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ATLANTO-AXIAL SUBLUXATION IN THE MORQUIO SYNDROME

Report of a Case

Peter Beighton 1; and James Craig 2

1 Department of Orthopaedic Surgery, University of Witwatersrand, Medical School, Johannesburg; Human Genetics, University of Cape Town
2 Department of Orthopaedic Surgery, University of Witwatersrand, Medical School, Johannesburg; South Africa

1. The case is described of an eight-year-old girl with the Morquio syndrome (mucopolysaccharidosis type IV) who died from acute tetraplegia, due to atlanto-axial subluxation which occurred during general anaesthesia.

2. Hypoplasia of the dens of the axis vertebra entails a high risk of vertebral dislocation and spinal cord damage especially in circumstances such as general anaesthesia when excessive movements of the head may occur.
(c) British Editorial Society of Bone and Joint Surgery All Rights Reserved
Registered charity no: 209299     Print ISSN: 0301-620X
Hip, Knee, Trauma, Upper limb, Foot & Ankle, Paediatrics, Oncology, Spine, Arthroplasty, General