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Cloacal Exstrophy

Cloacal Exstrophy

What is cloacal exstrophy?

Cloacal exstrophy is a rare and complicated defect that occurs during the prenatal development of the lower abdominal wall structures. (The cloaca is the part of an embryo that develops into these structures) A child with cloacal exstrophy is born with many inner-abdominal structures exposed. A portion of the large intestines 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, sometimes split in half. In females the clitoris is split. Oftentimes in cloacal exstrophy there may be two vaginal openings. The following are descriptions of the components that make up cloacal exstrophy:

Omphalocele: in this defect, some of the abdominal organs protrude through an opening in the abdominal muscles in the area of the umbilical cord. A translucent membrane covers the protruding organs. The omphalocele may be small, with only a portion of the intestine protruding outside the abdominal cavity, or large, with most of the abdominal organs (including intestine, liver, and spleen) present outside the abdominal cavity.
Extrophy of the cloaca: the bladder is open in half and the rectum is not open in the normal location, but rather communicates with the bladder.
Imperforate Anus: the anus has not been formed or perforated, and the colon communicates with the bladder. See anorectal malformations
Spinal defects: these defects may either be major or minor. Many times children born with cloacal exstrophy are also born with varying degrees of spina bifida.

How common is cloacal exstrophy?

Cloacal exstrophy is a very rare birth defect. It occurs once in every 250,000 births.

How is cloacal exstrophy diagnosed?

Cloacal exstrophy can usually be diagnosed by fetal ultrasound before an infant is born. After the infant is born, exstrophy can be determined by physical examination. Other diagnostic procedures can include:

computerized tomography scan (Also called a CT or CAT scan.) - a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called "slices"), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.
Magnetic Resonance Imaging (MRI) a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.
abdominal ultrasound (Also called sonography.) - a diagnostic imaging technique which uses high-frequency sound waves and a computer to create images of blood vessels, tissues, and organs. Ultrasounds are used to view internal organs as they function, and to assess blood flow through various vessels.
7 endoscopy: a diagnostic procedure in which doctors use a small instrument called an endoscope for visualization of the interior of a hallow organ, such as the rectum, urethra or vagina.

How is cloacal exstrophy treated?

Cloacal exstrophy requires surgical repair. The treatment plan devised for your child will depend on the type and the extent of the abnormality.

Treatment involves a series of surgeries over a number of years. This plan is called staged reconstruction. Advances in surgical techniques have enabled pediatric surgeons to minimize the number of stages involved, with the goal of minimizing the number of stomas (discussed below) needed and in some instances eliminating the need for a stoma altogether. The exact timing, nature, and outcome of these surgeries will depend on your child's particular situation. Your surgeon will discuss with you the plan for your child and how successful you can expect it to be.

Soon after your child is born, within the first 24 to 48 hours, surgery will likely be performed to repair the omphalocele. The surgeons will close the bladder and create a way for your child to eliminate waste. The latter is done by creating a colostomy. With a colostomy, the large intestine is divided into two sections and the ends of intestine are brought through openings in the abdomen. The upper section allows stool to pass through the opening (called a stoma) and then into in a collection bag. The lower section allows mucus that is produced by the intestine to pass into a collection bag. By doing a colostomy, the baby's digestion will not be impaired. Your child may also need to have a special appliance that is surgically implanted to help eliminate urine. Any spinal defects may also be repaired at this time.

The next step would be to get your child healthy enough to go home. This will involve allowing the child to heal from the operation. It may also involve allowing your child to grow if he or she was born prematurely and making sure you are prepared to take care of your child's extra needs both physically and emotionally.

The nursing staff and other healthcare professionals that work with your baby's surgeon can help you learn to take care of the colostomy. Local and national support groups may also be of help to you during this time.

Subsequent surgeries

Whether or not surgeons eventually create a rectum and close the stoma at a later time depends on the amount of colon the child was born with. 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" will eventually be performed. For this procedure, the surgeon opens the abdomen to connect the colon to the rectum.

Subsequent surgeries will also involve major urinary reconstructive surgery and further genital reconstruction.

What is the long-term outlook for a baby born with cloacal exstrophy?

Medical science has come a long way in helping children born with cloacal exstrophy. Prior to the last decade, many of these children died at birth or within a few days of birth. Nowadays, with advances in surgery, these children not only survive and thrive, but also can look forward to leading productive lives. How "normal" a life your child will have depends on the severity of the condition.


		Programs that treat this condition:

		Advanced Fetal Care Center
		Center for Bladder Exstrophy Care & Support Group


					Children's Hospital Boston is the primary pediatric teaching hospital of Harvard Medical School

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