Orthopedic management of the extremities in patients with Morquio A syndrome


BACKGROUND Musculoskeletal involvement in Morquio A syndrome (mucopolysaccharidosis IVA; MPS IVA) contributes significantly to morbidity and mortality. While the spinal manifestations of the disorder have received considerable attention in the literature, there have been few reported studies to date to guide the management of the orthopedic problems associated with the lower and upper extremities. PURPOSE The objective was to develop recommendations for the management of the extremities in patients with Morquio A syndrome. METHODS A group of specialists in orthopedics, pediatrics and genetics with experience in the management of Morquio A patients convened to review and discuss current clinical practices and to develop preliminary recommendations. Evidence from the literature was retrieved. Recommendations were further refined until consensus was reached. RESULTS AND CONCLUSIONS This present article provides a detailed review and discussion of the lower and upper extremity deformities in Morquio A syndrome and presents recommendations for the assessment and treatment of these complications. Key issues, including the importance of early diagnosis and the implications of medical therapy, are also addressed. The recommendations herein represent an attempt to develop a uniform and practical approach to managing patients with Morquio A syndrome and improving their outcomes.

DOI: 10.1007/s11832-014-0601-4

Extracted Key Phrases

6 Figures and Tables

Cite this paper

@inproceedings{White2014OrthopedicMO, title={Orthopedic management of the extremities in patients with Morquio A syndrome}, author={Klane K White and Andrea Jester and Christopher Edward Bache and Paul R. Harmatz and Ren{\'e}e Shediac and Mihir M. Thacker and William G. Mackenzie}, booktitle={Journal of children's orthopaedics}, year={2014} }