Bladder Exstrophy & Epispadias Repair

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Bladder exstrophy is a rare birth defect in which your baby’s bladder develops inside out, pokes out through the wall of the abdomen and is seen on the outside of the baby’s lower belly. Babies with bladder exstrophy may also have defects of the pelvic bones and muscles in the pelvis and abdomen.

Epispadias is a condition in which the opening of the urethra (the tube that carries urine from the bladder to outside the body) develops in the wrong place on your baby’s body. In boys, this usually means that the opening of the urethra is on the top of the penis. In girls, the opening of the urethra may be longer than normal or higher in the urethra.

The epispadias-exstrophy complex is a range of congenital conditions that involve the genitals, urinary tract, pelvic bones and muscles, abdominal muscles and abdominal wall, spine and anus. These conditions range in severity, from epispadias (least severe) to bladder exstrophy and exstrophy of the cloaca (most severe).

Causes of Bladder Exstrophy & Epispadias

We don’t know what causes conditions in the epispadias-exstrophy complex. Boys are slightly more likely to develop one of these conditions.

The symptoms of bladder exstrophy, epispadias and other conditions in the epispadias-exstrophy complex may include:

  • Triangle-shaped hole in the abdomen through which the bladder is visible
  • Urethral opening is in the wrong place
  • Abnormalities in the shape of the genitals
  • Widened pubic bones
  • Legs and feet turned out
  • Abnormally shaped abdominal muscles
  • Belly button is not in a normal place
  • Bulging of intestine through the abdomen

Diagnosis & Treatment at UVA

The symptoms of bladder exstrophy, epispadias and other conditions vary and may be diagnosed using ultrasound before birth. After birth, you child’s provider may order other imaging exams.

We treat conditions in the epispadias-exstrophy complex with surgery. Even with treatment, your child may have lasting abnormalities of the genitals and urinary tract. They may experience issues controlling their urine. They may also have issues with fertility.

Usually, we perform these surgeries over three stages:

  • Stage 1: Performed within a few days after birth. The bladder is put inside the body, and the abdomen is closed.
  • Stage 2: Performed after 6 months of age. This stage includes repair of the epispadias and of other genital problems.
  • Stage 3: Performed at age 4 to 5 years. This stage involves rebuilding the urinary tract, including the bladder and tubes.