Síndrome de Dandy-Walker: a propósito de 4 casos

Almeida, Gilberto Machado de

doi:10.1590/S0004-282X1960000300002

Resumos

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.

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.

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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Trabalho da Clínica Neurológica da Fac. Med. da Univ. de São Paulo (Prof. Adherbal Tolosa)

Clínica Neurológica - Hospital das Clínicas da Fac. Med. da Univ. de São Paulo - Caixa Postal 3461 - São Paulo, Brasil

1. BAKER, R. C; GRAVES, G. O. - Cerebellar agenesis. A. M. A. Arch. Neurol, a. Psychiat., 25:548-555, 1931.
2. BARR, M. L. - Observations on the foramen of Magendie in a series of human brains. Brain, 71:281-289, 1948.
3. BENDA, C. E. - The Dandy-Walker syndrome of the so-called atresia of the foramen Magendie. J. Neuropath, a. Exper. Neurol., 13:14-29, 1954.
4. BERTRAND, I.; MEDYNSKI, C; SALLES, P. - Étude d'un cas d'agénesie du vermis cérébelleux chez le chien. Rev. Neurol., 66:716-733, 1936.
5. BRODAL, A.; HAUGLIE-HANSSEN, E. - Congenital hydrocephalus with defective development of the cerebellar vermis (Dandy-Walker syndrome). J. Neurol., Neurosurg. a. Psychiat., 22:99-108, 1959.
6. CARREA, R.; GIRADO, M.; EURNEKIAN, A. - Hernia transtentorial de cerebelo, bloqueo tentorial y hidrocefalia. Actas y Trabajos del VI Congreso Latinoamericano de Neurocirurgia, Montevideo, págs. 103-111, 1955.
7. CLARKE, E.; LAIDLOW, J. - Silent hydrocephalus. Neurology, 8:382-386, 1958.
8. COLEMAN, C. C; TROLAND, C. E. - Congenital atresia of the foramina of Luschka and Magendie with report of 2 cases of surgical cure. J. Neurosurg., 5:84-88, 1948.
9. DANDY, W. E. - The diagnosis and treatment of hydrocephalus due to occlusions of the foramina of Magendie and Luschka. Surg., Gynec. a. Obst., 32:112-124, 1921.
10. DANDY, W. E.; BLACKFAN, K. D. - Internal hydrocephalus: an experimental, clinical and pathological study. Am. J. Dis. Child., 8:406-482, 1914.
11. DOW, R. S. - Partial agenesis of the cerebellum in dogs. J. Comp. Neurol., 72:569-586, 1940.
12. FOWLER, F. D.; ALEXANDER Jr., E. - Atresia of the foramina of Luschka and Magendie. A cause of obstructive hydrocephalus. A. M. A. J. Dis. Children, 92:131-137, 1956.
13. GARDNER, W. J. - Anatomic anomalies common to myelomeningocele of infancy and syringomyelia of adulthood suggest a common origin. Cleveland Clin. Quart., 26:118-133, 1959.
14. GARDNER, W. J. - Anatomic features common to the Arnold-Chiari and the Dandy-Walker malformations suggest a common origin. Cleveland Clin. Quart., 26:206-222, 1959.
15. GARDNER, W. J.; ABDULLAH, A. F.; McCORMACK, L. J. - The varying expressions of the embryonal atresia of the fourth ventricle in adults: Arnold-Chiari malformation, Dandy-Walker syndrome, "arachnoid" cyst of the cerebellum and syringomyelia. J. Neurosurg., 14:561-607, 1957.
16. GARDNER, W. J.; GOODALL, R. J. - The surgical treatment of Arnold-Chiari malformation in adults: an explanation of its mechanism and importance of encephalography. J. Neurosurg., 7:199-206, 1950.
