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Epispadias

Definition:

Epispadias is a rare defect that is present at birth (congenital). It is located at the opening of the urethra.

In this condition, the urethra does not develop into a full tube. The urine exits the body from the wrong place.



Causes, incidence, and risk factors:

The causes of epispadias are not known. It may occur because the pubic bone does not develop properly.

Epispadias can occur with bladder exstrophy. In this rare birth defect, the bladder is inside out and sticks through the abdomen wall. Epispadias can also occur with other birth defects.

Epispadias occurs in 1 out of every 117,000 newborn boys and 1 in 484,000 newborn girls. The condition is usually diagnosed at birth or soon afterward.



Symptoms:

Males usually have a short, wide penis with an abnormal curve. The urethra usually 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 clitorus and labia. The opening is usually between the clitoris and the labia, but it may be in the belly area. They may have trouble controlling urination (urinary incontinence).



Signs and tests:

Signs include:

  • Abnormal opening from the bladder neck to the area above the normal urethra opening
  • Backward flow of urine into the kidney (reflux nephropathy)
  • Urinary incontinence
  • Urinary tract infections
  • Widened pubic bone

Tests may include:

  • Blood test
  • Intravenous pyelogram (IVP), a special x-ray of the kidneys, bladder, and ureters
  • MRI and CT scans, depending on the condition
  • Pelvic x-ray
  • Ultrasound of the urinary system and genitals


Treatment:

Patients 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.



Support Groups:



Expectations (prognosis):

Surgery can help the person control the flow of urine. It will also fix the appearance of the genitals.



Complications:

Some people with this condition may continue to have urinary incontinence, even after surgery.

Ureter and kidney damage and infertility may occur.



Calling your health care provider:

Call your health care provider if you have any questions about the appearance or function of your child's genitals or urinary tract.



Prevention:



References:

Gearhart JP, Mathews RI. Exstrophy-epispadias complex. In: Wein AJ, ed. Campbell-Walsh Urology. 10th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 124.

Elder JS. Anomalies of the bladder. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 535.




Review Date: 10/18/2011
Reviewed By: Linda J. Vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assistant Studies, University of Washington, School of Medicine; Scott Miller, MD, Urologist in private practice in Atlanta, Georgia. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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