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Diagnosis and management of priapism in sickle cell disease

Authors
Joshua J Field, MD
Vijaya M Vemulakonda, MD, JD
Michael R DeBaun, MD, MPH
Section Editors
Stanley L Schrier, MD
Donald H Mahoney, Jr, MD
Deputy Editor
Jennifer S Tirnauer, MD

INTRODUCTION

Priapism is a sustained penile erection in the absence of sexual activity or desire. The definition of "sustained" in this setting is unclear, but priapism is generally defined as an unwanted erection lasting more than two to four hours.

Prompt recognition and appropriate treatment of a priapism episode in males with sickle cell disease (SCD) is critical, as the end result of prolonged and/or repeated episodes of priapism can be ischemia and fibrosis in the corpus cavernosa of the penis, potentially leading to impaired sexual function and impotence.

Few evidence-based guidelines exist for the management of these episodes. Thus, in the absence of randomized clinical trials and large, detailed prospective studies, clinicians must rely largely on case reports, small case series, and clinical experience. A practical approach to the diagnosis and management of priapism in patients with SCD will be presented here [1]. A general overview of the subject of priapism is presented separately. (See "Priapism".)

Overall management of the patient with SCD is discussed separately. (See "Overview of the management and prognosis of sickle cell disease".)

PATHOPHYSIOLOGY

Physiology of erection — Penile erection occurs when there is increased blood volume in the paired lateral corpora cavernosa and single corpus spongiosum, and is normally maintained by the partial obstruction of venous drainage (figure 1). (See "Overview of male sexual dysfunction", section on 'Physiology of male sexual function'.)

                                   

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Literature review current through: Nov 2016. | This topic last updated: Thu Sep 11 00:00:00 GMT+00:00 2014.
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