Síndrome de Wyburn Mason, uma facomatose mista

Mello, Luís Renato

doi:10.1590/S0004-282X1975000100010

Resumos

A síndrome de Wyburn-Mason é discutida apresentando-se uma revisão bibliográfica. Consiste na presença de angiomas anteriovenosos na face, retina e região mesodiencefálica, associada a alterações neurológicas e mentais. Um caso é apresentado, sendo comentadas suas particularidades pelas quais se evidencia que a classificação da síndrome como uma facomatose pura é discutível merecendo melhores estudos.

The Wyburn-Mason syndrome is discussed on basis of a bibliographycal review. It consists on arteriovenous angiomas in the face, retina and mesencephalon associated with neurological and mental disturbances. A case is reported with remarks on its peculiarities. The questionability of the classification of the syndrome as an isolated phacomatosis is emphasized.

Síndrome de Wyburn Mason, uma facomatose mista

Wyburn Mason syndrome: a mixed phacomatosis. A case report

Luís Renato Mello

Departamento de Cirurgia (Neurocirurgia) do Hospital Santa Isabel (Blumenau, SC): Neurocirurgião

RESUMO

A síndrome de Wyburn-Mason é discutida apresentando-se uma revisão bibliográfica. Consiste na presença de angiomas anteriovenosos na face, retina e região mesodiencefálica, associada a alterações neurológicas e mentais. Um caso é apresentado, sendo comentadas suas particularidades pelas quais se evidencia que a classificação da síndrome como uma facomatose pura é discutível merecendo melhores estudos.

SUMMARY

The Wyburn-Mason syndrome is discussed on basis of a bibliographycal review. It consists on arteriovenous angiomas in the face, retina and mesencephalon associated with neurological and mental disturbances. A case is reported with remarks on its peculiarities. The questionability of the classification of the syndrome as an isolated phacomatosis is emphasized.

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Departamento de Cirurgia (Neurocirurgia) Hospital Sta. Isabel 89100 Blumenau, SC Brasil.

Trabalho do Departamento de Cirurgia (Neurocirurgia) do Hospital Santa Isabel (Blumenau, SC) apresentado no 10.° Congresso da Sociedade Brasileira de Neurocirurgia (Campinas, SP julho de 1974)

1. BONNET, P.; DECHAUME, J. & BLANC, F. L'aneurisme cirsoide de la retine (aneurisme racemeux); ses relations avec l'aneurisme de la face e l'aneurisme cirsoide de cerveau. Bull. Soc. Franc. Opthal. 51:521, 1938.
2. BROWN, D.G.; HILAL, S. K. & TENNER, M. S. Wyburn-Mason syndrome. Report of two cases without retinal involvement. Arch. Neurol. (Chicago) 28.67, 1973.
3. CECILE, J. P.; FOURNIER; LAINE, E.; COUSIN, J.; JOMIN, M. & DUTHILLEUX, P. H. Malformations arterio-veineuses cerebrales revellées par des varices peri-orbitaires chez une enfant de 3 ans. J. Radiol. Electrol. Med. Nucl. 52:451, 1971.
4. CASTROVIEJO, P. Diagnóstico Clinico-Radiologico en Neurologia Infantil. Editorial Cientifico-Medica, Barcelona, 1971, pág. 87-89.
5. FISHGOLD, H.; BREGEAT, Y.; LE BESNERAIS, Y. & DAVID, M. Inconographie de l'angiomatose neuro-retinienne (síndrome de Bonnet-Dechaume Blanc). Prèsse Médicale 60:1790, 1952.
6. FORD, F. Diseases of the Nervous System in Infancy, Childhood and Adolecence. Charles C. Thomas, Springfield (Illinois), 1966.
7. HILAL, S.; SOLOMON, G. E.; GOLD, A. P. & CARTER, S. Primary cerebral arterial occlusive disease in children: neurocutaneous syndromes. Radiology 99:87, 1971.
8. KALIN, R. E. Facial angiomatosis with angioid streats. Association of angioid streats with a component of the Sturge-Weber syndrome. Arch. Optalmol. 84:528, 1970.
9. MAY, R. & NISSL, R. Beitrag zur Klassifizierung der gemischten kongenitalen Angiodysplasien. Fortschrt. geb. Roentgens. Nuklearmed. 113:170-189, 1970.
10. OBRADOR, S.; LAMAS, E.; GOMES BUENO, J. & SABORIO, F. Um caso de angiomatosis de Bonnet Dechaume-Blanc. Revista clinica Espanhola 78: 219, 1960.
11. OBRIEN, M. S. & SCHECHTER, M. M. Arterio-venous malformations involving the galenic systems. Am. J. Roent. 110:50, 1970.
12. TASANEN, A. Bonnet-Dechaume-Blanc syndrome accompanied by mandibular angiomatosis and thrombocytopenia. Brit. J. Oral. Surg. 4:213, 1967.
13. PAILLAS, J. E.; BONNAL, J. & RIGHINI, C. Angiome encéphalo-retino-facial (syndrome de Bonnet-Dechaume-Blanc). Revue Neurol. (Paris) 101:698, 1959.
14. RASMUSSEN, T.; MATHIESON, G. & LE BLANC, F. Surgical therapy of typical and a form fruste variety of the Sturge-Weber syndrome. Schw. Arch. Neurol. Neurosurg. Psychiatrie 11:393, 1972.
15. TAMAKI, N.; FUJITA & YAMASHITA, H. Multiple anteriovenous malformations involving yhe scalp, retina, cerebrum und posterior fossa. Case report. J. Neurosurgery 34:95, 1971.
16. WYBURN-MASON, R. Arteriovenous aneurysm of midbrais and retina, facial naevi and mental changes. Brain 66:163, 1943.

