Priapism in Sickle Cell Anemia

1. See Also
   1. Sickle Cell Anemia
   2. Priapism
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
   2. Duration: >2 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. Refractory
         1. Exchange Transfusion (Risk of CVA symptoms)
         2. Epidural Anesthesia
   3. Discharge criteria
      1. Priapism resolves and no recurrence during observation in ED
   4. Urology consultation indications
      1. Priapism refractory to medical management >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
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