (type of DW complex)isolated Dandy-Walker malformationmega cisterna magna (type of DW complex)

Definition

Dandy-Walker malformation (DWM) is the association of three signs: hydrocephalus, partial or complete absence of the cerebellar vermis, and posterior fossa cyst contiguous with the fourth ventricle, presenting early in life with hydrocephalus, bulging occiput and posterior fossa signs such as cranial nerve palsies, nystagmus and ataxia.