Dens Growth After Surgical Fixation in Children with Morquio-Brailsford Disease.

Abstract

Mucopolysaccharidosis type IVA is a lysosomal storage disorder caused by a deficiency of the enzyme N-acetyl-galactosamine-6-sulphate sulphatase. Mucopolysaccharidosis type IVA is multisystemic disease with significant spinal involvement and atlantoaxial instability leading to neural compression and significant morbidity. Dens hypoplasia is a common feature of this condition. In this study we demonstrate that after spinal fixation, there is new growth of dens in significant proportion of patients, suggesting atlantoaxial instability as one of the major driving forces of lack of development of dens in this condition.