Clinical findings in 12 patients with MPS IV A (Morquio's disease). Further evidence for heterogeneity. Part III: Odontoid dysplasia.
Odontoid dysplasia in the absence or presence of atlanto-axial instability was found in all cases. In general, this correlated well with the overall clinical severity of the condition and illustrates the importance of careful follow-up of these patients. Symptoms or signs of cervical cord compression merit further investigation. Proven compromise of the upper cervical cord is an indication for posterior fusion of the upper cervical spine.
D001268:Atlanto-Axial Joint / Q000000981:diagnostic imaging / Q000473:pathology; D001368:Axis, Cervical Vertebra / Q000002:abnormalities*; D005500:Follow-Up Studies; D014644:Genetic Variation; D006801:Humans; D009085:Mucopolysaccharidosis IV / Q000235:genetics* / Q000473:pathology; D009809:Odontoid Process / Q000002:abnormalities*; D011859:Radiography; D013117:Spinal Cord Compression / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology