Priapism

Background
Priapism is a sustained, unwanted painful erection lasting 4 or more hours. Stuttering priapism is the occurrence of multiple self-limited episodes of shorter duration (<4 hours) and can be a harbinger of sustained events. Priapism is a common complication of SCD, affecting 35 percent of boys and men. It is usually of the low­ flow ischemic type and characterized by pain and a soft glans. Blood aspirated from the corpora cavernosa of the penis is dark, with a low pO2, pH, and glucose concentration. Prompt recognition of priapism and initiation of conservative medical management may lead to detumescence and limit the need for more aggressive and invasive intervention. Delayed diagnosis and therapy can result in impotence. Summary of the Evidence Seven observational studies and 39 case reports described priapism in the setting of SCD.

Overall, the quality of the evidence in this area was low due to the observational and uncontrolled design of the available studies. The observational studies included more than 220 people and studied approaches such as shunts, aspiration, exchange transfusion, hydroxyurea, hormonal therapy (e.g., stilbestrol, finasteride, and leuprolide), bicalutamide, hydralazine, sildenafil, oxygen, and hyperhydration to treat priapism in men and boys with SCD. Results were limited, reporting variable success. Several of the studies highlighted the importance of prompt recognition and initial conservative medical management with analgesics, intravenous fluids, oxygen, and sedation if needed. Red blood cell transfusion therapy was inconsistently associated with improvement in acute priapism. In addition, case reports of acute neurological events following exchange transfusion for priapism further limit enthusiasm for routine adoption of this therapy in the absence of proven benefit. Both observational studies and case reports found that a variety of subsequent interventions used to treat symptoms that persist after initial conservative medical management appear to result in detumescence and retained potency. These include penile aspiration, corporal irrigation using a-adrenergic agents (e.g., pseudoephedrine, epinephrine, etilefrine), and the use of oral agents (e.g., PDE-5 inhibitors, pseudo-ephedrine). Surgical intervention, including shunting, has been utilized most often after more conservative measures fail, with inconsistent benefit. In developing recommendations for the care of males with SCD presenting with acute priapism, the expert panel placed great value on preventing pain and future long-term sequelae.

Recommendations For an episode of priapism lasting 4 hours or longer, initiate interventions to include vigorous oral or intravenous hydration and oral or intravenous analgesia (Strong Recommendation, Low-Quality Evidence); and consultation with an urologist who can perform further evaluation and intervention for symptoms which do not remit with initial conservative medical management. (Consensus-Panel Expertise) Do not use transfusion therapy for immediate treatment of priapism associated with SCD. (Moderate Recommendation, Low-Quality Evidence) Consult with a hematologist for possible preoperative transfusion if surgical intervention is required. (Consensus-Panel Expertise)

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