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Vol. 9. Issue 4.
Pages 316-320 (January 1998)
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Vol. 9. Issue 4.
Pages 316-320 (January 1998)
Diastematomielia associada a escoliose congénita. Apresentação de um caso
Diasternatornyelia associated to congenital scoliosiso. Case report
M. Resende, V. Martins
Serviço de Neurocirurgia, do Hospital de Santo António. Porto
A. Oliveira*, A. Geraldes*, J. Seabra*, R. García**
* Serviço de Ortopedia do Hospital de Santo António. Porto
** Serviço de Neuroradiologia do Hospital de Santo António. Porto
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Resumo

Diastematomielia é uma malformação embriológica rara que consiste na divisão da medula em duas hemimedulas iguais ou desiguais, por uma extensão variável, ocorrendo em situações de disrafismo espinal oculto ou “Spina bifida aperta”.

Associa-se a anomalias cutâneas, vertebrais e escoliose. Pode manifestar-se clínicamente por síndrome neuromusculoesquelética.

O tratamento cirúrgico deve ser profilático, segundo alguns autores.

Quando há escoliose congénita associada, a remoção do esporão ósseo deve ser prévia acorrecção da escoliose, se forem usadas técnicas de distracção.

Neste caso particular foi feito um tratamento preventivo duplo, ortopédico e neurocirúrgico, no mesmo tempo cirúrgico, pelo uso combinado de toracofrenolombotomia e artrodese anterior, seguindo-se laminotomia e remoção do esporão por via posterior.

Summary

Diastematomyelia is arare embryologic malformation with division of the cord in two equal or unequal portions, for a variable extension, occuring in ocult spinal disrafism or in “spina bifida aperta”.

There is association with vertebral and skin abnormalities and scoliosis. It can present with neuromusculoskeletal syndrome.

According to sorne authors the surgical treatment must be prophylactic.

When there is congenital scoliosis associated, the removal of the septum must be previous to the correction of the scoliosis, if there is indication to use distraction thecniques.

In this case we used a prophylactic combined treatment wiht anterior arthrodesis followed by a laminotomy and removal of the septum in the same surgical session.

Key words:
Diastematomyelia
Scoliosis
Surgical treatment

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