Dens growth following surgical fixation in children with

World Neurosurg · May 2024

Dens growth following surgical fixation in children with Morquio-Brailsford disease.

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.

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- Fardad T Afshari, Desiderio Rodrigues, and Guirish A Solanki.
- Department of Neurosurgery, Birmingham Children's Hospital, Birmingham, England. Electronic address: afsharifardad@googlemail.com.
- World Neurosurg. 2024 May 1; 185: 899089-90.
AbstractMucopolysaccharidosis 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.Copyright © 2024 Elsevier Inc. All rights reserved.

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Dens growth following surgical fixation in children with Morquio-Brailsford disease.

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