Stevens J M, Kendall B E, Crockard H A, Ransford A
National Hospital for Neurology and Neurosurgery, London, England.
J Bone Joint Surg Br. 1991 Sep;73(5):851-8. doi: 10.1302/0301-620X.73B5.1910048.
High definition computed cervical myelograms have been made in flexion and extension in 13 patients with Morquio-Brailsford's disease. We observed that: 1) odontoid dysplasia was present in every case, with a hypoplastic dens and a detached distal portion which was not always ossified; 2) atlanto-axial instability was mild, and anterior atlanto-axial subluxation was absent in most cases; 3) severe spinal cord compression, when present, was due to anterior extradural soft-tissue thickening; 4) this compression was not relieved by flexing or extending the neck and was manifested early in life; 5) posterior occipitocervical fusion resulted in disappearance of the soft-tissue thickening and normalisation of subsequent development of the dens. We conclude that the severity of neurological involvement at the craniovertebral junction was determined by soft-tissue changes, not by the type of odontoid dysplasia nor by subluxation. Posterior occipitocervical fusion proved to be an effective treatment.
我们对13例Morquio-Brailsford病患者在颈椎前屈和后伸位进行了高分辨率计算机颈椎脊髓造影。我们观察到：1）每例均存在齿状突发育异常，齿状突发育不全，远端部分分离，且不一定骨化；2）寰枢椎不稳定程度较轻，大多数病例无寰枢椎前脱位；3）严重脊髓受压时，是由于硬膜外前方软组织增厚所致；4）这种压迫不会因颈部屈伸而缓解，且在生命早期就有表现；5）枕颈后路融合术后，软组织增厚消失，齿状突随后发育正常。我们得出结论，颅颈交界区神经受累的严重程度取决于软组织变化，而非齿状突发育异常的类型或半脱位情况。枕颈后路融合术被证明是一种有效的治疗方法。
