Coluna / Columna v.15, n.1 /2016

ARTIGO ORIGINAL


RESULTS OF SURGICAL TREATMENT IN THE CRANIOCERVICAL JUNCTION IN MUCOPOLYSACCHARIDOSIS



Luís Eduardo Carelli Teixeira da Silva1 , Alderico Girão Campos de Barros1 , Lucas Rocha Cavalcanti1 , Caique Jauhar de Castro1 , Renato Henriques Tavares1 , Raphael Teofilo de Souza1

1 Instituto Nacional de Traumatologia e Ortopedia - Rio de Janeiro, RJ, Brasil

DOI: http://dx.doi.org/



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ABSTRACT

Objective:
Evaluate the results after decompression and stabilization of craniocervical junction in patients with mucopolysaccharidosis (MPS).

Method:
Retrospective study of 10 patients with MPS through the analysis of medical records and additional tests.

Result:
All patients with mid-term and long-term follow-up achieved consolidation of the arthrodesis and 87.5% had neurological improvement of Nurick score.

Conclusion:
Early diagnosis and intervention in cases of stenosis and/or craniocervical instability of patients with MPS provide patients a good recovery of neurological function, despite the great technical difficulty and risk of complications.


Keywords: Mucopolysaccharidosis IV,Spinal cord compression,Spinal Cord Diseases

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