17. GIBSON, J. B. - Congenital hydrocephalus due to atresia of the foramen of Magendie. J. Neuropath, a. Exper. Neurol., 14:244-262, 1955.
18. HEMMER, R. - Zum Hydrocephalus occlusus infolge congenitaler Miss-bildung am Ausgang des 4. Ventrikels. Arch. f. Psychiat. u. Zeitschr. f. d. ges. Neurol., 197:206-214, 1958.
19. HOLLAND, H. C; GRAHAM, W. i. - Congenital atresia of the foramina of Luschka and Magendie with hydrocephalus: report of a case in an adult. J. Neurosurg., 15:688-694, 1959.
20. INGRAHAM, F. D.; MATSON, D. D. - Neuro-Surgery of Infancy and Childhood. Charles Thomas, Springfield (Illinois) 1954, págs. 154-156.
21. LAURENCE, K. M. - The urinary phenolsulphon-phthalein (Phenol red) excretion test in hydrocephalus. Arch. Dis. Childhood, 32: 413-416, 1957.
22. LAURENCE, K. M. - Some applications of the urinary phenolsulphonphthalein excretion test in hydrocephalus and related conditions. Brain, 82: 551-565, 1959.
23. MALONEY, A. F. J. - Two cases of congenital atresia of the foramina of Magendie and Luschka. J. Neurol. Neurosurg. a. Psychiat., 17:134-138, 1954.
24. MATERA, R. F. - Tratamiento quirúrgico de la hidrocefalia en el niño (operación de Spitz-Holter). Rev. Brasil. Neurocir., 1:7-25, 1960.
25. MATSON, D. D. - Pre-natal obstruction of the fourth ventricle. Am. J. Roentgenol., 76:499-506, 1956.
26. McNAB, G. M. - The Spitz-Holter valve. J. Neurol. Neurosurg. a. Psychiat., 22:82, 1959.
27. RUSSEL, D. S. - Observations on the pathology of hydrocephalus. Med. Res. Council, Spec. Rep. Series, no 265, H. M. Stationary Office, Londres, 1949.
28. SAHS, A. L. - Congenital anomaly of the cerebellar vermis. Arch. Pathol., 32:52-63, 1941.
29. SCARCELLA, G. - Radiologic aspects of the Dandy-Walker syndrome. Neurology, 10:260-266, 1960.
30. SCARFF, J. E. - Spastic hemiplegia produced by a congenital cyst replacing the cerebellar vermis. J. Nerv. a. Ment. Dis., 78:400-401, 1933.
31. SCHREIBER, M. S.; REYE, R. D. - Posterior fossa cysts due to congenital atresia of the foramina of Luschka and Magendie. Med. J. Australia, 41:743-748, 1954.
32. SHRYOCK, E. H.; ALEXANDER, H. B. - Congenital malformations of the cerebellar vermis associated with dilatations of the fourth ventricle and cisternal arachnoidal cyst: report of a case. Bull. Los Angeles Neurol. Soc, 8:11-17, 1943.
33. SIDENBERG, S. S.; KESSLER, M. M.; WOLPAW, R. - A case of tetralogy of Fallot with absence of cerebellar vermis; termination by brain abscess. J. Pediat, 28:719-728, 1946.
34. SPITZ, E. B. - Neurosurgery in the prevention of exogenous mental retardation. Pediatric Clin. North America, 6:1215-1235, 1959.
35. TAGGART, J. K.; WALKER, A. E. - Congenital atresia of the foramens of Luschka and Magendie. A. M. A. Arch. Neurol, a. Psychiat., 48:583-609, 1942.
36. Van EPFS, E. F. - Agenesis of the corpus callosum with concomitant malformations, including atresia of the foramina of Luschka and Magendie. Am. J. Roentgenol., 70:47-60, 1953.
37. VERHAART, W. J. C. - Partial agenesis of the cerebellum and medulla and total agenesis of the corpus callosum in a goat. J. Comp. Neurol., 77:49-60, 1942.

Síndrome de Dandy-Walker: a propósito de 4 casos

The Dandy-Walker syndrome: considerations on four cases

Datas de Publicação

Publicação nesta coleção
05 Dez 2013
Data do Fascículo
Set 1960

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

[1] 1. BAKER, R. C; GRAVES, G. O. - Cerebellar agenesis. A. M. A. Arch. Neurol, a. Psychiat., 25:548-555, 1931.

[2] 2. BARR, M. L. - Observations on the foramen of Magendie in a series of human brains. Brain, 71:281-289, 1948.

[3] 3. BENDA, C. E. - The Dandy-Walker syndrome of the so-called atresia of the foramen Magendie. J. Neuropath, a. Exper. Neurol., 13:14-29, 1954.

[4] 4. BERTRAND, I.; MEDYNSKI, C; SALLES, P. - Étude d'un cas d'agénesie du vermis cérébelleux chez le chien. Rev. Neurol., 66:716-733, 1936.

[5] 5. BRODAL, A.; HAUGLIE-HANSSEN, E. - Congenital hydrocephalus with defective development of the cerebellar vermis (Dandy-Walker syndrome). J. Neurol., Neurosurg. a. Psychiat., 22:99-108, 1959.

[6] 6. CARREA, R.; GIRADO, M.; EURNEKIAN, A. - Hernia transtentorial de cerebelo, bloqueo tentorial y hidrocefalia. Actas y Trabajos del VI Congreso Latinoamericano de Neurocirurgia, Montevideo, págs. 103-111, 1955.

[7] 7. CLARKE, E.; LAIDLOW, J. - Silent hydrocephalus. Neurology, 8:382-386, 1958.

[8] 8. COLEMAN, C. C; TROLAND, C. E. - Congenital atresia of the foramina of Luschka and Magendie with report of 2 cases of surgical cure. J. Neurosurg., 5:84-88, 1948.

[9] 9. DANDY, W. E. - The diagnosis and treatment of hydrocephalus due to occlusions of the foramina of Magendie and Luschka. Surg., Gynec. a. Obst., 32:112-124, 1921.

[10] 10. DANDY, W. E.; BLACKFAN, K. D. - Internal hydrocephalus: an experimental, clinical and pathological study. Am. J. Dis. Child., 8:406-482, 1914.

[11] 11. DOW, R. S. - Partial agenesis of the cerebellum in dogs. J. Comp. Neurol., 72:569-586, 1940.

[12] 12. FOWLER, F. D.; ALEXANDER Jr., E. - Atresia of the foramina of Luschka and Magendie. A cause of obstructive hydrocephalus. A. M. A. J. Dis. Children, 92:131-137, 1956.

[13] 13. GARDNER, W. J. - Anatomic anomalies common to myelomeningocele of infancy and syringomyelia of adulthood suggest a common origin. Cleveland Clin. Quart., 26:118-133, 1959.

[14] 14. GARDNER, W. J. - Anatomic features common to the Arnold-Chiari and the Dandy-Walker malformations suggest a common origin. Cleveland Clin. Quart., 26:206-222, 1959.

[15] 15. GARDNER, W. J.; ABDULLAH, A. F.; McCORMACK, L. J. - The varying expressions of the embryonal atresia of the fourth ventricle in adults: Arnold-Chiari malformation, Dandy-Walker syndrome, "arachnoid" cyst of the cerebellum and syringomyelia. J. Neurosurg., 14:561-607, 1957.

[16] 16. GARDNER, W. J.; GOODALL, R. J. - The surgical treatment of Arnold-Chiari malformation in adults: an explanation of its mechanism and importance of encephalography. J. Neurosurg., 7:199-206, 1950.

[17] 17. GIBSON, J. B. - Congenital hydrocephalus due to atresia of the foramen of Magendie. J. Neuropath, a. Exper. Neurol., 14:244-262, 1955.

