A 30-year-old man with sickle cell disease presents with priapism lasting 4 hours. Which immediate management is MOST appropriate?

• A IV morphine and oral hydration only
• B Exchange transfusion immediately
• C Sildenafil to reduce pulmonary hypertension-related priapism
• D Aspiration of the corpus cavernosum + intracavernosal phenylephrine injection ✓

Explanation

Priapism in sickle cell disease is a urological emergency due to sickling in the penile venous system causing ischemic (low-flow) priapism. Initial management for acute priapism > 4 hours includes aspiration of the corpus cavernosum and intracavernosal injection of a sympathomimetic (phenylephrine preferred over epinephrine due to selectivity) to cause vasoconstriction and detumescence. Systemic analgesia, IV hydration, and supplemental oxygen are supportive. Exchange transfusion (RBC exchange targeting HbS < 30%) is considered for priapism not responding to local measures or for stuttering priapism prophylaxis with hydroxyurea long-term.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

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Written and medically reviewed by the StethoPrep medical team.