Prolonged ischemic priapism in an adolescent with sickle cell anemia: Challenges of management

Abstract

Introduction: Persistent penile erection lasting more than 4 hours and unrelated to sexual interest or stimulation is called priapism. Priapism is uncommon in children. Of the three widely accepted types: ischemic, non-ischemic, and stuttering priapism; ischemic priapism is the predominant type seen in children. A common cause of ischemic priapism in children is sickle cell disease. Ischemic priapism of prolonged duration is associated with a higher risk of complications especially irreversible loss of erectile function. It is a difficult clinical condition to manage and there are no established guidelines for its management in children. Adequate surgical treatment does not guarantee against the risk of devastating longterm sequelae. Case Report: We report a case of a 12-year-old boy with sickle cell anemia who presented after 48 hours of sustained painful penile erection. He required multiple surgical shunting procedures including distal corporoglanular shunt with tunneling of both corpora cavernosa with Hegar’s dilator to achieve detumescence. The management challenges encountered are highlighted. Conclusion: A potential role for anticoagulation and the use of low-dose hydroxyurea in the prevention of recurrent ischemic priapism in children with sickle cell anemia are advocated.