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Dandy–Walker Malformation

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Atlas of Genetic Diagnosis and Counseling

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Abstract

In 1954, Benda (Benda 1954) introduced the term Dandy–Walker syndrome to indicate the association of (1) ventriculomegaly of variable degree, (2) a large cisterna magna, and (3) a defect in the cerebellar vermis through which the cyst communicates with the fourth ventricle. Currently, the following findings are included in the Dandy–Walker syndrome or malformation: (4) cystic dilatation of the fourth ventricle, (5) dysgenesis of the cerebellar vermis, and (6) a high position of the tentorium. The Dandy–Walker malformation has a prevalence of about 1 in 30,000 live births and is found in 4–12% of infantile hydrocephalus.

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Authors and Affiliations

  1. Medical Genetics, Shriners Hospitals for Children, Perinatal and Clinical Genetics, Department of Pediatrics, LSU Health Sciences Center, Shreveport, LA, USA

    Harold Chen

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  1. Harold Chen
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Chen, H. (2017). Dandy–Walker Malformation. In: Atlas of Genetic Diagnosis and Counseling. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-2401-1_64

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  • DOI: https://doi.org/10.1007/978-1-4939-2401-1_64

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4939-2400-4

  • Online ISBN: 978-1-4939-2401-1

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