| Abstrakt: |
The article focuses on priapism in children with sickle cell anemia. The painful symptoms of sickle cell disease result from local tissue anoxia secondary to mechanical occlusion of the vascular system by a log jam by the sickled erythrocytes. It is unfortunate that the misnomer thrombotic crisis has been applied to this situation, because there is no clot or thrombus formation with conversion of fibrinogen to fibrin. Priapism in patients with sickle cell anemia has been treated unsuccessfully with anticoagulants, dextran, fibrinolytic agents, stilbestrol, ice water enemas, ice packs, hot packs, spinal and caudal anesthesia and vasodilators. Aspiration of the corpus cavernosum with a large bore needle, along with irrigation with heparin, hyaluronidase, fibrinolysin or procaine, is usually followed by rapid reaccumulation, in spite of a pressure dressing. Because the tissues are engorged and thus relatively ischemic, infection and penile sloughing are complications of aspiration or incision and drainage. None of these treatments attack the fundamental disturbance, which is a mechanical obstruction of the corpus cavemosum with sickled erythrocytes. Transfusions as therapy have been previously suggested, but are not reported as being efficacious. In retrospect, whole blood or relatively small amounts of packed erythrocytes were infused. |
