What is the management approach for a patient presenting with priapism, potentially due to underlying conditions such as sickle cell disease or leukemia?

Management of Priapism

Immediately administer intracavernosal phenylephrine with corporal aspiration as first-line treatment for acute ischemic priapism, regardless of underlying conditions like sickle cell disease or leukemia—do not delay urologic intervention for disease-specific systemic therapies. 1

Initial Diagnostic Differentiation

The critical first step is distinguishing ischemic from non-ischemic priapism through corporal blood gas analysis, as this fundamentally determines urgency and treatment approach: 2, 3

• Ischemic priapism: PO₂ <30 mmHg, PCO₂ >60 mmHg, pH <7.25—completely rigid corpora with severe pain 2, 3
• Non-ischemic priapism: PO₂ >90 mmHg, PCO₂ <40 mmHg, pH 7.40—partial tumescence without full rigidity, typically painless 2, 3

Acute Ischemic Priapism Management

Primary Treatment (All Patients Including SCD/Leukemia)

The standard urologic approach takes absolute priority over hematologic interventions: 1

• Intracavernosal phenylephrine (100-500 mcg/mL concentration, maximum 1000 mcg within first hour) combined with corporal aspiration ± irrigation 2
• Success rates of 43-81% when aspiration and phenylephrine are combined 2
• Patients should present for urologic evaluation when priapism exceeds 4 hours to prevent permanent corporal damage and erectile dysfunction 1

Critical Timing Considerations

Duration directly correlates with erectile dysfunction risk: 2

• <24 hours: Reasonable chance of preserving erectile function
• 24-36 hours: Significantly increased ED risk
• >36 hours: High likelihood of permanent erectile dysfunction

Special Populations: Sickle Cell Disease and Leukemia

Do not delay standard priapism management for disease-specific interventions such as exchange transfusion or leukapheresis. 1

• Exchange transfusion should NOT be used as primary treatment for acute ischemic priapism in SCD patients 1, 4
• Acute exchange transfusion requires 6+ hours to prepare with extended red cell antigen-matched products, placing patients at increased impotence risk from prolonged ischemia 1
• No data demonstrates exchange transfusion terminates episodes sooner than standard procedures 1
• Standard sickle cell assessment and interventions should occur concurrent with (not instead of) urologic intervention 1

Escalation for Refractory Cases

If intracavernosal phenylephrine and aspiration fail: 1

• Exchange transfusion may be considered for prolonged episodes unresponsive to standard treatment 1
• If operative shunting procedures are required, consider simple transfusion of packed RBCs to raise hemoglobin to 9-10 g/dL prior to general anesthesia 1, 4

Non-Ischemic Priapism Management

Observe for up to 4 weeks as initial management—this is not a urologic emergency: 2, 3

• Many arterio-cavernous fistulas close spontaneously resulting in detumescence 2, 3
• If priapism persists after observation and patient desires treatment, perform penile duplex Doppler ultrasound to identify fistula location 2
• Percutaneous fistula embolization is first-line therapy if observation fails, with 85% success rate 3

Recurrent Ischemic Priapism Prevention

For patients with recurrent episodes, preventative strategies include: 1

• PDE5 inhibitors (tadalafil or sildenafil): Recent reports suggest regimented therapy reduces frequency and duration with no negative side effects 1
• Ketoconazole with prednisone: Highest success rate but requires frequent liver function monitoring due to hepatotoxicity risk 1
• Hydroxyurea (for SCD patients) 1
• Home self-injection of phenylephrine on as-needed basis is reasonable but not preventative 1

Important Counseling for Hormonal Regulators

Patients must be informed that hormonal regulators (ketoconazole, cyproterone acetate) may impair fertility and sexual function: 1

• Potential side effects include fatigue, hot flashes, breast tenderness, mood changes, and erectile dysfunction 1
• These therapies may negatively impact sperm parameters 1
• Particularly poorly tolerated in younger men with potential long-term consequences 1

Common Pitfalls to Avoid

• Never delay intracavernosal phenylephrine for exchange transfusion in SCD patients—this is the most common error 1, 4
• Do not treat non-ischemic priapism as an emergency or perform aggressive interventions like aspiration/phenylephrine injection 3
• Do not exceed hemoglobin of 10 g/dL in SCD patients receiving transfusion, as this increases risk of vaso-occlusive complications and hyperviscosity 4
• Delaying corporal blood gas analysis leads to misdiagnosis and inappropriate treatment urgency 2, 3