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Bladder Exstrophy (Pediatric)

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What is bladder exstrophy?

Exstrophy is a rare and complex disorder in which the abdominal wall fails to close while a baby grows in the womb. The intestine may be abnormally connected to the bladder, and the urethra and genitals may not be completely formed. Exstrophy must be diagnosed before birth to prepare a multidisciplinary team and surgical plan.

Our reconstructive surgeons form one of the few medical teams on the East Coast able to treat exstrophy and have trained with world leaders and innovators in the treatment of this disorder.

Cloacal exstrophy usually involves many systems in the body, including the urinary tract, skeletal muscles and bones, and the digestive system. Bladder exstrophy means the bladder is essentially open and exposed on the outside of the abdomen. As a result, urine constantly trickles onto the skin and causes local irritation.

Epispadias often occurs with bladder exstrophy. Epispadias is when the urethral opening, the hollow tube that drains urine from the bladder to the outside of the body, is in an abnormal location. In males, the urethral opening is usually on the top side of the penis and not the tip. Epispadias differs from hypospadias, where the urethral opening is usually underneath the penis. In females, the urethral opening may be bigger and longer than normal, and positioned further up the urethra, often extending to the bladder.

Who is affected by bladder exstrophy?

Bladder exstrophy occurs in about one in every 30,000 births. The disorder is more common in males and varies in severity. Its cause is unknown, though some reports show a clustering of bladder exstrophy in families, suggesting an inherited factor. However, the chance for parents to have another child with bladder exstrophy is small (1 percent or less). In many cases, bladder exstrophy is associated with:

  • widened pubic bones
  • outwardly rotated legs and feet
  • triangular defect in the abdomen and visible bladder membrane of a bright pink hue
  • abnormally-shaped abdominal muscles
  • displacement of the umbilicus (belly button), usually above the defect
  • umbilical hernia (a section of intestine protrudes through a weakness in the abdominal muscles)
  • short, small penis with urethral opening along the top (epispadias) or divided penis
  • narrow vaginal opening, wide labia, and short urethra

How is bladder exstrophy diagnosed?

Bladder exstrophy is usually diagnosed by fetal ultrasound before an infant is born. After the birth, exstrophy is determined by physical examination. Your child's doctor also may order other diagnostic procedures.

What is the treatment for bladder exstrophy?

Specific treatment for bladder exstrophy and epispadias is determined by your child's doctor based on:

  • your child's age, overall health, and medical history
  • extent of the disorder
  • your child's tolerance for specific medications, procedures, or therapies
  • expectations for the course of the disorder
  • your opinion or preference

After a diagnosis is made, your child will be referred to a surgeon. There are usually three stages to the surgical repair process that may start when your child is as young as 48 hours old.

  • Stage 1 involves internalization of the bladder and closing the abdomen.
  • Stage 2 is usually done between ages 1 and 2 to repair the epispadias and other genital abnormalities.
  • Stage 3 is done around age 3 and reconstructs the bladder and other structures of the urinary tract.