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Bladder Exstrophy

Conditions

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Bladder exstrophy is a rare birth defect that occurs early in fetal development. The abdominal wall of the developing baby and its underlying organs don't properly join together. The infant is born with its bladder exposed on the outside of its body. This condition is complex and involves the urinary tract, reproductive tract, intestinal tract and musculoskeletal system. It also causes the bladder and the pelvis to form abnormally.

Bladder exstrophy affects about 1 in every 40,000 babies.

Patients can be seen by Texas Children's experts in Urology.

Causes & Risk Factors

The cause of bladder exstrophy is not known. Some studies suggest that it is inherited from the child's parents. The risk of having a child with bladder exstrophy is 1 in 70 if one or both parents have had the condition themselves. However, the chance for parents to have more than one child with bladder exstrophy is less than 1%.1

Symptoms & Types

Bladder exstrophy can cause a variety of complications and problems in a child. These may include:

  • infertility
  • need for repeated reconstructive surgery
  • urinary incontinence (involuntary urination)
  • vesicoureteral reflux (back-up of urine from the bladder to the kidneys)

Defects associated with bladder exstrophy can include:

  • epispadias (when the inner lining of the urethra is exposed on the penis in males, or between the labia in females)
  • shortened penis (in males)
  • clitoris that is divided into two parts (in females)
  • narrow vaginal opening, wide-spread labia and short urethra (in females)
  • widened and shortened pubic and other pelvic bones
  • displacement or absence of the belly button
  • outwardly rotated legs and feet
  • lower abdominal wall muscles that are spread to the side
  • displacement of the umbilical cord (often immediately above the bladder)
  • umbilical hernia (a bulge near the belly button caused by intestine, fat, or fluid that pushes through a weak spot)
  • anterior displacement of the anus (when the anus opens on the skin close to the scrotum in males, and close to the vagina in girls)

Diagnosis & Tests

Bladder exstrophy can be diagnosed immediately at birth since the bladder is visible outside the body. In some cases, the condition can be discovered before the baby is born by using ultrasound. Prenatal diagnosis of bladder exstrophy should be considered any time the bladder is not visible in the ultrasound, or if the fetus shows any of the following signs:

  • lower abdominal bulge
  • small penis with displaced scrotum
  • low set umbilical insertion
  • abnormal widening of the iliac crest (the curved ridge at the top of the pelvic bone)

Treatment & Care

Treatment for bladder exstrophy begins at birth. The most important things to do once bladder exstrophy is discovered are to:

  • preserve normal kidney function
  • develop healthy bladder function with normal urine flow
  • provide acceptable appearance and function of the external genitalia
  • ensure that the child has a typical and normal childhood

Immediately following birth, your child's exposed bladder should be covered with a non-adherent film (plastic wrap) to prevent sticking to diapers or clothing. With each diaper change, the plastic wrap should be removed. The exposed bladder should be irrigated with sterile saline and a new, sterile wrap should be applied.

Your child should be transferred to a pediatric hospital for management of bladder exstrophy as soon as possible.  Surgical treatment involves reconstruction of the deformity. This can include:

  • closing the bladder
  • repairing the penis
  • prevention of urine leakage

Living & Managing

Your child may need a range of surgical procedures and tests. In many cases, additional surgeries are required during childhood, adolescence and young adulthood. Your child may require great emotional and psychological support. 

Even with successful surgery, patients may have long-term problems with the following:

  • ability to hold in urine
  • urinary reflux (back-up of urine to the kidneys)
  • repeated urinary tract infections (UTIs)
  • bladder or colonic adenocarcinoma (cancer that develops in the glandular tissues of the body)
  • uterine prolapse (the uterus slips down into or protrudes out of the vagina)
  • self-image

Fortunately, sexual function and libido remain normal in bladder exstrophy patients. Successful pregnancies and delivery are possible.

More Information & Related Links

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Links

Articles

1 Columbia University Medical Center. "Pediatric Urology Library: Exstrophy of the Bladder."