Epispadias
Definition
Epispadias is a rare congenital (present from birth) defect in the location of the opening of the urethra.
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
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:
In females:
- Abnormal opening from the bladder neck to the area above the normal urethral opening
- Bladder exstrophy (may or may not be present)
- Widened pubic bone
- Bifid clitoris, rudimentary labia
- Urinary incontinence
- Reflux nephropathy
- Urinary tract infections
Exams and Tests
Treatment
Surgical repair of epispadias is recommended. Leakage of urine (incontinence) is not uncommon and may require a second operation.
Outlook (Prognosis)
Surgical repairs generally produce both continence (the ability to control the flow of urine) and a good cosmetic outcome.
Possible 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.
When to Contact a Medical Professional
Call your health care provider if you have any questions or concerns regarding your child's genitourinary tract appearance or function.
Review Date:
6/14/2006
Reviewed By:
Neil D. Sherman, MD, Urologist, Essex County, NJ. Review provided by VeriMed Healthcare Network.
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