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: |
| _version_ | 1818692886659596288 |
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| author | Florence Müller Khaled Alomar Pierre Journeau |
| author_facet | Florence Müller Khaled Alomar Pierre Journeau |
| author_sort | Florence Müller |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-17T13:04:55Z |
| format | Article |
| id | doaj.art-5066ea6f0692438a9cfb03aea7352d70 |
| institution | Directory Open Access Journal |
| issn | 1028-4427 2542-131X |
| language | English |
| last_indexed | 2024-12-17T13:04:55Z |
| publishDate | 2021-08-01 |
| publisher | Russian Ilizarov Scientific Center for Restorative Traumatology and Orthopaedics |
| record_format | Article |
| series | Гений oртопедии |
| spelling | doaj.art-5066ea6f0692438a9cfb03aea7352d702022-12-21T21:47:17ZengRussian Ilizarov Scientific Center for Restorative Traumatology and OrthopaedicsГений oртопедии1028-44272542-131X2021-08-0127444644910.18019/1028-4427-2021-27-4-446-449Musculoskeletal anomalies in children with MucopolysaccaridosesFlorence Müller0Khaled Alomar1Pierre Journeau2CHU de Nancy, Hôpital d’enfants de Brabois, Nancy, FranceCHU de Nancy, Hôpital d’enfants de Brabois, Nancy, FranceCHU de Nancy, Hôpital d’enfants de Brabois, Nancy, FranceIntroduction 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.mucopolysaccharidosesmusculoskeletal anomaliesorthopedic surgery |
| spellingShingle | Florence Müller Khaled Alomar Pierre Journeau Musculoskeletal anomalies in children with Mucopolysaccaridoses Гений oртопедии mucopolysaccharidoses musculoskeletal anomalies orthopedic surgery |
| title | Musculoskeletal anomalies in children with Mucopolysaccaridoses |
| title_full | Musculoskeletal anomalies in children with Mucopolysaccaridoses |
| title_fullStr | Musculoskeletal anomalies in children with Mucopolysaccaridoses |
| title_full_unstemmed | Musculoskeletal anomalies in children with Mucopolysaccaridoses |
| title_short | Musculoskeletal anomalies in children with Mucopolysaccaridoses |
| title_sort | musculoskeletal anomalies in children with mucopolysaccaridoses |
| topic | mucopolysaccharidoses musculoskeletal anomalies orthopedic surgery |
| work_keys_str_mv | AT florencemuller musculoskeletalanomaliesinchildrenwithmucopolysaccaridoses AT khaledalomar musculoskeletalanomaliesinchildrenwithmucopolysaccaridoses AT pierrejourneau musculoskeletalanomaliesinchildrenwithmucopolysaccaridoses |