Treatment of priapism in pediatric patients with sickle cell disease.

PURPOSE: The available treatment options for priapism in pediatric (1-21 years of age) patients with sickle cell disease (SCD) are reviewed.
SUMMARY: Priapism is a complication of SCD that receives little attention, yet it is a particularly bothersome issue for many pediatric patients. Numerous therapeutic options have been attempted, including diethylstilbestrol, gonadotropin-releasing hormone analogues, various adrenergic agonists, and hydroxyurea. Few agents have actually been examined in a controlled clinical trial, making it difficult for practitioners to treat this complication. It is our recommendation that treatment should be conservative initially, with the patient being encouraged to urinate, exercise, increase his fluid intake, and take oral analgesics. If the episode of priapism persists beyond 2 hours, the patient should report to the emergency department for i.v. hydration and analgesics. If the episode persists beyond 4 hours, intracavernosal aspiration and instillation of an alpha-agonist should be performed and repeated as needed. If the priapism remains for longer than 12 hours, surgery should be considered for shunt placement.
CONCLUSION: There is little evidence available regarding the definitive treatment of priapism, which affects a large number of pediatric patients with SCD. It is quite clear that more research is needed to determine what the best treatment options are for patients with this condition. Health care providers should educate their patients with SCD about the condition. Education, combined with more research of treatment options, may help patients avoid the damaging social, psychological, and medical implications of this bothersome and often embarrassing complication of SCD.