Datas de Publicação

Publicação nesta coleção
09 Abr 2013
Data do Fascículo
Mar 1975

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

[1] 1. BONNET, P.; DECHAUME, J. & BLANC, F. L'aneurisme cirsoide de la retine (aneurisme racemeux); ses relations avec l'aneurisme de la face e l'aneurisme cirsoide de cerveau. Bull. Soc. Franc. Opthal. 51:521, 1938.

[2] 2. BROWN, D.G.; HILAL, S. K. & TENNER, M. S. Wyburn-Mason syndrome. Report of two cases without retinal involvement. Arch. Neurol. (Chicago) 28.67, 1973.

[3] 3. CECILE, J. P.; FOURNIER; LAINE, E.; COUSIN, J.; JOMIN, M. & DUTHILLEUX, P. H. Malformations arterio-veineuses cerebrales revellées par des varices peri-orbitaires chez une enfant de 3 ans. J. Radiol. Electrol. Med. Nucl. 52:451, 1971.

[4] 4. CASTROVIEJO, P. Diagnóstico Clinico-Radiologico en Neurologia Infantil. Editorial Cientifico-Medica, Barcelona, 1971, pág. 87-89.

[5] 5. FISHGOLD, H.; BREGEAT, Y.; LE BESNERAIS, Y. & DAVID, M. Inconographie de l'angiomatose neuro-retinienne (síndrome de Bonnet-Dechaume Blanc). Prèsse Médicale 60:1790, 1952.

[6] 6. FORD, F. Diseases of the Nervous System in Infancy, Childhood and Adolecence. Charles C. Thomas, Springfield (Illinois), 1966.

[7] 7. HILAL, S.; SOLOMON, G. E.; GOLD, A. P. & CARTER, S. Primary cerebral arterial occlusive disease in children: neurocutaneous syndromes. Radiology 99:87, 1971.

[8] 8. KALIN, R. E. Facial angiomatosis with angioid streats. Association of angioid streats with a component of the Sturge-Weber syndrome. Arch. Optalmol. 84:528, 1970.

[9] 9. MAY, R. & NISSL, R. Beitrag zur Klassifizierung der gemischten kongenitalen Angiodysplasien. Fortschrt. geb. Roentgens. Nuklearmed. 113:170-189, 1970.

[10] 10. OBRADOR, S.; LAMAS, E.; GOMES BUENO, J. & SABORIO, F. Um caso de angiomatosis de Bonnet Dechaume-Blanc. Revista clinica Espanhola 78: 219, 1960.

[11] 11. OBRIEN, M. S. & SCHECHTER, M. M. Arterio-venous malformations involving the galenic systems. Am. J. Roent. 110:50, 1970.

[12] 12. TASANEN, A. Bonnet-Dechaume-Blanc syndrome accompanied by mandibular angiomatosis and thrombocytopenia. Brit. J. Oral. Surg. 4:213, 1967.

[13] 13. PAILLAS, J. E.; BONNAL, J. & RIGHINI, C. Angiome encéphalo-retino-facial (syndrome de Bonnet-Dechaume-Blanc). Revue Neurol. (Paris) 101:698, 1959.

[14] 14. RASMUSSEN, T.; MATHIESON, G. & LE BLANC, F. Surgical therapy of typical and a form fruste variety of the Sturge-Weber syndrome. Schw. Arch. Neurol. Neurosurg. Psychiatrie 11:393, 1972.

[15] 15. TAMAKI, N.; FUJITA & YAMASHITA, H. Multiple anteriovenous malformations involving yhe scalp, retina, cerebrum und posterior fossa. Case report. J. Neurosurgery 34:95, 1971.

[16] 16. WYBURN-MASON, R. Arteriovenous aneurysm of midbrais and retina, facial naevi and mental changes. Brain 66:163, 1943.