Síndrome de Dandy-walker

Costa, Jaderson Costa da; Anicet, Adão; Coutinho, Mario Ferreira

doi:10.1590/S0004-282X1973000100007

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 Costa^I; Adão Anicet^II; Mario Ferreira Coutinho^III

^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.

1. ALENCAR, A. & BROCK, M. The Dandy-Walker syndrome. Clinical and pathological study. Acta Neurol. Latino-Amer. 8:41, 1962.
2. CARPENTER, M. B. & DRUCKEMILLER, C. W. H. Agenesis of the corpus callosum diagnosed during life: review of the literature and presentation of two cases. A. M. A. Arch. Neurol. & Psychiat. 69:305, 1953.
3. DANDY, W. E. The diagnosis and treatment of hydrocephalus due to oclusion of the foramina of Magendie and Luschka. Surg. Gynec. Obst. 32:112, 1921.
4. FAURÉ, C; LEPINTRE, J. & LYON, G. Étude radiologique du syndrome de Dandy-Walker. Acta Radiologica 1:843, 1963.
5. GARDNER, W. J.; ABDULLAH, A. F. & McCORMACK, L. J. The varying expression of embryonal atresia of the fourth ventricle in adults. Arnold-Chiari malformations, Dandy-Walker syndrome, "arachnoid cyst" of the cerebellum, and syringomyelia. J. Neurosurg. 14:591, 1957.
6. GARDNER, W. J.; McCORMACK, L. J. & DOHN, D. F. Embryonal atresia of the fourth ventricle. The cause of "arachnoid cyst" of the cerebello-pontine angle. J. Neurosurg. 17:226, 1960.
7. GUELFI, A. G.; De GARCIA, M. T. S. & De BONI, J. A. Síndrome de Dandy -Walker en el adulto. Acta Neurol. Latino-Amer. 8:284, 1962.
8. HALLER, J. S.; WOLPERT, S. M.; RABE, E. F. & HILLS, J. R. Cystic lesions of the posterior fossa in infants: a comparison of the clinical, radiological, and pathological findings in Dandy-Walker syndrome and extra-axial cysts. Neurology (Minneapolis) 21:494, 1971.
9. MACHADO DE ALMEIDA, G. Síndrome de Dandy-Walker: a propósito de 4 casos. Arq. Neuro-Psiquiat. (São Paulo) 18:224, 1960.
10. MILHORAT, T. H.; HAMMOCK, M. K. & DI CHIRO, G. The subarachnoid space in congenital obstrutive hydrocephalus: cisternographic finds. J. Neurosurg. 35:1, 1971.
11. MILHORAT, T. H.; HAMMOCK, K. & CHANDRA, R. S. The subarachnoid space in congenital hydrocephalus: microscopic finds. J. Neurosurg. 35:7, 1971.
12. PORTUGAL, J. R. & BROCK, M. Considerações sobre a patogenia da síndrome de Dandy-Walker-Brodal. Neurobiol. (Recife) 26:1, 1963.
13. RAIMONDI, A. J.; SAMUELSON, G.; YARZAGARAY, L. & NORTON, T. Atresia of the foramina of Luschka and Magendie: the Dandy-Walker cyst. J. Neurosurg. 31:202, 1969.
14. STRANDGAARD, L. The Dandy-Walker syndrome: a case with a patent foramen of the 4th. ventricle demonstrated by encephalography. British J. Radiol. 43:734, 1970.
15. TAGGART, J. K., Jr. & WALKER, A. E. Congenital atresia of foramina of Luschka and Magendie. Arch. Neurol. Psychiat. (Chicago) 48:583, 1942.
16. VAN EPPS, E. F. Agenesis of the corpus callosum with congenital malformations, including atresia of the foramina of Luschka and Magendie. Am. J. Roentg. 70:47, 1953.

Datas de Publicação

Publicação nesta coleção
16 Abr 2013
Data do Fascículo
Mar 1973

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. ALENCAR, A. & BROCK, M. The Dandy-Walker syndrome. Clinical and pathological study. Acta Neurol. Latino-Amer. 8:41, 1962.

[2] 2. CARPENTER, M. B. & DRUCKEMILLER, C. W. H. Agenesis of the corpus callosum diagnosed during life: review of the literature and presentation of two cases. A. M. A. Arch. Neurol. & Psychiat. 69:305, 1953.

[3] 3. DANDY, W. E. The diagnosis and treatment of hydrocephalus due to oclusion of the foramina of Magendie and Luschka. Surg. Gynec. Obst. 32:112, 1921.

[4] 4. FAURÉ, C; LEPINTRE, J. & LYON, G. Étude radiologique du syndrome de Dandy-Walker. Acta Radiologica 1:843, 1963.

[5] 5. GARDNER, W. J.; ABDULLAH, A. F. & McCORMACK, L. J. The varying expression of embryonal atresia of the fourth ventricle in adults. Arnold-Chiari malformations, Dandy-Walker syndrome, "arachnoid cyst" of the cerebellum, and syringomyelia. J. Neurosurg. 14:591, 1957.

[6] 6. GARDNER, W. J.; McCORMACK, L. J. & DOHN, D. F. Embryonal atresia of the fourth ventricle. The cause of "arachnoid cyst" of the cerebello-pontine angle. J. Neurosurg. 17:226, 1960.

[7] 7. GUELFI, A. G.; De GARCIA, M. T. S. & De BONI, J. A. Síndrome de Dandy -Walker en el adulto. Acta Neurol. Latino-Amer. 8:284, 1962.

[8] 8. HALLER, J. S.; WOLPERT, S. M.; RABE, E. F. & HILLS, J. R. Cystic lesions of the posterior fossa in infants: a comparison of the clinical, radiological, and pathological findings in Dandy-Walker syndrome and extra-axial cysts. Neurology (Minneapolis) 21:494, 1971.

[9] 9. MACHADO DE ALMEIDA, G. Síndrome de Dandy-Walker: a propósito de 4 casos. Arq. Neuro-Psiquiat. (São Paulo) 18:224, 1960.

[10] 10. MILHORAT, T. H.; HAMMOCK, M. K. & DI CHIRO, G. The subarachnoid space in congenital obstrutive hydrocephalus: cisternographic finds. J. Neurosurg. 35:1, 1971.

[11] 11. MILHORAT, T. H.; HAMMOCK, K. & CHANDRA, R. S. The subarachnoid space in congenital hydrocephalus: microscopic finds. J. Neurosurg. 35:7, 1971.

[12] 12. PORTUGAL, J. R. & BROCK, M. Considerações sobre a patogenia da síndrome de Dandy-Walker-Brodal. Neurobiol. (Recife) 26:1, 1963.

[13] 13. RAIMONDI, A. J.; SAMUELSON, G.; YARZAGARAY, L. & NORTON, T. Atresia of the foramina of Luschka and Magendie: the Dandy-Walker cyst. J. Neurosurg. 31:202, 1969.

[14] 14. STRANDGAARD, L. The Dandy-Walker syndrome: a case with a patent foramen of the 4th. ventricle demonstrated by encephalography. British J. Radiol. 43:734, 1970.

[15] 15. TAGGART, J. K., Jr. & WALKER, A. E. Congenital atresia of foramina of Luschka and Magendie. Arch. Neurol. Psychiat. (Chicago) 48:583, 1942.

[16] 16. VAN EPPS, E. F. Agenesis of the corpus callosum with congenital malformations, including atresia of the foramina of Luschka and Magendie. Am. J. Roentg. 70:47, 1953.