Hemoglobin

Priapism in Sickle Cell Anemia

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Priapism in Sickle Cell Anemia

  • See Also
  1. Sickle Cell Anemia
  2. Priapism
  3. Transient Red Cell Aplasia
  4. Acute Chest Syndrome
  5. Acute Vaso-Occlusive Episode in Sickle Cell Anemia
  6. Aplastic Crisis in Sickle Cell Anemia
  7. Cerebrovascular Accident in Sickle Cell Anemia
  8. Dactylitis in Sickle Cell Anemia (Hand Foot Syndrome in Sickle Cell Anemia)
  9. Hematuria in Sickle Cell Anemia
  10. Osteomyelitis in Sickle Cell Anemia
  11. Pulmonary Hypertension in Sickle Cell Anemia
  12. Septic Arthritis in Sickle Cell Anemia
  13. Sickle Cell Anemia Related Pulmonary Hypertension
  14. Sickle Cell Anemia with Splenic Sequestration
  • Epidemiology
  1. Incidence of Priapism in Sickle Cell Anemia
    1. Occurs in 35 to 45% of male SCA patients (up to 89% of patients by age 20 years old)
  • Pathophysiology
  1. Sickled RBCs sludge in the corpora cavernosa veins, blocking venous outflow
    1. Results in low-flow ischemic Priapism
  • Management
  1. Duration: <2 hours
    1. Analgesics
    2. IV Fluids
    3. Do not transfuse for Priapism <4 hours
  2. Duration: 2-4 hours
    1. First
      1. Intracavernosal Epinephrine (1:1,000,000 dilution) or Phenylephrine injection
    2. Other measures
      1. Nifedipine 10 mg (in repeated doses)
  3. Duration >4 hours
    1. Administer above measures if not already attempted
    2. Exchange Transfusion
      1. No longer routinely recommended (but may consult hematology)
      2. Do not delay other specific Priapism management
      3. Risk of CVA symptoms
    3. Epidural Anesthesia
  4. Urology Consultation indications
    1. Priapism refractory to medical management >4-6 hours
    2. Corpora spongiosa and cavernosa Shunt (Priapism >24 hours)
      1. Placed through glans penis
      2. Often effective (may need repeat)
      3. Does not interfere with subsequent Erection
  5. Discharge criteria
    1. Priapism resolves and no recurrence during observation in ED
  • Prevention
  1. Nitroglycerin Patch (0.2 - 0.4 mg/hour)
    1. Applied at bedtime may prevent nighttime attacks
  • Course
  1. Often resolves spontaneously and may occur frequently
  2. However, persistent Priapism >2 hours should be aggressively managed to prevent complications
  • Complications
  1. Repeated Priapism results
    1. Cellular damage and fibrosis
    2. Thickening and gross enlargement of the penis
    3. Penis may remain semi-erect
  2. Erectile Dysfunction
    1. Penile Implants may be required
  • References
  1. Dwyer, Kleinmann, Goswami and Lopez (2025) Crit Dec Emerg Med 39(1): 26-35
  2. Glassberg and Weingart in Majoewsky (2012) EM: Rap 12(9): 4
  3. Yawn (2015) Am Fam Physician 92(12): 1069-76 [PubMed]