Musculo-Skeletal Complications of Hemophilia

Abstract

The most important clinical strategy for management of patients with hemophilia is the avoidance of recurrent hemarthroses by means of continuous, intravenous hematological prophylaxis. When only intravenous ondemand hematological treatment is available, frequent evaluations are necessary for the early diagnosis and treatment of episodes of intra-articular bleeding. The natural history of the disease in patients with poorly controlled intra-articular bleeding is the development of chronic synovitis and, later, multi-articular hemophilic arthropathy. Once arthropathy develops, the functional prognosis is poor. Treatment of these patients should be conducted through a comprehensive program by a multidisciplinary hemophilia unit. Although continuous prophylaxis can avoid the development of the orthopedic complications of hemophilia still seen in the twenty-first century, such a goal has not, so far, been achieved even in developed countries. Therefore, many different surgical procedures such as arthrocentesis, radiosynoviorthesis (radiosynovectomy) (yttrium-90, rhenium-186), tendon lengthenings, alignment osteotomies, joint arthroplasties, removal of pseudotumours, and fixation of fractures are still frequently needed in the care of these patients.

Pain assessment and management in haemophilia: A survey among Italian patients and specialist physicians

“…Despite such clinical relevance, literature and research interest in pain management were quite limited in this setting until recent years. Indeed, the general improvement of comprehensive haemophilia care and the increasing attention of HTC specialists to gain patients' quality of life by multidisciplinary approaches led to better address pain assessment and management in PWH 5,[20][21][22][23][24]. More than describing characteristics of pain and its determinants or consequences, as reported in several studies,5,8,14,15 our survey was focused on current approaches to assess and manage pain by Italian HTC physicians, in comparison to what is experienced and perceived by PWH.The first important information retrieved indicates that patients' perception of pain is quite different from that of physicians.…”

Compartment Syndrome, Femoral Nerve Lesion and Recurrent Haemarthrosis in one and the same Patient after Total Knee Arthroplasty

“…Incidence of permanent neurologic injury, as manifested by weak quadriceps power, diminished sensation, and dysesthesia, is estimated to be 0.2% (Feibel et al, 2009). Femoral neuropathy can be caused by iliacus hematoma and is a well-known complication in hemophiliacs or in patients undergoing anticoagulant therapy (Puechal et al, 1992, Rodriguez-Merchan, 2012. In patients without coagulation disturbances, however, femoral neuropathy caused by iliacus hematoma rarely occurs, the cause is usually traumatism instead (Puechal et al, 1992).…”