Dandy-Walker Syndrome (definition)

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Dandy-Walker Syndrome

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Definition: A congenital abnormality of the central nervous system marked by failure of the midline structures of the cerebellum to develop, dilation of the fourth ventricle, and upward displacement of the transverse sinuses, tentorium, and torcula. Clinical features include occipital bossing, progressive head enlargement, bulging of anterior fontanelle, papilledema, ataxia, gait disturbances, nystagmus, and intellectual compromise. (From Menkes, Textbook of Child Neurology, 5th ed, pp294-5)

Other names Dandy-Walker Malformation; Syndrome, Familial Dandy-Walker; Syndrome, Dandy-Walker; Malformation, Dandy-Walker; Familial Dandy-Walker Syndrome; Dandy Walker Syndrome, Familial; Dandy Walker Syndrome; Dandy Walker Malformation; Dandy-Walker Syndrome, Familial

Substance	CAS Registry & name	Categories	Source
Dandy Walker cyst	0	*Dandy-Walker Syndrome.
Nasopharyngeal teratoma with Dandy Walker diaphragmatic hernia	0	Abnormalities, Multiple Dandy-Walker Syndrome Hernia, Diaphragmatic Nasopharyngeal Neoplasms *Teratoma.
Renal hepatic pancreatic dysplasia Dandy Walker cyst	0	Abnormalities, Multiple Dandy-Walker Syndrome *Pancreatic Cyst.
Volcke Soekarman syndrome	0	Dandy-Walker Syndrome Growth Disorders Intellectual Disability Spastic Paraplegia, Hereditary Brain/abnormalities Skull/abnormalities.
Craniosynostosis, sagittal, with Dandy-Walker malformation and hydrocephalus	0	Craniosynostoses Dandy-Walker Syndrome.
Dandy-walker malformation with mental retardation, macrocephaly, myopia, and brachytelephalangy	0	Myopia Dandy-Walker Syndrome.
Chitayat Moore Del Bigio syndrome	0	*Dandy-Walker Syndrome Developmental Disabilities Facial Bones/abnormalities.
Mental retardation, X-linked, syndromic 5	0	Basal Ganglia Diseases Dandy-Walker Syndrome Seizures Mental Retardation, X-Linked.
Dandy Walker variant	0	*Dandy-Walker Syndrome.
Dandy Walker malformation postaxial polydactyly	0	Dandy-Walker Syndrome Polydactyly.
3C syndrome	0	Abnormalities, Multiple Dandy-Walker Syndrome Heart Septal Defects, Atrial Craniofacial Abnormalities.

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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