As open synovectomy has

been effective in controlling synovitis and recurrent bleeding, it requires a large incision, prolonged VX-809 order hospitalization, protocolized rehabilitation, and large amount of factor replacement, and has been associated with a infection rate and reduced range of motion [10,11,12]. Long-term results on joint bleeding control and on overall joint function were satisfactory with these traditional techniques. Arthroscopic synovectomy represents a less invasive approach, with a low complication rate [13], and it has become the preferred alternative to the open technique. The use of arthroscopic synovectomy in persons with haemophilia was first attempted on knees and reported in 1983 [14,15], producing satisfactory results even after a prolonged follow-up with a significant reduction of joint bleeding recurrence and preservation of joint mobility [16]. This procedure, Selleck Tyrosine Kinase Inhibitor Library mainly performed in children or adolescents, allowed a significant reduction of bleeding rate and pain relief, suggesting that the beneficial effects of this surgery are greater if it is performed before the onset of severe radiological changes [17]. Additional data has been published suggesting that arthroscopic synovectomy is a cost-effective

means of addressing target joint bleeding [18]. Associated musculoskeletal disorders (i.e. flat foot, axial deviation of lower limbs) have to be treated as soon as possible in order to reduce the likelihood of secondary joint disease. Orthopaedic surgeons have to deal with two challenges:

the management of haemophilic paediatric patients coming from countries where factor replacement therapy is not available and patients with inhibitors. Cases of severe arthropathy with severe joint involvement that we commonly encountered many years ago are now seen Pomalidomide supplier only in such patients. Patients with inhibitors have more severe and incapacitating degrees of arthropathy than those without [19], with fixed knee flexion deformity as a common problem. If conservative treatment fails, surgical procedures have to be considered, including supracondylar femoral extension osteotomy, joint distraction, posterior capsulotomy and arthroscopic release. Potential complications of these procedures are fractures, neurovascular lesions, knee instability, and recurrent deformity with continued growth [20]. In patients with an immature skeleton, anterior femoral stapling is a less invasive method to treat fixed knee flexion deformity, is well tolerated, and provides an excellent alternative to osteotomy by allowing gradual correction through growth manipulation [21]. Joint replacement surgery, as last resort, could be performed in such patients when marked joint destruction is present and pain or deformity compromises function. Relief of pain, reduction of the deformity, and dramatic improvement in functional status and quality of life can be achieved in most patients.