Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Orthop. Jun 18, 2016; 7(6): 370-375
Published online Jun 18, 2016. doi: 10.5312/wjo.v7.i6.370
Orthopedic disorders of the knee in hemophilia: A current concept review
E Carlos Rodriguez-Merchan, Leonard A Valentino
E Carlos Rodriguez-Merchan, Department of Orthopedic Surgery, La Paz University Hospital-IdiPaz, 28046 Madrid, Spain
Leonard A Valentino, Rush University Medical Center, Chicago, IL 60612, United States
Author contributions: Rodriguez-Merchan EC and Valentino LA wrote the article and reviewed the literature.
Conflict-of-interest statement: The authors declare no conflicts of interest for this article.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: E Carlos Rodriguez-Merchan, MD, PhD, Department of Orthopedic Surgery, La Paz University Hospital-IdiPaz, Paseo de la Castellana 261, 28046 Madrid, Spain.
Telephone: +34-91-5712871 Fax: +34-91-5712871
Received: January 8, 2016
Peer-review started: January 12, 2016
First decision: March 1, 2016
Revised: April 7, 2016
Accepted: April 21, 2016
Article in press: April 22, 2016
Published online: June 18, 2016

The knee is frequently affected by severe orthopedic changes known as hemophilic arthropathy (HA) in patients with deficiency of coagulation factor VIII or IX and thus this manuscript seeks to present a current perspective of the role of the orthopedic surgeon in the management of these problems. Lifelong factor replacement therapy (FRT) is optimal to prevent HA, however adherence to this regerous treatment is challenging leading to breakthrough bleeding. In patients with chronic hemophilic synovitis, the prelude to HA, radiosynovectomy (RS) is the optimal to ameliorate bleeding. Surgery in people with hemophilia (PWH) is associated with a high risk of bleeding and infection, and must be performed with FRT. A coordinated effort including orthopedic surgeons, hematologists, physical medicine and rehabilitation physicians, physiotherapists and other team members is key to optimal outcomes. Ideally, orthopedic procedures should be performed in specialized hospitals with experienced teams. Until we are able to prevent orthopedic problems of the knee in PWH will have to continue performing orthopedic procedures (arthrocentesis, RS, arthroscopic synovectomy, hamstring release, arthroscopic debridement, alignment osteotomy, and total knee arthroplasty). By using the aforementioned procedures, the quality of life of PWH will be improved.

Keywords: Hemophilia, Knee, Orthopedic problems, Prevention, Surgical treatment

Core tip: Hemophilia is an inherited bleeding disorder due to deficiency of factor VIII (hemophilia A) or factor IX (hemophilia B) resulting in insufficient thrombin generation leading to recurrent intra-articular hemorrhages (hemarthroses). Prevention of hemarthroses with intravenous infusions of the deficient protein from infancy to adulthood (primary prophylaxis) should be considered to achieve optimal outcomes. If factor replacement therapy (FRT) is insufficient, or if patients are not adherent to the prescribed regimen, recurrent hemarthroses results in chondrocyte apoptosis (cartilage degeneration) and hypertrophy of the synovium (synovitis). Many surgical interventions are available for the knee joint. For example, to treat synovitis recalcitrant to FRT, there are two primary orthopedic modalities: Radiosynovectomy and arthroscopic synovectomy. This article reviews the pathogenesis, diagnosis and treatment of hemophilic arthropathy of the knee.