Advertisement for orthosearch.org.uk
You currently have no access to view or download this content. Please log in with your institutional or personal account if you should have access to through either of these
The Bone & Joint Journal Logo

Receive monthly Table of Contents alerts from The Bone & Joint Journal

Comprehensive article alerts can be set up and managed through your account settings

View my account settings

Children's Orthopaedics

OCCIPITO-ATLANTO-AXIAL FUSION IN MORQUIO-BRAILSFORD SYNDROME

A TEN-YEAR EXPERIENCE



Download PDF

Abstract

In 17 patients (eleven males, six females) with Morquio-Brailsford syndrome (mucopolysaccharidosis IV) we have used onlay femoral and tibial autografts placed posteriorly and secured to the laminae of C1 and C2 to obtain satisfactory occipito-C1/C2 posterior fusion. They were immobilised postoperatively in a halo-plaster body jacket for four months. The age at operation varied between three and 28 years. Those with myelopathic symptoms of recent onset made some recovery, but severely myelopathic patients showed little or no recovery.

We advise prophylactic occipitocervical fusion in these patients since the cartilaginous dens is not strong enough to ensure atlanto-axial mechanical stability.


Correspondence should be sent to Mr A. O. Ransford.

For access options please click here