DIAGNOSIS, GUIDELINES FOR COMPREHENSIVE CARE, AND CARE PATHS AND PROTOCOLS FOR MANAGEMENT OF ACUTE AND CHRONIC COMPLICATIONS  the Sickle Cell Disease Care Consortium (Arizona, Colorado, Georgia, Missouri, New Mexico, Tennessee, Texas, and Utah)

Priapism is a prolonged painful erection of the penis that commonly occurs in children and adolescents with sickle cell disease, often starting during the early morning hours. It occurs in two forms: (a) stuttering episodes which last less than 2-4 hours but are often recurrent and may precede a severe episode, and (b) severe events that last more than 2-4 hours and may eventually result in impotence. Simple maneuvers such as increasing oral fluids, taking analgesics, urination, moderate exercise, and/or taking a bath or shower may help end an episode of priapism, and no further specific intervention may be required. Patients who have frequent episodes (³ 2 within one month or ³ 4 within one year) should contact their sickle cell program for elective evaluation. For such patients, priapism prophylaxis with pseudoephedrine 30 mg/po hs (<10 years) or 60 mg/po hs (>10 years) should be considered. Any episode that lasts longer than 3-4 hours should be considered an emergency that requires prompt medical intervention as described below.

1. Rapid triage - immediately upon presentation. Place immediately into exam room.

2. History with emphasis on:

• length of current episode
• associated symptoms - especially fever, dysuria, evidence of dehydration, or pain in other locations
• history of prior episodes of priapism, previous treatments and effectiveness

• symptoms of obstructive sleep apnea.

3. Physical Examination with emphasis on:

• vital signs
• hydration status
• degree of pallor and cardiopulmonary status
• genitourinary (severity of pain, any evidence of detumescence)

4. Laboratory:

• consider CBC, diff, platelet, reticulocyte count (compare with patient's baseline values)
• blood cultures if febrile (see fever care path, p. 16)
• urinalysis and urine culture for history of dysuria or fever
• type and cross match if extreme pallor, respiratory or neurologic symptoms, or acute splenic enlargement present. Consider requesting, if available, minor antigen matched, sickle negative, and leukocyte-depleted RBCs.

5. If patient has not detumesced and episode has lasted longer than 3-4 hours, contact urologist to perform aspiration and irrigation as described in #9 below.

6. Review summary of patients last comprehensive evaluation or seek baseline information by phone.

7. Contact pediatric hematologist or patient's primary care physician with expertise in sickle cell disease.

8. Treatment (discuss with patient, family, and hematologist or primary physician on-call)

• Do not use ice, ice packs, or ice water enemas.
• IV fluids: 10 cc/kg bolus over one hour then at maintenance rate
• analgesia: for moderate to severe pain, morphine 0.1-0.15 mg/kg IV. Reassess pain q 15-30 minutes. Patients with severe pain may require repeated doses of morphine 0.02-0.05 mg/kg IV q 15-30 minutes to achieve pain relief
• monitor pulse ox for patients receiving opioid analgesia
• use O₂ by nasal cannula or face mask as needed to keep O₂ saturation ³ 92% or ³ patient's baseline value (if >92%).

9. Aspiration and irrigation: The following procedure should be performed by a staff urologist or experienced urology resident as soon as possible for episodes that have lasted more than 4 hours from onset of erection. Conscious sedation may be appropriate for selected patients if administered by experienced staff, but usually is not required.

• The lateral side of the penis is prepped with betadine and approximately 0.5 ml of 1% lidocaine is infiltrated subcutaneously into the lateral surface of the penis and then more deeply into the tunica albuginea.
• A 23 gauge needle is inserted into the corpora cavernosa and as much blood as possible is aspirated into a dry 10 ml syringe through a three-way stopcock.
• Another 10 ml syringe containing 1:1,000,000 solution of epinephrine (ie 1ml of 1:1,000 epinephrine diluted in 1 liter of normal saline) is attached to the three-way stopcock. The corpora cavernosa are irrigated with 10 ml of the 1:1,000,000 epinephrine solution, with additional blood aspirated via dry syringes until detumescence has occurred. Some urologists prefer using a dilute solution of phenylephrine as an alternative to epinephrine.
• The needle is withdrawn and five minutes of firm pressure (timed by the clock) is applied by the physician doing the procedure to prevent hematoma formation.
• If the patient retains detumescent for ³ 1 hour, he may be discharged home with hematologist/urologist/PCP approval and a specific plan for outpatient follow-up.
• If priapism recurs, aspiration and irrigation may be repeated up to 3-4 times if needed.
• Consider pseudoephedrine 30 mg po hs (< 10 years-of-age) or 60 mg po hs (> 10 years-of-age) for priapism prophylaxis.
• If the episode fails to respond to aspiration and irrigation, the patient should be hospitalized for inpatient management (p. 25).
• Consider sleep study if obstructive sleep apnea suspected

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References

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Copyright © 1997 Sickle Cell Information Center
Last modified: November 01, 2002