Priapism

Priapism occurs in two-thirds of males with SCD, with a peak incidence in the second and third decades. It is caused by vaso-occlusion leading to obstruction of venous drainage from the penis. It typically affects the corpora cavernosa alone, resulting in a hard penis with a soft glans. Episodes can be brief (stuttering) or prolonged, when they last for longer than 3 h. Recurrent priapism leads to fibrosis and eventual impotence. Young boys require explanation of symptoms and the need to seek early help for priapism. At the onset of priapism, patients should drink extra fluids and attempt to urinate. Persistent priapism requires intravenous hydration and analgesia. Partial exchange transfusion, aspiration of the cavernosa or the creation of fistula between glans and corpora cavernosa (Winter procedure) are performed for resistant cases. Agents used to prevent recurrences of priapism include etilefrine, gonadotropin-releasing hormone analogues, stilbestrol and pseudoephedrine.

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