Epispadias

Epispadias

Definition:

Epispadias is a rare congenital (present from birth) defect in which the opening of the urethra is in an abnormal location.

In boys with epispadias, the urethra generally opens on the top or side, rather than the tip, of the penis, though it is possible for the urethra to be open the entire length of the penis. In girls, the opening is usually between the clitoris and the labia but may be in the abdomen.

Causes, incidence, and risk factors:

The causes of epispadias are unknown at this time. It is believed to be related to improper development of the pubic bone. Epispadias is often associated with bladder exstrophy. However, it can also occur alone or with defects other than exstrophy.

Epispadias occurs in 1 in 117,000 newborn boys and 1 in 484,000 newborn girls. The condition is usually diagnosed at birth or shortly thereafter.

Symptoms:

In males:

Abnormal opening from the pubic symphysis to the area above the tip of the penis
Bladder exstrophy (may or may not be present)
Widened pubic bone
Short, widened penis with chordee (abnormal curvature of the penis)
Urinary incontinence
Reflux nephropathy
Urinary tract infections

In females:

Abnormal opening from the bladder neck to the area above the normal urethal opening
Bladder exstrophy (may or may not be present)
Widened pubic bone
Bifid clitoris, rudimentary labia
Urinary incontinence
Reflux nephropathy
Urinary tract infections

Signs and tests:

CBC
Serum electrolytes
Pelvic X-ray
Intravenous pyelogram (IVP)
Ultrasound of the urogenital system

Treatment:

Surgical repair of epispadias is recommended. Leakage of urine (incontinence) is not uncommon and may require a second operation.

Expectations (prognosis):

Surgical repair generally results in both continence (the ability to control the flow of urine) and a good cosmetic outcome.

Complications:

Persistent urinary incontinence can occur in some people even after multiple operations. Also upper urinary tract (ureter and kidney) damage as well as infertility may occur.

Calling your health care provider:

Call your health care provider if you have any questions or concerns regarding your child's genitourinary tract appearance or function.

Review Date: 5/25/2002
Reviewed By: Young Kang, M.D., Department of Urology, Columbia University College of Physicians and Surgeons, New York, NY. Review provided by VeriMed Healthcare Network.

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