[Sickle cell disease priapism: treatment with intracavernous injections of etilefrine].

Intracavernous injection (ICI) of adrenergic agonists has recently been proposed for treatment of priapism associated with sickle cell hemoglobinopathy and appears to be effective in cases less than 28 hours old. The purpose of this study was to confirm the usefulness of this technique in a large series of patients. From January 1996 through September 1998, 19 patients with sickle cell disease were treated by ICI of etilefrine for one or several episodes of low-flow priapism lasting between 2 hours and 15 days. A total of 72 consecutive ICI were performed alone in patients with priapism less than 6 hours old or in combination with prior drainage in patients with priapism more than 6 hours old. Results depended mainly on the delay to treatment. Detumescence was achieved in all 10 cases treated within 29 hours as opposed to only 2 of 9 cases treated beyond 29 hours. This finding is in agreement with experimental findings demonstrating histological evidence of necrosis of endothelial cells and cavernous smooth muscle fibers after 24 hours. However based on our experience showing that good results with preservation of erectile function can be achieved after delays longer than 40 hours (42 hours and 5 days in two of our patients), we recommend that treatment by ICI be tried before surgery in sickle cell anemia patients even with long-standing priapism. Untoward side effects including intense pain in the penis and retrosternal pain were observed in 4 patients. ICI of adrenergic agonists is one of the best treatment currently available for management of priapism in patients with sickle cell disease. Surgery should be used only after failure of ICI.