What is cloacal exstrophy?

Cloacal exstrophy is a rare and complicated condition that occurs during the prenatal development of the lower abdominal wall structures. Cloacal exstrophy occurs in 1 of every 250,000 births. A child with cloacal exstrophy is born with many inner-abdominal structures exposed. A portion of the large intestine lies outside of the body, and on either side of it are the two halves of the bladder. In males, the penis is usually flat and short and each penile half is separated. In females the clitoris is also separated into a right half and left half.

Cloacal exstrophy is also known as OEIS Syndrome because of the four features that are typically found together:

• Omphalocele: Some of the abdominal organs protrude through an opening in the abdominal muscles in the area of the umbilical cord. The omphalocele may be small, with only a portion of the intestine protruding outside the abdominal cavity, or large, with many of the abdominal organs (including intestine, liver and spleen) protruding outside the abdominal cavity.
• Exstrophy of the bladder and rectum: The bladder is open and separated into two halves. The rectum and colon are similarly open and the segment of the rectum is placed between the bladder halves on the surface of the abdomen.
• Imperforate anus: The anus has not been formed or perforated and the colon connects to the bladder.
• Spinal defects: These defects may either be major or minor. Often children born with cloacal exstrophy are also born with some degree of spina bifida.

Testing and diagnosis

Cloacal exstrophy can usually be diagnosed by fetal ultrasound before an infant is born. Upon birth, a physical exam will confirm the diagnosis.

Treatment of cloacal exstrophy

Cloacal exstrophy requires surgical repair. Your child will undergo a series of surgeries over a number of years — a staged reconstruction. The exact timing, nature and outcome of each cloacal exstrophy surgery will depend on your child's particular situation. Our surgeons will create a treatment plan based on the type and the extent of your child’s condition and discuss the plan with you.

Treatment may include:

Abdominal repair: Typically, soon after your child is born, the surgeons will repair the omphalocele by closing the bladder and creating a colostomy so your child can eliminate stool. With a colostomy, the large intestine is separated from the bladder halves and reclosed. The two halves of the bladder are brought together and placed into the abdomen. The end of the large intestine is brought to the surface of the skin through an opening in the abdomen. A plastic bag, called a colostomy pouch, is placed over the opening to collect the stool.

Other surgery, such as surgery to repair the spine, may be planned around the initial stage of the abdominal repair.

After the initial surgery, your child will remain in the hospital where we will monitor the intestine as it begins to function. Our team will work with you and your family to ensure that the plan for your child is clear and that you have access to the supports you need.

Osteotomies: Once your child has healed from his first procedure and had some time to grow, we will schedule the second stage of the repair. This primarily involves working on the bladder. The orthopedic surgeon on our team will perform osteotomies to help ensure that your child's pelvis can best support the bladder over time. During the osteotomy the hip bones are cut and adjusted. Your child will need to be in traction or in a spica cast for several weeks following this surgery.

Pull-through procedure: If your child was born with a significant amount of colon and is capable of forming solid stool, a surgical procedure, known as a "pull through" may eventually be performed. The purpose of this procedure is to connect the colon to the rectum.

Subsequent surgeries may also involve major urinary reconstructive surgery and further genital reconstruction. These issues will be discussed with you and your family as your child grows up.

Your child may be referred to the Pediatric Colorectal Program for long-term post-operative bowel management.

Reviewed by: Division of Urology