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Definition/Background
Dandy-Walker malformation is a congenital abnormality
characterized by an enlarged posterior fossa, high position of the tentorium
and torcula herophili, partial or complete absence of the cerebellar vermis,
and a cystic dilation of the fourth ventricle.
Characteristic
Clinical Features
Developmental delay and the signs and symptoms of
hydrocephalus are known presenting features.
Characteristic
Radiologic Findings
Imaging shows partial or complete hypoplasia of the
cerebellar vermis, a large fourth ventricle, a large posterior fossa, and
highly placed tentorium and torcula herophili.
Less Common
Radiologic Manifestations
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Associated central nervous system anomalies include
hydrocephalus, corpus callosum agenesis, heterotopias, schizencephaly, and
cephaloceles.
Primary Differential
Diagnoses
1-Dandy-Walker Variant
2-Mega Cisterna Magna
3-Arachnoid Cyst
Discussion of
Differential Diagnoses
Dandy-Walker Variant:
Consists of vermian hypoplasia and cystic dilation of the fourth ventricle without
enlargement of the posterior fossa.
Mega Cisterna Magna:
Consists of an enlarged cisterna magna that can result in an enlarged posterior
fossa. However, the cerebellar vermis and fourth ventricle are normal.
Arachnoid Cysts:
Sometimes can be large and result in an enlarged posterior fossa. However, the
fourth ventricle and vermis are normally formed and often displaced by the
cyst.
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Labels: Brain, Congenital Anomalies
