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Syndrome reference sheet
Generated 13 Oct 2026
Neurology · Neurology / Pediatric neurology; Neurosurgery

Dandy–Walker malformation

Also known as: Dandy–Walker syndrome

Posterior fossa malformation with vermis hypoplasia and cystic fourth ventricle.

Medical specialty
Neurology
Subspecialty
Neurology / Pediatric neurology; Neurosurgery
Sex & age distribution
Male: affected; Female: affected equally; Children: yes, congenital
Gene(s) involved
FOXC1, LAMC1, NID1, ZIC1, ZIC2, ZIC3, ZIC4

Etiology & mechanism

Developmental failure of the cerebellar vermis; heterogeneous.

Gene(s) involved (7)

FOXC1LAMC1NID1ZIC1ZIC2ZIC3ZIC4

Symptoms & signs (4)

ataxiadevelopmental delayenlarged posterior fossahydrocephalus
SyndromeScope — educational reference. Content is model-generated and not clinically verified; always confirm against OMIM, Orphanet and GeneReviews before clinical use.