Resumos
São relatados os casos de 4 pacientes com síndrome de Dandy-Walker diagnosticada pela pneumencefalografia fracionada e ventriculografia. O tratamento e a patogenia desta malformação são discutidos com base em dados embriológicos.
Four cases of Dandy-Walker syndrome diagnosed through encephalography and ventriculography with air are reported. The pathogenesis of this malformation is discussed. Particularly stressed are the radiological aspects of the condition, which are considered to be pathognomonic. The roentgenological findings are quite typical, indicating the extreme dolichocephaly, thinning and bulging of the bones of the posterior cranial fossa, prominent separation of the lambdoid sutures and an abnormal position of the lateral sinuses. Ventriculography shows symmetric hydrocephalus with extremely large cyst -like formation in the posterior fossa. Surgical indications are also considered. The ventricle lateral shunt is the recommended treatment for the DandyWalker malformations which show cerebral subarachnoid agenesis by pneumoencephalography.
Síndrome de Dandy-walker
The Dandy-Walker syndrome. Report of four cases
Jaderson Costa da CostaI; Adão AnicetII; Mario Ferreira CoutinhoIII
IServiço de Neurologia e Neurocirurgia da Sociedade Portuguesa de Beneficência: Estagiário
IIServiço de Neurologia e Neurocirurgia da Sociedade Portuguesa de Beneficência: Neurorradiologista
IIIServiço de Neurologia e Neurocirurgia da Sociedade Portuguesa de Beneficência: Neurocirurgião-Chefe
RESUMO
São relatados os casos de 4 pacientes com síndrome de Dandy-Walker diagnosticada pela pneumencefalografia fracionada e ventriculografia. O tratamento e a patogenia desta malformação são discutidos com base em dados embriológicos.
SUMMARY
Four cases of Dandy-Walker syndrome diagnosed through encephalography and ventriculography with air are reported. The pathogenesis of this malformation is discussed. Particularly stressed are the radiological aspects of the condition, which are considered to be pathognomonic. The roentgenological findings are quite typical, indicating the extreme dolichocephaly, thinning and bulging of the bones of the posterior cranial fossa, prominent separation of the lambdoid sutures and an abnormal position of the lateral sinuses. Ventriculography shows symmetric hydrocephalus with extremely large cyst -like formation in the posterior fossa. Surgical indications are also considered. The ventricle lateral shunt is the recommended treatment for the DandyWalker malformations which show cerebral subarachnoid agenesis by pneumoencephalography.
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Serviço de Neurologia e Neurocirurgia Hospital da Sociedade Portuguesa de Beneficência Av. Independência 270 90000 Porto Alegre. RS Brasil.
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Datas de Publicação
-
Publicação nesta coleção
16 Abr 2013 -
Data do Fascículo
Mar 1973