Hemoglobin

Priapism in Sickle Cell Anemia

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Priapism in Sickle Cell Anemia

  • Epidemiology
  1. Incidence of Priapism in Sickle Cell Anemia: 89% by age 20 years old
  • 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
  • Prevention
  1. Nitroglycerin patch (0.2 - 0.4 mg/hour)
    1. Applied at bedtime may prevent nighttime attacks
  • Course
  1. Resolves spontaneously
  2. May occur frequently
  • 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
  • References
  1. Glassberg and Weingart in Majoewsky (2012) EM: Rap 12(9): 4
  2. Yawn (2015) Am Fam Physician 92(12): 1069-76 [PubMed]