Optimal management of hemophilic arthropathy and hematomas

Abstract

Hemophilia is a hematological disorder characterized by a partial or complete deficiency of clotting factor VIII or IX. Its bleeding complications primarily affect the musculoskeletal system. Hemarthrosis is a major hemophilia-related complication, responsible for a particularly debilitating chronic arthropathy, in the long term. In addition to clotting factor concentrates, usually prescribed by the hematologist, managing acute hemarthrosis and chronic arthropathy requires a close collaboration between the orthopedic surgeon and physiotherapist. This collaboration, comprising a coagulation and musculoskeletal specialist, is key to effectively preventing hemarthrosis, managing acute joint bleeding episodes, assessing joint function, and actively treating chronic arthropathy. This paper reviews, from a practical point of view, the pathophysiology, clinical manifestations, and treatment of hemarthrosis and chronic hemophilia-induced arthropathy for hematologists, orthopedic surgeons, and physiotherapists.

Keywords: arthropathy; hemarthrosis; hematoma; hemophilia; physiotherapy; target joint.

Figure 1

Acute hemarthrosis of the right knee in a young boy with severe hemophilia A.

Figure 2

(A) Patient with severe arthropathy of the left knee. The clinical Gilbert score was calculated to be 8/12, comprising presence of extension lag, limited flexion, severe muscular atrophy, crepitus on motion, and instability. (B) The clinical score could not be calculated in the same patient at the time of an acute hemarthrosis.

Figure 3

Severe arthropathy of the right tibio-talar and subtalar joints, characterized by a plano-valgus deformation. Note: The presence of an important atrophy of the calf muscles of the right leg.

Figure 4

Pettersson radiological score (the maximum of 13 points representing most severe arthropathy) in a 27-year-old severe hemophilia A patient showing (A) a normal ankle (left) and a moderate arthropathy (right). (B) End-stage hemophilic arthropathy (avascular necrosis of the talar dome, multiple osteophytes, severe joint space narrowing, and natural fusion of the joint).

Figure 5

(A) Severe right knee arthropathy in a 32-year-old severe hemophilia A patient. It should be noted that the left knee never experienced any hemarthroses, and therefore presents no articular damage. (B) The same subject following TKR on the right side. Abbreviation: TKR, total knee replacement.