[18] 18. HEMMER, R. - Zum Hydrocephalus occlusus infolge congenitaler Miss-bildung am Ausgang des 4. Ventrikels. Arch. f. Psychiat. u. Zeitschr. f. d. ges. Neurol., 197:206-214, 1958.

[19] 19. HOLLAND, H. C; GRAHAM, W. i. - Congenital atresia of the foramina of Luschka and Magendie with hydrocephalus: report of a case in an adult. J. Neurosurg., 15:688-694, 1959.

[20] 20. INGRAHAM, F. D.; MATSON, D. D. - Neuro-Surgery of Infancy and Childhood. Charles Thomas, Springfield (Illinois) 1954, págs. 154-156.

[21] 21. LAURENCE, K. M. - The urinary phenolsulphon-phthalein (Phenol red) excretion test in hydrocephalus. Arch. Dis. Childhood, 32: 413-416, 1957.

[22] 22. LAURENCE, K. M. - Some applications of the urinary phenolsulphonphthalein excretion test in hydrocephalus and related conditions. Brain, 82: 551-565, 1959.

[23] 23. MALONEY, A. F. J. - Two cases of congenital atresia of the foramina of Magendie and Luschka. J. Neurol. Neurosurg. a. Psychiat., 17:134-138, 1954.

[24] 24. MATERA, R. F. - Tratamiento quirúrgico de la hidrocefalia en el niño (operación de Spitz-Holter). Rev. Brasil. Neurocir., 1:7-25, 1960.

[25] 25. MATSON, D. D. - Pre-natal obstruction of the fourth ventricle. Am. J. Roentgenol., 76:499-506, 1956.

[26] 26. McNAB, G. M. - The Spitz-Holter valve. J. Neurol. Neurosurg. a. Psychiat., 22:82, 1959.

[27] 27. RUSSEL, D. S. - Observations on the pathology of hydrocephalus. Med. Res. Council, Spec. Rep. Series, no 265, H. M. Stationary Office, Londres, 1949.

[28] 28. SAHS, A. L. - Congenital anomaly of the cerebellar vermis. Arch. Pathol., 32:52-63, 1941.

[29] 29. SCARCELLA, G. - Radiologic aspects of the Dandy-Walker syndrome. Neurology, 10:260-266, 1960.

[30] 30. SCARFF, J. E. - Spastic hemiplegia produced by a congenital cyst replacing the cerebellar vermis. J. Nerv. a. Ment. Dis., 78:400-401, 1933.

[31] 31. SCHREIBER, M. S.; REYE, R. D. - Posterior fossa cysts due to congenital atresia of the foramina of Luschka and Magendie. Med. J. Australia, 41:743-748, 1954.

[32] 32. SHRYOCK, E. H.; ALEXANDER, H. B. - Congenital malformations of the cerebellar vermis associated with dilatations of the fourth ventricle and cisternal arachnoidal cyst: report of a case. Bull. Los Angeles Neurol. Soc, 8:11-17, 1943.

[33] 33. SIDENBERG, S. S.; KESSLER, M. M.; WOLPAW, R. - A case of tetralogy of Fallot with absence of cerebellar vermis; termination by brain abscess. J. Pediat, 28:719-728, 1946.

[34] 34. SPITZ, E. B. - Neurosurgery in the prevention of exogenous mental retardation. Pediatric Clin. North America, 6:1215-1235, 1959.

[35] 35. TAGGART, J. K.; WALKER, A. E. - Congenital atresia of the foramens of Luschka and Magendie. A. M. A. Arch. Neurol, a. Psychiat., 48:583-609, 1942.

[36] 36. Van EPFS, E. F. - Agenesis of the corpus callosum with concomitant malformations, including atresia of the foramina of Luschka and Magendie. Am. J. Roentgenol., 70:47-60, 1953.

[37] 37. VERHAART, W. J. C. - Partial agenesis of the cerebellum and medulla and total agenesis of the corpus callosum in a goat. J. Comp. Neurol., 77:49-60, 1942.