Priapism in children with sickle cell disease

A review of hospital admissions during 80 months revealed only 8 patients with episodes of priapism of approximately 400 pediatric male patients with sickle cell disease. The patients, who ranged in age from 5 to 19 years, underwent a 99mtechnetium penile scan, and 4 had a low and 4 had a high flow scan. Three cases resolved with hydration alone. Five patients received exchange transfusion of whom 3 subsequently underwent shunt procedures. One patient with a 5-year history of recurrent stuttering episodes was placed on transfusion therapy for 6 months and stuttering episodes have not recurred. One patient had a cerebrovascular accident 1 day after hospital discharge and another had priapism while on chronic transfusion therapy for a cerebrovascular accident. Each postpubertal patient had a severe clinical course; 1 had temporary impotence for 3 months and another had impotence at 2 weeks but was lost to followup. While 99mtechnetium penile scans may help clarify the severity of vascular stagnation, in our small group they were not helpful in predicting clinical course.