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Orthopedic management of mucopolysaccharide disease

Issue title: Promoting Bone Health in MPS VI (Maroteaux-Lamy Syndrome): Framing New Therapies, Part 1

Article type: Research Article

Authors: White, Klane K.; | Harmatz, Paul

Affiliations: Seattle Children's Hospital, Department of Orthopedic Surgery, Seattle, WA, USA | University of Washington, Department of Orthopedics and Sports Medicine, Seattle, WA, USA | Children's Hospital & Research Center Oakland, Oakland, CA, USA

Note: [] Address for correspondence: K.K. White, 4800 Sand Point Way, W-7706, Seattle, WA 98105, USA. Tel.: +1 206 987 5678; Fax: +1 206 987 3852; E-mail: klane.white@seattlechildrens.org

Keywords: Mucopolysaccharidosis, orthopedics, spine, hip, knee

DOI: 10.3233/PRM-2010-0102

Journal: Journal of Pediatric Rehabilitation Medicine, vol. 3, no. 1, pp. 47-56, 2010

Received 21 January 2010
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Accepted 21 January 2010
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Published: 2010
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Abstract

With advances in the treatment of the mucopolysaccharidosis (MPS) disorders, musculoskeletal problems are increasingly becoming a focus of care for these patients. This review discusses the current understanding of the pathophysiology of musculoskeletal disease in MPS and its orthopedic management. Deformities of the spine, hips and extremities are common and often functionally limiting. Carpal tunnel syndrome and flexor tendon triggering are common. Surgical intervention is often required to optimize long-term function.

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