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Cloacal Exstrophy

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Cloacal exstrophy is a severe congenital defect of the lower abdominal organs,and occurs before birth. A baby with cloacal exstrophy is born with several abdominal structures outside of the body, including the bladder and part of the large intestine. In boys, the penis is usually flat and short with the inner surface of the urethra exposed on top. Sometimes the penis is split. Girls may have a split clitoris and may have one or two vaginal openings. The intestine may be short and typically there is no anus present.

Cook Children's pediatric urologists are known for their expertise in treating this very rare and complicated condition.

Who gets cloacal exstrophy?

Cloacal exstrophy is rare. It occurs once in 250,000 births and is slightly more common in males.

What causes cloacal exstrophy?

No one knows what causes cloacal exstrophy and there is no known way to prevent it. Nothing the mother did or did not do during pregnancy causes the condition. Experts do not know whether heredity is a factor in cloacal exstrophy because it is rare.

What are the symptoms of cloacal exstrophy?

Four components of cloacal exstrophy are frequently present:

  • The spleen, liver, intestines or other organs may protrude outside the abdomen.
  • The bladder is open in the front and the inner surface is exposed on the lower surface of the abdominal wall.
  • The anus is not fully formed and the colon may connect to the urethra, bladder or vagina.
  • The baby may have spinal complications such as spina bifida (when the vertebrae don't form correctly around the spinal cord).

How is cloacal exstrophy diagnosed?

Experts may see cloacal exstrophy before birth, during a routine ultrasound; doctors can then confirm the diagnosis at birth.

How is cloacal exstrophy treated?

Experts perform major reconstructive surgery in separate stages – often starting within the first few days of the baby's life.

Early surgeries return the bladder, intestines and other protruding organs into the body and repair any spinal abnormalities. Surgery can help with how the bowel, bladder and urinary system function, and can improve the way the sex organs and genitals look. Doctors may perform the surgeries over a number years. The timing of each depends on the child's condition and overall health.

What is the long-term outlook?

Medical science has made significant progress in helping children with cloacal exstrophy. It is a serious condition requiring many surgeries. How much the condition affects a child's life depends on the severity of the original condition. The long-term outcome is good for many children.

We're here to help.

If your child has been diagnosed, you probably have lots of questions. We can help. If you would like to schedule an appointment, refer a patient or speak to our staff, please call our offices.

Call 682-303-0376

Patient and family resources for cloacal exstrophy