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The association of fourth ventricle dilatation cyst, varying degrees of cerebelar vermis deficit and supratentorial hydrocephalus was first described in 1887 by Sutton and confirmed in 1914 by Dandy and Blackfan. Syndrome definition, diagnostic criteria and nomenclature remain a standing item of discussion among the authors. The pathogenesis appears to be based mainly in abnormal development of communication routes of cerebrospinal fluid. Genetic predisposition has been suggested, and it has been associated with various malformations and chromosomal abnormalities. The clinical manifestations are varied and are directly related to anatomical disturbance, new born most affected are asymptomatic or with minor manifestations, is the macrocephaly and evidence of high intracranial pressure that dominates the clinical picture. The diagnosis can be made prenatal by ultrasound examination, or postnatal coupled with clinical and imaging studies such as CT and MRI. The definitive treatment is performed when patients have symptomatic manifestations of hydrocephalus.

Síndrome de Dandy Walker.