Priapism in Sickle Cell Anemia

1. 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
2. Epidemiology
   1. Incidence of Priapism in Sickle Cell Anemia: 89% by age 20 years old
3. 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 (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
4. Prevention
   1. Nitroglycerin patch (0.2 - 0.4 mg/hour)
      1. Applied at bedtime may prevent nighttime attacks
5. Course
   1. Resolves spontaneously
   2. May occur frequently
6. Complications
   1. Repeated Priapism results in
      1. Thickening and gross enlargement of the penis
      2. Penis may remain semi-erect
   2. Impotence
      1. Penile Implants
7. References
   1. Glassberg and Weingart in Majoewsky (2012) EM: Rap 12(9): 4
   2. Yawn (2015) Am Fam Physician 92(12): 1069-76 [PubMed]