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  Priapism
 
Author: Jonathan Jarow Last modified: March 19, 2002
DIAGNOSTIC CRITERIA

  EPIDEMIOLOGY

  • Priapism is defined as a prolonged painful erection that fails to subside despite orgasm.
  • This is an extremely rare disorder that occurs most commonly in adults with a history of sickle cell disease or trait.
  • Peak incidence occurs in ages 5 to 10 years and 20 to 50 years.
  • Priapism has been classified into two types that are managed differently, low flow (ischemic) and high flow.

  RISK FACTORS

  • Sickle cell disease or sickle cell trait as well as other hemoglobinopathies.
  • Medications, including but not limited to antidepressants, antipsychotics, antihypertensive and hormonal agents have been associated with priapism.
  • Pharmacologic injection therapy for erectile dysfunction.
  • Pelvic trauma
  • Malignancy including leukemia, prostate cancer, renal cancer and melanoma.
  • Total parenteral nutrition has been associated with priapism, particularly use of 20% IV fat emulsion.

  SIGNS AND SYMPTOMS

  • Painful erection
  • Prolonged erection (greater than 3 to 6 hours duration).
  • Non-sexual erection.
  • Glans penis is typically not erect.

  DIFFERENTIAL DIAGNOSIS

  • Sickle cell disease or trait and rarely other hemoglobinopathies.
  • Arterial injury or fistula.
  • Medication induced low flow or high flow priapism.
  • Erectile dysfunction treatment induced, most commonly intracavernous injection.
  • Idiopathic

  LABORATORY FINDINGS

  • Intracorporeal blood gas to differentiate high flow versus low flow priapism.
  • Hemoglobin electrophoresis to detect a hemoglobinopathy.
  • Color flow penile duplex ultrasonography may be utilized to detect a cavernosal arterial injury/fistula in patients with high flow priapism.
  • Pelvic arteriogram may be utilized to diagnose cavernosal arterial injury in patients with high flow priapism and to perform embolization treatment.
 
TREATMENT
CONSERVATIVE MEASURES
  • Local application of cool compresses or ice is sometimes effective for prolonged erections following intracavernosal injection.
  • Oral medications such as terbutaline (5 mg) may be effective in patients with pharmacologically induced priapism.
  • Oxygenation, alkalinization, and plasmaphoresis may be employed in patients with a hemoglobinopathy.
INTRACORPOREAL IRRIGATION WITH VASOCONSTRICTOR
  • Drainage of blood from the corpora is the first step in the management of patients with prolonged erections particularly drug induced.
  • Irrigation with a dilute alpha agonist vasoconstrictor, phenylephrine (5 to 50 mcg), should be performed while monitoring vital signs.
SHUNTING PROCEDURES
  • Surgical intervention is indicated for patients with low flow priapism unresponsive to conservative measures.
  • Creation of shunt between the glans penis and distal corpora is the preferred initial surgical approach.
  • Shunting between the corpora and the corpus spongiousm of the bulbar urethra may be performed if simpler procedures fail.
  • There is a risk of erectile dysfunction following shunting procedures.
EMBOLIZATION
  • Indicated for high flow priapism.
  • Preferable to embolize with a dissolvable substance such as autologous clot.
  • Abscess formation has occurred following this procedure.
PREVENTION
  • Prophylactic measures are employed in patients with recurrent or stuttering priapism.
  • Daily treatment with terbutaline (5 mg) or early in the course of a prolonged erection.
  • There is experimental evidence that digoxin may inhibit the development of priapism.
  • Hormonal therapy with GnRH agonists (depot Lupron or Zoladex) has been the most successful treatment for the prevention of priapism and typically normal sexual function is preserved.
 
IMPORTANT POINTS/RECOMMENDATIONS
  • Priapism is a medical emergency that requires prompt attention to avoid loss of penile tissue or function.
  • Conservative measures are the best initial management for patients with priapism due to a hempoglobinopathy.
  • There is a risk of erectile dysfunction from both priapism itself and the treatments for this disorder.
  • It is important to distinguish between low and high flow priapism since patients with high flow priapism can be safely observed.
  • Some patients develop frequent recurrent priapism (stuttering priapism) that require preventative treatment.
 
REFERENCES
  1. Gitlin MJ. ;  Psychotropic medications and their effects on sexual function: diagnosis, biology, and treatment approaches. ;  J Clin Psychiatry. 1995 Nov;56(11):536-7

  2. Harmon WJ, Nehra A. ;  Priapism: diagnosis and management. ;  Mayo Clin Proc 1997 Apr;72(4):350-5

  3. Kerlan RK Jr, Gordon RL, LaBerge JM, Ring EJ. ;  Superselective microcoil embolization in the management of high-flow priapism. ;  J Vasc Interv Radiol 1998 Jan-Feb;9(1 Pt 1):85-9

  4. Lomas GM, Jarow JP. ;  Risk factors for papaverine-induced priapism. ;  J Urol 1992 May;147(5):1280-1

  5. Lowe FC, Jarow JP. ;  Placebo-controlled study of oral terbutaline and pseudoephedrine in management of prostaglandin E1-induced prolonged erections. ;  Urology 1993 Jul;42(1):51-3

  6. Lue TF, Hellstrom WJ, McAninch JW, Tanagho EA. ;  Priapism: a refined approach to diagnosis and treatment. ;  J Urol 1986 Jul;136(1):104-8

  7. Muruve N, Hosking DH. ;  Intracorporeal phenylephrine in the treatment of priapism. ;  J Urol 1996 Jan;155(1):141-3

  8. Powars DR, Johnson CS. ;  Priapism. ;  Hematol Oncol Clin North Am 1996 Dec;10(6):1363-72

  9. Sancak T, Conkbayir I. ;  Post-traumatic high-flow priapism: management by superselective transcatheter autologous clot embolization and duplex sonography-guided compression ;  J Clin Ultrasound 2001 Jul-Aug;29(6):349-53

  10. Virag R, Bachir D, Lee K, Galacteros F. ;  Preventive treatment of priapism in sickle cell disease with oral and self-administered intracavernous injection of etilefrine. ;  Urology 1996 May;47(5):777-81

  11. Witt MA, Goldstein I, Saenz de Tejada I, Greenfield A, Krane RJ. ;  Traumatic laceration of intracavernosal arteries: the pathophysiology of nonischemic, high flow, arterial priapism. ;  J Urol 1990 Jan;143(1):129-32

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