What is Cloacal Exstrophy?

Sometimes the bladder doesn’t develop properly, as is the case for children with cloacal exstrophy. This is the most severe birth defect of the lower abdominal organs.

A child with cloacal exstrophy is born with the bladder and a part of the intestines open to the outside. The bony pelvis is also split open like a book. In males the penis is either flat and short or sometimes split. In females the clitoris is split and there may be two vaginal openings. The intestine may be short and the anus may not open.

With this condition there are often other birth defects, like spina bifida. This occurs in up to 75 percent of cases. Kidney abnormalities and omphalocele are also common. An omphalocele is when an infant's intestine or other abdominal organs are open to the outside the body. This is from a hole in the belly button (navel) area. The intestines are covered only by a thin layer of tissue and can be easily seen.

If your newborn has been diagnosed with this condition, there is a lot to learn. The information here should help you talk with your child's doctor and plan for the future.

How often does it occur?

This is a rare condition. It occurs in about one in every 250,000 births. It is slightly more common in males than females.



What Causes Cloacal Exstrophy?

There is no known cause, so there is also no known way to prevent it.



How is Cloacal Exstrophy Diagnosed?

It may be seen before birth, during a routine sonogram. This condition is obvious at birth.



How is Cloacal Exstrophy Treated?

As soon as possible, surgical reconstruction is done. Surgery is major, and often done in parts. The schedule of surgery depends on the child’s condition and overall health. Surgery can return the bladder and bowel organs back into the body, to a healthy position. It can provide ways for bowel and urinary control, better kidney function, and improve the way the sex organs or genitals look.

What is the Treatment for Cloacal Exstrophy?

Reconstruction surgery often starts within the first few days of life. It is sometimes delayed to allow the baby to grow and develop. Surgical repair is generally divided into steps and include:

  • Repair of spinal abnormalities, and if needed, the repair of a large omphalocele.
  • Once the child has recovered from spinal surgery, the gastrointestinal tract is treated. Many babies require a stoma because the colon is not normal, and the anus is not formed. The stoma will allow for waste to be released from the intestines to a pouch on the outside of the body.
  • Closure of the exposed bladder and bowel and reconstruction of the genitals are next. This may be done in steps if the pelvic bones are widely separated. For a successful closure, a pelvic osteotomy (cutting the bones to allow the pelvis to close more easily) is critical. In some cases, the abdominal wall, the bladder and genitals (genitourinary system) and the bowel may be repaired at the same time. Bladder reconstruction often includes the use of a catheter for some time.


What can be Expected After Treatment?

The goal of surgeons and doctors is to help improve the child’s quality of life. Better tools for anesthesia and infant nutrition have helped to increase the survival rate for newborns with this condition.

It’s important to work closely with your health care team to prevent infection after surgery, and learn about long-term care. After surgery, a child born with cloacal exstrophy can usually grow to manage urine and stool in a socially acceptable way. Further operations may be needed over time to improve the child’s ability to control their bladder and bowel function. More surgery may also be needed to rebuild and/or make better the outer sex organs.

Time and patience will be important for the parents and child. Neurologic issues from spina bifida, if present, can be managed, but requires ongoing medical care.



Frequently Asked Questions

Will my child be able to have children when they reach adulthood?

In many cases, the answer to this question is: yes. But almost always, assisted fertility is necessary for adults.