Abstract
Primary osteonecrosis of the hip-knee, Legg-Calve-Perthes disease (pediatric osteonecrosis), and chronic alveolar osteonecrosis of the jaws are associated with pathoetiologic, anticoagulant treatable, familial thrombophilia and hypofibrinolysis. The Factor V Leiden mutation is the most common familial procoagulant associated with osteonecrosis of the hip-knee, Legg-Calve Perthes disease, and alveolar osteonecrosis of the jaw. The following familial thrombophilias, beyond Factor V Leiden heterozygosity, associated with osteonecrosis include the G20210A Prothrombin gene, C677T MTHFR homozygosity, high Factors VIII, XI, high anticardiolipin antibodies IgM and IgG, high homocysteine, low proteins C, S, antithrombin III, and the lupus anticoagulant. Familial hypofibrinolysis associated with osteonecrosis includes 4G4G homozygosity of the Plasminogen Activator Inhibitor gene, and high lipoprotein (a). In primary osteonecrosis of the hip and knee associated with familial thrombophilia-hypofibrinolysis, provided anticoagulation (Enoxaparin or Apixaban-Rivaroxaban) is started at Ficat Stages I-II before hip/knee joint collapse, pain is relieved, and joint collapse prevented, avoiding the necessity for joint replacement surgery. In chronic alveolar osteonecrosis of the jaws with recalcitrant pain, anticoagulation with Warfarin (before Apixaban-Rivaroxaban were available) relieved pain in 60% of cases, and Apixaban-Rivaroxaban entirely resolved jaw pain. In Legg-Calve-Perthes disease, there are multiple bracing and surgical procedures designed to optimize hip function and to prevent the necessity for total hip replacement in young adulthood. In early osteonecrosis in adults, Ficat stages I-II, there are multiple surgical approaches to osteonecrosis designed to prevent collapse requiring total hip or knee replacement. We review below primary osteonecrosis of the hip-knee, Legg-Calve-Perthes disease, and the jaws. We review the success of anticoagulant treatment, relieving pain in chronic alveolar osteonecrosis, and relieving pain and preventing hip-knee joint collapse, starting in early hip-knee disease, at Ficat Stages I-II.