Abstract
Mucopolysaccharidosis IVA (MPS IV), is a lysosomal storage disorder caused by a deficiency of the enzyme N-acetyl-galactosamine-6-sulphate sulphatase. MPS 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 following 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.
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