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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.18 no.3 São Paulo Sept. 1960

http://dx.doi.org/10.1590/S0004-282X1960000300002 

Síndrome de Dandy-Walker: a propósito de 4 casos

 

The Dandy-Walker syndrome: considerations on four cases

 

Gilberto Machado de Almeida

Neurocirurgião

 

 


RESUMO

Dá-se o nome de síndrome de Dandy-Walker a um tipo de hidrocefalia cuja principal característica é a transformação do IV ventrículo em um grande cisto, que ocupa quase tôda a fossa posterior; esta, por sua vez, está aumentada, devido à elevação da tenda do cerebelo e abaulamento do occipital. A patogenia do processo é discutida; admite-se geralmente que se trate de um vício de desenvolvimento determinado pela falta de abertura dos orifícios de Luschka e Magendie.
Em revisão bibliográfica foram encontrados 60 casos. São apresentados mais 4, dois dos quais foram submetidos à derivação ventrículo-jugular pela técnica de Spitz-Holter. São discutidos os principais sinais clínicos, os exames subsidiários e a terapêutica. A posição anormalmente alta dos seios transversos, observada no craniograma ou na sinugrafia, é considerada como sinal patognomônico. A transiluminação do crânio também pode apresentar aspecto característico. Êstes exames, quando típicos, permitem evitar a realização de exames mais traumatizantes, como as ventriculografias. A terapêutica mais empregada tem sido a abertura da parede do cisto. Não há na literatura referência à operação de Spitz que, em nossa opinião, é a melhor forma de tratamento.


SUMMARY

Dandy-Walker syndrome is a particular form of hydrocephalus whose main feature is the transformation of the IV ventricle into a large cyst, which occupies almost the whole of the posterior fossa; the latter, owing to the lifting of the tentorium cerebelli and bulging of the occipital bone, is enlarged. The pathogenesis is incertain; it is generally agreed that it is originated from a developmental disorder brought about by the non-opening of the foramina of Luschka and Magendie.
In the literature were found 60 cases reported. Four additional cases are reported, two of which underwent the ventriculo-jugular shunt by Spitz-Holter's technique. The main clinical signs, laboratory data and therapy are discussed. The abnormally high position of the transverse sinuses, as seen in the X-ray of the skull or in the sinugram, is considered a pathognomonical sign. The trans-illumination of the skull may also show a characteristic aspect. When the results of these exames are typical, it is possible to avoid more traumatizing ones such as ventriculographies. The therapy more commonly used is the opening of the wall of the cyst. There is no reference in the literature to Spitz's operation, which is, in our opinion, the best treatment.


 

 

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