Congenital defect - epispadias DefinitionEpispadias is a rare defect that is present at birth. In this condition, the urethra does not develop into a full tube. The urethra is the tube that carries urine out of the body from the bladder. The urine exits the body from the wrong place with epispadias. CausesThe causes of epispadias are not known. It may occur because the pubic bone does not develop properly. Epispadias can occur with a rare birth defect called bladder exstrophy. In this birth defect, the bladder is open through the wall of the abdomen. Epispadias can also occur with other birth defects. The condition occurs more often in boys than girls. It is most often diagnosed at birth or soon afterward. SymptomsMales will have a short, wide penis with an abnormal curve. The urethra most often opens on the top or side of the penis instead of the tip. However, the urethra may be open along the whole length of the penis. Females have an abnormal clitoris and labia. The urethral opening is often between the clitoris and the labia, but it may be in the belly area. They may have trouble controlling urination (urinary incontinence). Exams and TestsSigns include:
Tests may include:
TreatmentPeople who have more than a mild case of epispadias will need surgery. Leakage of urine (incontinence) can often be repaired at the same time. However, a second surgery may be needed either soon after the first surgery, or sometime in the future. Outlook (Prognosis)Surgery can help the person control the flow of urine. It will also fix the appearance of the genitals. Possible ComplicationsSome people with this condition may continue to have urinary incontinence, even after surgery. Ureter and kidney damage and infertility may occur. When to Contact a Medical ProfessionalContact your health care provider if you have any questions about the appearance or function of your child's genitals or urinary tract. ReferencesChaudhry R, Cannon GM. Urologic disorders. In: Zitelli BJ, McIntire SC, Nowalk AJ, Garrison J, eds. Zitelli and Davis' Atlas of Pediatric Physical Diagnosis. 7th ed. Philadelphia, PA: Elsevier; 2023:chap 15. Elder JS. Anomalies of the bladder. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 556. Gearhart JP, Di Carlo HN. Exstrophy-epispadias complex. In: Partin AW, Dmochowski RR, Kavoussi LR, Peters CA, eds. Campbell-Walsh-Wein Urology. 12th ed. Philadelphia, PA: Elsevier; 2021:chap 31. | |
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Review Date: 10/15/2023 Reviewed By: Kelly L. Stratton, MD, FACS, Associate Professor, Department of Urology, University of Oklahoma Health Sciences Center, Oklahoma City, OK. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. No warranty of any kind, either expressed or implied, is made as to the accuracy, reliability, timeliness, or correctness of any translations made by a third-party service of the information provided herein into any other language. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited. | |
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