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Epispadias

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

The 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 inside out and sticks through the abdomen wall. 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.

Symptoms

Males 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 clitorus 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 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.

Outlook (Prognosis)

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

Possible Complications

Some 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 Professional

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

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.

Updated: 1/12/2014

Scott Miller, MD, Urologist in private practice in Atlanta, GA. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.


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