Priapism in Sickle Cell Anemia

Aka: Priapism in Sickle Cell Anemia

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II. Epidemiology

  1. Incidence of Priapism in Sickle Cell Anemia: 89% by age 20 years old

III. 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

IV. Prevention

  1. Nitroglycerin Patch (0.2 - 0.4 mg/hour)
    1. Applied at bedtime may prevent nighttime attacks

V. Course

  1. Resolves spontaneously
  2. May occur frequently

VI. 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

VII. References

  1. Glassberg and Weingart in Majoewsky (2012) EM: Rap 12(9): 4
  2. Yawn (2015) Am Fam Physician 92(12): 1069-76 [PubMed]

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