Diagnosis and management of priapism in sickle cell disease
- Joshua J Field, MD
Joshua J Field, MD
- Associate Professor of Medicine
- Medical College of Wisconsin
- Vijaya M Vemulakonda, MD, JD
Vijaya M Vemulakonda, MD, JD
- Assistant Professor, Pediatric Urology
- University of Colorado Denver School of Medicine
- Michael R DeBaun, MD, MPH
Michael R DeBaun, MD, MPH
- Professor of Pediatrics and Internal Medicine
- Vanderbilt University School of Medicine
- Section Editors
- Stanley L Schrier, MD
Stanley L Schrier, MD
- Editor-in-Chief — Hematology
- Section Editor — Myeloproliferative Disorders; Red Cell Disorders
- Professor of Medicine
- Stanford University School of Medicine
- Donald H Mahoney, Jr, MD
Donald H Mahoney, Jr, MD
- Section Editor — Pediatric Hematology
- Professor of Pediatrics
- Baylor College of Medicine
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 . 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".)
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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- Physiology of erection
- Pathophysiology of priapism
- - High flow (arterial) priapism
- - Low flow (ischemic) priapism
- - Nitric oxide pathway
- - Adenosine pathway (adenosine 2b receptor)
- - Genetic influences
- - Risk factors
- CLINICAL PRESENTATION
- Physical examination
- Natural history
- - Erectile dysfunction
- High flow priapism
- Low flow priapism
- TREATMENT OF ISCHEMIC PRIAPISM
- Acute priapism
- - Aspiration and irrigation of the corpus cavernosum
- Technique for aspiration and irrigation
- - Surgical management
- - Transfusion therapy
- Stuttering priapism
- SECONDARY PREVENTION
- Alpha and beta agonists
- Regular blood transfusion
- Therapies of questionable value
- - Hormonal therapy
- - Anti-androgens
- - PDE-5 inhibitors
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS