Exostose múltipla hereditária com compressão medular

Facure, José Jorge; Facure, Nubor O.; Castro, Luiz F.

doi:10.1590/S0004-282X1975000200007

Resumos

Os autores registram um caso raro de exostose múltipla hereditária em que ocorreu compressão medular pelo crescimento de massa cartilaginosa na luz do canal raqueano. A descompessão cirúrgica por laminectomia resultou em pronta regressão do quadro neurológico.

A case of hereditary multiple exostoses successfully operated is reported. The patient, a 15 year-old white brazilian boy, was admitted with tetraplegia and Babinski's sign. Early diagnosis followed by prompt surgery may prevent permanent spinal cord damage.

Exostose múltipla hereditaria com compressão medular

Hereditary multiple exostosis with cervical cord compression: a case report

J. Jorge Facure^I; Nubor O. Facure^II; Luiz F. Castro^III

^IDepartamento de Neurologia da Faculdade de Ciências Médicas da UNICAMP: Assistente Doutor

^IIDepartamento de Neurologia da Faculdade de Ciências Médicas da UNICAMP: Coordenador do Departamento

^IIIDepartamento de Neurologia da Faculdade de Ciências Médicas da UNICAMP: Médico Residente

RESUMO

Os autores registram um caso raro de exostose múltipla hereditária em que ocorreu compressão medular pelo crescimento de massa cartilaginosa na luz do canal raqueano. A descompessão cirúrgica por laminectomia resultou em pronta regressão do quadro neurológico.

SUMMARY

A case of hereditary multiple exostoses successfully operated is reported. The patient, a 15 year-old white brazilian boy, was admitted with tetraplegia and Babinski's sign. Early diagnosis followed by prompt surgery may prevent permanent spinal cord damage.

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Full text available only in PDF format.

Clínica Neurológica Faculdade de Ciências Médicas Caixa Postal 1170 13100 Campinas, SP Brasil.

Referências bibliográficas

1. ADAM, H. & MORIN, P. Compression médullaire par exostose au cours de la maladie exostosante. Prèsse Médicale 13:660, 1966.
2. CANNON, J. F. Hereditary multiple exostoses. Am. J. Hum. Genet. 6:419, 1954.
3. CARMEL, P. W. & CRAMER, F. J. Cervical cord compression due to exostosis in patient with hereditary multiple exostosis. J. Neurosurg. 29:500, 1968.
4. CHIURCO, A. A. Multiple exostosis of bone with fatal spinal cord compression. Neurology (Minneapolis) 20:275, 1970.
5. DECKER, R. E. & WEI, W. C. Thoracic cord compression from multiple hereditary exostoses associated with cerebellar astrocytoma. J. Neurosurg. 30:310, 1969.
6. GOKAY, H. & BUCY, P. C. Osteochondroma of lumbar spine: report of a case. J. Neurosurg. 12:72, 1955.
7. JAFFE, H. L. Hereditary multiple exostosis. Arch. Path. 36:355, 1943.
8. KROOTH, R. S.; MACKLIN, M. T. & HILBISH, Diaphysial aclasis (multiple exostosis) on Guam. Am. J. Hum. Genet. 13:340, 1961.
9. LARSON, E. N.; DODGE, H. W.; RUSHTON, J. G.; & DAMLIN, D. C. Hereditary multiple exostoses with compression of the spinal cord. Proc. Staff Meet. Mayo Clin. 32:728, 1957.
10. OCHSNER, E. H. & ROTHSTEIN, T. Multiple exostosis including an exostosis within the spinal canal with surgical and neurological observations. Ann. Surg. 46:608, 1907.
11. SLEPIAN, A. & HAMBY, W. B. Neurologic complications associated with hereditary deforming chondrodysplasia: review of literature and report of two cases occurring in the same family. J. Neurosurg. 8:529, 1951.
12. SOLOMON, L. Hereditary multiple exostoses. Am. J. Hum. Genet. 16:531, 1964.
13. THIERRY, A.; AIMARD, G.; BOCHU, M.; RAMBAUD, G.; ROCHET, M. & BRUNON, J. Paraplégie au cours d'une maladie exostosante multiple. J. Med. Lyon 51:1473, 1970.
14. VINSTEIN, A. L. & FANKEN, E. A. Report of a case of hereditary multiple exostosis with spinal cord. compression. Am. J. Roentgenol. Radium Ther. Nucl. Med. 112:405, 1971.

Datas de Publicação

Publicação nesta coleção
09 Abr 2013
Data do Fascículo
Jun 1975

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

[1] 1. ADAM, H. & MORIN, P. Compression médullaire par exostose au cours de la maladie exostosante. Prèsse Médicale 13:660, 1966.

[2] 2. CANNON, J. F. Hereditary multiple exostoses. Am. J. Hum. Genet. 6:419, 1954.

[3] 3. CARMEL, P. W. & CRAMER, F. J. Cervical cord compression due to exostosis in patient with hereditary multiple exostosis. J. Neurosurg. 29:500, 1968.

[4] 4. CHIURCO, A. A. Multiple exostosis of bone with fatal spinal cord compression. Neurology (Minneapolis) 20:275, 1970.

[5] 5. DECKER, R. E. & WEI, W. C. Thoracic cord compression from multiple hereditary exostoses associated with cerebellar astrocytoma. J. Neurosurg. 30:310, 1969.

[6] 6. GOKAY, H. & BUCY, P. C. Osteochondroma of lumbar spine: report of a case. J. Neurosurg. 12:72, 1955.

[7] 7. JAFFE, H. L. Hereditary multiple exostosis. Arch. Path. 36:355, 1943.

[8] 8. KROOTH, R. S.; MACKLIN, M. T. & HILBISH, Diaphysial aclasis (multiple exostosis) on Guam. Am. J. Hum. Genet. 13:340, 1961.

[9] 9. LARSON, E. N.; DODGE, H. W.; RUSHTON, J. G.; & DAMLIN, D. C. Hereditary multiple exostoses with compression of the spinal cord. Proc. Staff Meet. Mayo Clin. 32:728, 1957.

[10] 10. OCHSNER, E. H. & ROTHSTEIN, T. Multiple exostosis including an exostosis within the spinal canal with surgical and neurological observations. Ann. Surg. 46:608, 1907.

[11] 11. SLEPIAN, A. & HAMBY, W. B. Neurologic complications associated with hereditary deforming chondrodysplasia: review of literature and report of two cases occurring in the same family. J. Neurosurg. 8:529, 1951.

[12] 12. SOLOMON, L. Hereditary multiple exostoses. Am. J. Hum. Genet. 16:531, 1964.

[13] 13. THIERRY, A.; AIMARD, G.; BOCHU, M.; RAMBAUD, G.; ROCHET, M. & BRUNON, J. Paraplégie au cours d'une maladie exostosante multiple. J. Med. Lyon 51:1473, 1970.

[14] 14. VINSTEIN, A. L. & FANKEN, E. A. Report of a case of hereditary multiple exostosis with spinal cord. compression. Am. J. Roentgenol. Radium Ther. Nucl. Med. 112:405, 1971.