[Cranio-vertebral junction and Morquio disease. Apropos of 6 cases]

The authors study the anomalies of the cranio-vertebral junction in the Morquio disease. The first 6 cases reported, in addition to the usual vertebral anomalies, suffered from a junctionnal malformations that were operated by posterior decompression and occipito-vertebral arthrodesis. In these cases, they especially study: --the neurological repercussion in 5 cases that could only be evidenced by a very careful examination after a stress test; --the simple roentgenographic examination, in forced position, and gaseous myelography proved on one band, that the instability, which is supposed to be responsible for the neurological symptoms, is actually inconstant (2 case out of 6). But on the other hand a narrow canal was present in every cases; --the surgical procedure performed in the 6 cases consisted of a removal of the posterior arch of the Atlas, of a widening of the occipital foramen if necessary (4 cases) and of an occipito-vertebral arthrodesis. There has been no aggravation compared to the previous state. Three indisputable improvements in the cases with a pyramidal syndrome. The authors compared their cases to those published in the literature and on which most of the time only a simple stabilisation was performed. For the authors this stabilisation can only be considered as a complementary procedure to the decompression which remains the main part of the treatment. The indications are discussed: they are based on the neurological examination and the myelography.