Priapism in children: review of pathophysiology and treatment.

OBJECTIVE: Priapism may cause serious sequelae concerning the future sex life of the patient, as it can determine impotence, erectile dysfunction or psychogenic sexual aversion. It is a common symptom of sickle cell disease in children and adolescents. There are few good quality evidence manuscripts about the problem in current medical literature. SOURCES: Literature review on the databases MEDLINE and LILACS covering the period from 1966 to 2008. SUMMARY OF THE
FINDINGS: The basis for the treatment of low flow priapism includes treating sickle cell disease and the usage of intracavernous adrenergic agents as necessary. Surgery is indicated in a minority of cases. The treatment of pediatric cases demands dose adjustments, adequate drug choice and sedoanalgesia to cover procedures involving pain or trauma.
CONCLUSIONS: A new physiopathologic theory concerning sickle cell disease, which questions the traditional vascular blockage mechanisms by deformed red cells and proposes that endothelial inflammatory activation is the main cause of clinical problems, allows to propose new therapeutic maneuvers to solve sickle cell priapism. The absence of good quality evidence to treat sickle cell priapism suggests the necessity to conduct good prospective multicenter protocols to investigate the condition.