Musculoskeletal anomalies in children with Mucopolysaccaridoses
Introduction The accumulation of glycosaminoglycan (GAGs) in the tissues in Mucopolysaccharidoses (MPS) can lead to skeletal anomalies (DYSOSTOSIS MULTIPLEX) and to soft tissue impairments (neural or medullar compression, joint stiffness, tenosynovitis). Here is a review of orthopedic issues freque...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
| Published: |
Russian Ilizarov Scientific Center for Restorative Traumatology and Orthopaedics
2021-08-01
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| Series: | Гений oртопедии |
| Subjects: |
| Summary: | Introduction The accumulation of glycosaminoglycan (GAGs) in the tissues in Mucopolysaccharidoses (MPS) can lead to skeletal anomalies (DYSOSTOSIS
MULTIPLEX) and to soft tissue impairments (neural or medullar compression, joint stiffness, tenosynovitis). Here is a review of orthopedic issues frequently
encountered in patients with MPS. Material and methods Surgery may be justified at different age and according to the type of MPS. Different surgical
approaches and their indications are exposed in the article. Results The article exposes indications and techniques for orthopedic issues in MPS children:
cervical stenosis, cervical instability, kyphosis, hip dysplasia and hip dislocation, genu valgum. Conclusion Various musculoskeletal anomalies can be found
in patients with mucopolysaccharidoses. Neurological impairments are frequently seen due to cervical stenosis or instability and should be early detected with
regular MRI of the cervical spine. Well-codified management should lead to favorable functional results and maintain functional and walking abilities. |
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| ISSN: | 1028-4427 2542-131X |