Dysplasia of the odontoid process in Morquio's syndrome causing quadriparesis

Eleven patients with documented Morquio's syndrome who had dysplasia of the odontoid process and resulting atlanto-axial instability were reviewed. They were found to be at risk for acute traumatic quadriparesis, chronic myelopathy of a variable and often rapid rate of progression, and sudden death by respiratory arrest. The evidence suggests that early prophylactic posterior cervical fusion is indicated for patients with this malformation and that once quadriparesis is established, recovery of function is limited.