Os odontoideum and craniovertebral junction instability secondary to dystonia: case series and review of the literature.

Abstract

Os odontoideum refers to a rounded ossicle detached from a hypoplastic odontoid process at the body of the axis. The aetiology has been debated and believed to be either congenital or acquired (resulting from trauma). Os odontoideum results in incompetence of the transverse ligament and thus predisposes to atlantoaxial instability and spinal cord injury. Three cases of children with severe dystonic cerebral palsy presenting with myelopathic deterioration secondary to atlantoaxial instability due to os odontoideum are presented. This observation supports the hypothesis of os odontoideum being an acquired phenomenon, secondary to chronic excessive movement with damage to the developing odontoid process. In children with cerebral palsy and dystonia, pre-existing motor deficits may conceal an evolving myelopathy and result in delayed diagnosis of clinically significant atlantoaxial subluxation.