Cloacal Exstrophy

Also known as: EC.

What is cloacal exstrophy?

Cloacal exstrophy is a serious birth defect in which the pelvic organs do not form properly. It is a very rare problem, occurring is every 300,000 live births. The colon, bladder and genital organs do not separate during development in the uterus. At birth, they remain fused and are open to the outside of the body, exposed on the lower abdominal wall. In addition, the pelvic bones and muscles do not form completely in front of the baby as they should.

What are the symptoms of cloacal exstrophy?

This anomaly is clearly visible at birth as a moist, red membrane (instead of skin) on the lower part of the abdomen, below the umbilical cord. Both stool and urine exit from the membrane. Often the penis and scrotum are divided, with half of each organ on one side of the membrane and half on the other. The physical symptoms of cloacal exstrophy are related to the intestinal tract, urological tract and genital tract. Early treatment is required to prevent infection and dehydration, and critical assessment with a team experienced with this unusual condition is required.

What are cloacal exstrophy care options?

The problems related to cloacal exstrophy include urinary, intestinal and sexual dysfunction. The malformation requires careful imaging and surgical planning with radiologic, orthopedic, pediatric surgical and urologic specialists.

Reconstruction entails a series of operations to restore the baby’s anatomy. This usually starts with a colostomy in the first days of life, perhaps accompanied by closure of the pelvic bones and bladder. All of these children require long-term, dedicated care, and a multidisciplinary team, such as the Nicklaus Children’s Hospital Colorectal Center, is best.

Many patients can have their colostomies closed after a rectal pull-through procedure. The urological tract is always affected, and patients may undergo bladder augmentation procedures, urinary conduits and other reconstruction to normalize the genitals under the guidance of specialized pediatric urologists.

What is the long-term outcome for children with cloacal exstrophy?

While these children require life-long follow-up, most lead productive lives with the assistance of their specialized medical team. The goal is to optimize intestinal function with a bowel management program, allow attain urinary continence and improve sexual function.

Reviewed by: Juan L Calisto, MD

This page was last updated on: November 11, 2020 01:22 PM

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