Cystic malformations of the posterior fossa: differential diagnosis clarified through embryologic analysis.
Cystic or cyst-like malformations of the posterior fossa represent a spectrum of disorders, including the Dandy-Walker malformation, vermian-cerebellar hypoplasia, mega cisterna magna, and arachnoid cyst. Differentiation of these lesions may be difficult with routine cross-sectional imaging; however, an accurate diagnosis is essential for proper treatment planning and genetic counseling. Dandy-Walker malformation is easily diagnosed on the basis of the classic triad: complete or partial agenesis of the vermis, cystic dilatation of the fourth ventricle, and enlarged posterior fossa. Vermian-cerebellar hypoplasia is a general classification that describes congenital malformations with a normal-sized posterior fossa, varying degrees of vermian and cerebellar hypoplasia, and a prominent retrocerebellar cerebrospinal fluid space that communicates freely with a normal or dilated fourth ventricle. Mega cisterna magna can be asymmetric and can manifest apparent mass effect, simulating the appearance of an arachnoid cyst; therefore, ventriculography or cisternography may be needed to demonstrate communication of the cystic mass with the subarachnoid space. A careful review of the embryologic development is essential in understanding these malformations and in making a more accurate radiologic diagnosis.
Department of Radiology, Children's Hospital Medical Center, Cincinnati, OH 45229-2899.
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D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000235:genetics; D016080:Arachnoid Cysts / Q000175:diagnosis*; D001921:Brain / Q000002:abnormalities*; D002531:Cerebellum / Q000196:embryology*; D002946:Cisterna Magna / Q000002:abnormalities; D003388:Cranial Fossa, Posterior; D003616:Dandy-Walker Syndrome / Q000175:diagnosis*; D003937:Diagnosis, Differential; D003952:Diagnostic Imaging; D006801:Humans