Priapism in Sickle Cell Disease

https://doi.org/10.1016/j.hoc.2005.08.003Get rights and content

Section snippets

Physiology

The penis contains three vascular chambers: the paired lateral corpora cavernosa and a corpus spongiosum surrounding the urethra. Erection occurs when increased blood volume in the cavernosa and spongiosum is constrained by the tunica albuginea, leading to mechanical elongation of the penis. An erection is normally maintained by the partial obstruction of venous drainage [5]. Priapism occurs when there is either unregulated arterial inflow or persistent obstruction of venous outflow [2].

Epidemiology and natural history

Historically, only 5% to 10% of male patients of any age with sickle cell disease were said to have had priapism [6], [12], [13], [14]. A retrospective review of adult patients now indicates that at least 30% to 45% of patients have a history of this complication [15], [16]. An interview survey of sequential male patients aged 5 to 20 years from Dallas who had sickle cell anemia demonstrated a prevalence of 27.5% by 15 years of age. The calculated actuarial probability of experiencing priapism

Management of acute episodes

Physical examination revealing a tumescent phallus with a history of the unwanted painful erection lasting 30 minutes or more is adequate for diagnosis of priapism. When priapism lasts 4 hours or more, the potential for permanent ischemic injury is present, and intervention is warranted [3]. A suggested approach to the patient presenting for medical attention during an episode of priapism is presented in Box 1.

Numerous therapeutic modalities have been used for treatment of priapism associated

Strategies for the secondary prevention of priapism

For patients who have frequently recurrent stuttering spells or several prolonged episodes requiring repeated aspiration and irrigation, alternative strategies to prevent episodes should be considered. Treatment with hydroxyurea has been suggested by some authors to prevent priapism [45], [46], although others found it ineffective [47]. A controlled trial of the efficacy of hydroxyurea treatment in the prevention of priapism has never been reported. However, this agent, which increases total

Summary

Priapism is, for most patients, an unpredictable, recurrent event associated with devastating consequences for erectile function and quality of life. It occurs in a significant number of males who have all forms of sickle cell disease. Patients should be educated about priapism and currently available treatment options; this education should focus on the need to report recurrent events and to present for care within 4 hours of onset during a prolonged episode. Hematologists, as the physicians

First page preview

First page preview
Click to open first page preview

References (53)

  • R. Virag et al.

    Preventive treatment of priapism in sickle cell disease with oral and self-administered intracavernous injection of etilefrine

    Urology

    (1996)
  • E. Mantadakis et al.

    Outpatient penile aspiration and epinephrine irrigation for young patients with sickle cell anemia and prolonged priapism

    Blood

    (2000)
  • R.J. Bosch et al.

    Penile detumescence: characterization of three phases

    J Urol

    (1991)
  • M. Lee et al.

    Chart for preparation of dilutions of α-adrenergic agonists for intracavernous use in treatment of priapism

    J Urol

    (1995)
  • T.R. Shantha et al.

    Treatment of persistent penile erection and priapism using terbutaline

    J Urol

    (1989)
  • F.C. Lowe et al.

    Placebo-controlled study of oral terbutaline and pseudoephedrine in management of prostaglandin E1-induced prolonged erections

    Urology

    (1993)
  • F.E. Govier et al.

    Oral terbutaline for the treatment of priapism

    J Urol

    (1994)
  • A. Dittrich et al.

    Treatment of pharmacologic priapism with phenylephrine

    J Urol

    (1991)
  • D. Bachir et al.

    Prevention and treatment of erectile disorders in sickle cell disease

    Rev Med Interne

    (1997)
  • R.A. Seeler

    Intensive transfusion therapy for priapism in boys with sickle cell anemia

    J Urol

    (1973)
  • W.R. Rackoff et al.

    Neurologic events after partial exchange transfusion for priapism in sickle cell disease

    J Pediatr

    (1992)
  • C.C. Winter et al.

    Experience with 105 patients with priapism: update review of all aspects

    J Urol

    (1988)
  • A.H. Al Jam'a et al.

    Hydroxyurea in the treatment of sickle cell associated priapism

    J Urol

    (1998)
  • E.S. Bialecki et al.

    Slidenafil relieves priapism in patients with sickle cell disease

    Am J Med

    (2002)
  • G.R. Serjeant et al.

    Stilboestrol and stuttering priapism in homozygous sickle-cell disease

    Lancet

    (1985)
  • L.A. Levine et al.

    Gonadotropin-releasing hormone analogues in the treatment of sickle cell anemia–associated priapism

    J Urol

    (1993)
  • Cited by (0)

    View full text