Bladder Exstrophy and Epispadias Symptoms & Causes

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What is bladder exstrophy?
Bladder exstrophy is a complex combination of disorders that occur while a baby is still developing (in utero). If the abdominal wall and underlying structures don't properly join together, a baby can be born with the bladder and other structures exposed on the outer surface of the body.

Bladder exstrophy usually involves several systems within the body, including the urinary tract, reproductive tract (external genitalia) and pelvic skeletal muscles and bones. In rare cases, intestinal tracts are involved.

The most commonly associated defect is epispadias, a malformation in which the inner lining of the urethra is exposed and visible on the top surface of the penis (in boys) or between the labia (in girls).

Other associated defects include:

  • displacement of the belly button, usually immediately above the defect and lower than normal on the abdominal wall
  • narrow vaginal opening, wide-spread labia and short urethra in girls
  • outwardly rotated legs and feet

What causes exstrophy of the bladder?

The cause of bladder exstrophy is not known. Some studies show a clustering of the condition in families, suggesting that there’s an inherited factor. However, the chance for parents to have another child with exstrophy of the bladder is less than 1 percent.

How is it diagnosed?

Because the bladder and other structures are exposed on the outer surface of the body, bladder exstrophy can be diagnosed immediately at birth. In some cases, however, the condition is discovered before the baby is born through images produced by a fetal ultrasound or other imaging techniques such as magnetic resonance imaging (MRI).

How is it treated?

There are several options for initial repair of bladder exstrophy, including complete primary repair of exstrophy (CPRE). With this technique, the bladder is closed and the epispadias is repaired (entire urethra is closed into a tube) at the same time. This “primary closure technique” is the preferred approach at Boston Children’s.

Treatment for exstrophy of the bladder begins at birth. The most important goals in the care of a boy or girl with bladder exstrophy are to:

  • preserve normal kidney function
  • develop adequate bladder function and promote urinary continence
  • provide acceptable appearance and function of the external genitalia
  • ensure that your child has a typical and normal childhood

What is the long-term outlook for bladder exstrophy patients?

After initial treatment for bladder exstrophy, care is ongoing and can involve a range of surgical procedures and tests. In many cases, additional surgeries are required during childhood, adolescence and young adulthood. Moreover, bladder exstrophy patients may require emotional and psychological support to manage their conditions.

For information on support services at Boston Children’s, visit the Bladder Exstrophy Program.

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- Sandra L. Fenwick, President and CEO

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