European Journal of Pediatrics

, Volume 154, Issue 7, pp 504-512

First online:

Craniovertebral junction anomalies in inherited disorders: Part of the syndrome or caused by the disorder?

  • H. Alan CrockardAffiliated withDepartment of Surgical Neurology, The National Hospital for Neurology and Neurosurgery
  • , John M. StevensAffiliated withDepartment of Radiology, The National Hospital for Neurology and Neurosurgery

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Patterns of skeletal abnormality at the craniovertebral junction in the normal population and in syndromes such as Down, Morquio etc, are compared and the recent embryological data and comparative anatomy reviewed. The authors' view based on their own clinical and radiological experience is that the osodontoideum is the product of excessive movement at the time of ossification of the cartilaginous dens and is exactly analogous to the unfused Type II odontoid fracture. True hypoplasia of the odontoid peg is part of a wider segmentation defect associated with Klippel Feil, occipitalised atlas and/or basilar invagination; it is hardly every associated with instability.

Key words

Craniocervical junction Atlanto-axial subluxation Basilar invagination Down syndrome Morquio disease