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, 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 repairs generally produce 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.

Johns Hopkins patient information

Last revised: December 7, 2012
by Mamikon Bozoyan, M.D.

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