What is the management for a patient with a history of sickle cell disease presenting with painful erection (priapism)?

Management of Painful Erection in Sickle Cell Disease

Immediately initiate urologic intervention with intracavernosal phenylephrine and corporal aspiration—do not delay treatment for systemic sickle cell therapies, as this is a compartment syndrome requiring direct penile intervention to prevent permanent erectile dysfunction. 1, 2

Emergency Recognition and Diagnosis

This is acute ischemic priapism, a true urologic emergency requiring intervention within hours to preserve erectile function. 1, 3

Critical Diagnostic Steps

• Obtain corporal blood gas immediately to confirm ischemic priapism: expect PO₂ <30 mmHg, PCO₂ >60 mmHg, and pH <7.25. 1, 2, 4
• Do not delay treatment to obtain blood gas if clinical presentation is clear (rigid corpora cavernosa with soft glans, duration >4 hours, painful erection). 2, 3
• The rigid penis with normal scrotum is pathognomonic for acute ischemic priapism in sickle cell patients. 2

What NOT to Do

• Never use systemic sickle cell treatment alone (hydration, exchange transfusion) as primary therapy—this guarantees erectile dysfunction by delaying definitive urologic intervention. 1, 2
• Do not waste time with penile duplex Doppler ultrasound when diagnosis is clinically obvious. 2, 3
• Exchange transfusion takes 6+ hours to prepare and shows no benefit in terminating priapism faster than natural history. 1

Stepwise Treatment Algorithm

Step 1: Corporal Aspiration (First-Line)

• Insert 19 or 21 gauge needle into corpus cavernosum and aspirate blood. 2
• Success rate: 24-36% with aspiration/irrigation alone. 2, 4

Step 2: Intracavernosal Phenylephrine (If Aspiration Fails)

• Inject phenylephrine 100-500 mcg directly into corpus cavernosum (maximum 1000 mcg in first hour). 3, 4
• Success rate: 43-81% when combined with aspiration. 2, 4
• Repeat phenylephrine injections multiple times before considering surgery. 2, 3
• Phenylephrine is superior to other sympathomimetics due to minimal cardiovascular side effects. 3, 4

Step 3: Surgical Shunting (If Medical Management Fails)

• Distal shunts (Winter, Ebbehoj, T-shunt) have 60-80% success rate. 4
• Consider early penile prosthesis if priapism >36-48 hours to prevent corporal fibrosis and preserve penile length. 3

Concurrent Sickle Cell Management

Urologic intervention is the priority—standard sickle cell interventions (hydration, oxygenation, analgesia) should occur concurrently but never replace direct penile treatment. 1, 3

• Systemic sickle cell treatments alone resolve priapism in only 0-37% of cases. 4
• If surgical shunting is required, consider simple transfusion to raise hemoglobin to 9-10 g/dL before general anesthesia. 1

Critical Prognostic Counseling

Time is erectile tissue: 2, 4

• 0% return of spontaneous erections if untreated >36 hours. 2
• Risk of permanent erectile dysfunction approaches 90% after 48 hours. 4
• Minimal smooth muscle damage if treated within 12 hours. 3

Prevention of Recurrent Episodes

Patients with sickle cell disease experiencing priapism should be counseled to present for evaluation at >4 hours duration. 1

The pathophysiology involves insufficient phosphodiesterase type-5 levels leading to unchecked cyclic GMP surges during nocturnal erections. 5, 6