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Orthopaedic Manifestations of Sickle Cell Disease

Vanderhave, Kelly L. MD; Perkins, Crystal A. MD; Scannell, Brian MD; Brighton, Brian K. MD

JAAOS - Journal of the American Academy of Orthopaedic Surgeons: February 1, 2018 - Volume 26 - Issue 3 - p 94-101
doi: 10.5435/JAAOS-D-16-00255
Review Article

Sickle cell disease (SCD) is an autosomal recessive disorder that results in hemolytic anemia related to abnormal hemoglobin and erythrocyte levels. SCD is characterized by vascular occlusive episodes, visceral sequestration, and aplastic or hemolytic crises. These crises most commonly occur in bone. The orthopaedic manifestations of SCD comprise much of the morbidity associated with this disorder. Osteonecrosis and osteomyelitis are among the most disabling and serious musculoskeletal complications in patients with SCD. Effective management of the bone and joint sequelae requires an accurate diagnosis, an understanding of the pathophysiology of the disease, and knowledge of available medical and surgical treatment alternatives. The major orthopaedic manifestations of SCD are osteonecrosis, osteomyelitis, septic arthritis, and bone infarction. Patients with SCD require close monitoring in the perioperative period because of the risk for vasoocclusive crisis.

From the Department of Orthopaedic Surgery, Carolinas Medical Center, Charlotte, NC (Dr. Vanderhave, Dr. Scannell, and Dr. Brighton), and Children’s Healthcare of Atlanta, Atlanta, GA (Dr. Perkins).

Dr. Brighton or an immediate family member serves as a paid consultant to or is an employee of DePuy Synthes and serves as a board member, owner, officer, or committee member of the American College of Surgeons and the Pediatric Orthopaedic Society of North America. None of the following authors or any immediate family member has received anything of value from or has stock or stock options held in a commercial company or institution related directly or indirectly to the subject of this article: Dr. Vanderhave, Dr. Perkins, and Dr. Scannell.

Copyright 2018 by the American Academy of Orthopaedic Surgeons.
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