Abstract
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.
Highlights
The accumulation of glycosaminoglycan (GAGs) in the tissues in Mucopolysaccharidoses (MPS) can lead to skeletal anomalies (DYSOSTOSIS MULTIPLEX) and to soft tissue impairments
The osteoarticular system is always involved in Mucopolysaccaridoses (MPS)
Various bone problems in MPS are explained by abnormal enchondral ossification (*) and intramembranous ossification (**) (Table 2) [2, 4]. These skeletal anomalies are called DYSOSTOSIS MULTIPLEX and they are found in the skull, trunk, spine, limbs, hands and pelvis
Summary
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). 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
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have