Orthopedic management of hemophilia.
Hemophilia is an X-linked inherited bleeding disorder characterized by a deficiency of clotting factor VIII or IX in the intrinsic pathway of the coagulation cascade. This disease can have a profound, debilitating effect on the musculoskeletal system through recurrent hemarthroses and intermuscular hematomas. Although medical management with replacement factor is often effective, patients with severe hemophilia may develop chronic synovitis and arthropathy best treated with more invasive means. Radioactive synovectomy performed at select hemophilia centers and arthroscopic or open synovectomy are helpful for chronic, active synovitis. Arthroplasty helps improve function and pain relief for most patients, but complication rates, particularly for infection, are higher than those for arthroplasty performed on patients without hemophilia.
Heyworth
Benton E
BE
Hospital for Special Surgery, 535 East 70th Street, New York, NY 10021, USA.
Su
Edwin P
EP
Figgie
Mark P
MP
Acharya
Suchitra S
SS
Sculco
Thomas P
TP
D000208:Acute Disease; D019643:Arthroplasty, Replacement / Q000009:adverse effects / Q000379:methods*; D001779:Blood Coagulation Factors / Q000627:therapeutic use*; D002908:Chronic Disease; D003131:Combined Modality Therapy; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006395:Hemarthrosis / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery*; D006467:Hemophilia A / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D007595:Joint Prosthesis; D008297:Male; D009985:Orthopedics / Q000379:methods; D018570:Risk Assessment; D012720:Severity of Illness Index; D013585:Synovitis / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D016896:Treatment Outcome
D001779:Blood Coagulation Factors