title: Orthopedic management of the extremities in patients with Morquio A syndrome.
abstract: 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.
  
  
  OBJECTIVE
  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.
  
  
  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.
authors.affiliation: Department of Orthopaedic Surgery and Sports Medicine, Seattle Children's Hospital, University of Washington, Seattle, WA, USA, klane.white@seattlechildrens.org.
authors.forename: Klane K
authors.initials: KK
authors.lastname: White
authors.forename: Andrea
authors.initials: A
authors.lastname: Jester
authors.forename: C Edward
authors.initials: CE
authors.lastname: Bache
authors.forename: Paul R
authors.initials: PR
authors.lastname: Harmatz
authors.forename: Renée
authors.initials: R
authors.lastname: Shediac
authors.forename: Mihir M
authors.initials: MM
authors.lastname: Thacker
authors.forename: William G
authors.initials: WG
authors.lastname: Mackenzie