Cloacal Exstrophy

Sometimes factors can occasionally interfere with bladder development, as is the case for children with cloacal exstrophy. If your newborn has been diagnosed with this condition, what can you expect? The following information should help you talk to your child's doctor if he/she has been diagnosed with cloacal exstrophy.

What is cloacal exstrophy?

This is the most severe birth defect in the exstrophy-epispadias complex. A child with this condition will have the bladder and a portion of the intestines, exposed outside the abdomen, with the bony pelvis 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. Also, frequently the intestine is short and the anus is not open. There is a high association with other birth defects, especially spina bifida, which occurs in up to 75 percent of cases. Omphalocele, a defect of the abdominal wall in the region of the umbilicus, is also common as are kidney abnormalities.

How often does it occur?

It is rare, occurring in approximately one in every 250,000 births and is slightly more common in males than females.

What caused this condition?

There is no known cause but it is also very unlikely that anything could have been done to prevent it.

How is it diagnosed?

Frequently it can be detected before birth during a routine sonogram. Nonetheless, this condition will be obvious at birth.

What is the treatment?

Surgical reconstruction is undertaken when the child is medically stable. The surgery is staged, but the schedule of surgery is very dependent on the individual child. The first surgical consideration is repair of any coexistent spinal abnormality, and perhaps repair of a large omphalocele. Once the child has recovered sufficiently from this, the gastrointestinal tract is then treated. A significant number of cases require a stoma because the colon is not normal, and the anus is not formed. Closure of the bladder and reconstruction of the genitalia are similar to that for classic exstrophy, although the procedure is sometimes staged because the pelvic bones are widely separated. In select cases the abdominal wall and genitourinary system can be repaired at the same time as the bowel. For a successful closure, a pelvic osteotomy (cutting the bones to allow the pelvis to close more easily) is mandatory. Achieving eventual continence almost always involves bladder reconstruction and using a catheter.

What can be expected after treatment?

The management of cloacal exstrophy has advanced to provide great improvement in the quality of life of affected children. With advances in pediatric anesthesia and infant nutrition the survival rate in the newborn is high and the incidence of life-threatening complications from surgery has reduced significantly. The child born with cloacal exstrophy can usually, with reconstructive surgery, achieve the ability to manage urine and stool in a socially acceptable way over time. The neurological deficit associated with spina bifida, if present, is manageable but requires ongoing medical services.

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. However, this will almost certainly require assisted fertility treatment.