Efficacy of intracavernous etilefrin injections in curative treatment of priapism in sickle-cell patients

OBJECTIVE: To date, the treatment of priapism in sickle-cell patients has relied an measures aimed at lowering blood viscosity and acidosis and reducing the level of circulating hemoglobin S (hyperhydration, alkalinization, or exsanguinotransfusion...) Surgical cavernous-venous shunt may be proposed if conservative treatment fails. We examined the efficacy of intracavernous etilefrin injections. PATIENTS AND METHODS: From January 1996 through October 1997 (20 months) we performed 35 consecutive intracavernous injections of an alphastimulant, etilefrin in 7 sickle-cell patients (6 SS, 1 AS) who had experienced one or several episodes of low-flow priapism lasting 2 to 28 hours. RESULTS: Involution of the tumefaction was rapidly achieved in all cases. Tolerance was satisfactory, although some post-injection undesirable effects were reported by certain patients: moderate transient pain (2-5 min) in the retrosternal area, or intense pain in the penis (more intense than the priapism) which lasted 10 to 30 minutes. This work confirmed the earlier reported efficacy of intracavernous injections of etilefrin and suggests that the autonomous nervous system plays an important role in the genesis of this condition in sickle-c:ell patients. Patients should be informed about the observed undesirable effects which have not been reported previously in the literature. CONCLUSION: Etilefrin can be proposed as first line treatment for priapism in sickle-cell patients (at least in cases lasting less than 24 h). The pathogenic mechanism could involve neuromuscular dysfunction.