Priapism and erectile dysfunction 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
- Associate Professor, Pediatric Urology
- University of Colorado School of Medicine
- Michael R DeBaun, MD, MPH
Michael R DeBaun, MD, MPH
- Professor of Pediatrics and Internal Medicine
- Vanderbilt University School of Medicine
- Arthur L Burnett, MD, MBA, FACS
Arthur L Burnett, MD, MBA, FACS
- Patrick C. Walsh Distinguished Professor of Urology, Cellular and Molecular Medicine
- Johns Hopkins Medicine
- Section Editors
- Stanley L Schrier, MD
Stanley L Schrier, MD
- Editor-in-Chief — Hematology
- Section Editor — Myeloproliferative Disorders; Red Blood 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 (penile erection in the absence of sexual activity or desire) is a common complication of sickle cell disease (SCD) in men. The vast majority of cases are ischemic, in which increased pressure compromises the vascular circulation (ie, a type of compartment syndrome). Over time, repeated episodes cause permanent damage and erectile dysfunction. Thus, priapism is considered a medical emergency in which timely diagnosis and appropriate management are vital to preserving normal function. This is a challenging management area because there are few experts dedicated to managing priapism in SCD and few large trials on which to base practice, and management often involves multiple specialties including urology, emergency medicine, pediatrics, and hematology.
This topic review discusses our approach to the evaluation and treatment of priapism and erectile dysfunction in boys and men with SCD.
Related subjects, including the use of hydroxyurea and regular blood transfusion, as well as overviews of the other clinical findings and routine management of SCD, are presented separately:
●Hydroxyurea – (See "Hydroxyurea use in sickle cell disease".)
●Transfusion – (See "Red blood cell transfusion in sickle cell disease".)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- PATHOPHYSIOLOGY AND CLASSIFICATION
- Mechanisms of priapism
- Mechanisms of ED
- CLINICAL FEATURES AND EVALUATION
- Typical presentations
- Precipitating factors
- History, examination, and cavernosal blood gas
- Laboratory testing and imaging
- ISCHEMIC PRIAPISM: ACUTE MANAGEMENT
- Management at home
- Management in the emergency department and hospital
- Aspiration and irrigation of the corpus cavernosum
- Surgical management
- Overview of prevention
- Regular red blood cell transfusions
- PDE-5 inhibitors (sildenafil)
- Alpha- and beta-adrenergic agonists
- Hormonal therapies
- EVALUATION AND MANAGEMENT OF ERECTILE DYSFUNCTION
- MANAGEMENT OF HIGH-FLOW PRIAPISM
- SOCIETY GUIDELINE LINKS
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS