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Cloacal Exstrophy

  • A child with cloacal exstrophy is typically born with many intra-abdominal structures exposed. Although it is a serious condition, and requires a series of operations, the long-term outcome is good for many children.

    In cloacal exstrophy, a portion of the large intestine lies outside of the body, and on either side of it—and connected to it—are the two halves of the bladder. In boys, the penis is usually flat and short with the exposed inner surface of the urethra on top. The penis is sometimes split into a right and left half. In girls, the clitoris is split and there may be one or two vaginal openings.

    How does Boston Children's Hospital treat cloacal exstrophy?

    Cloacal exstrophy is corrected through a series of operations. Children’s physicians and surgeons have extensive experience in repairing cloacal exstrophy and supporting you and your baby throughout the process.

  • How common is cloacal exstrophy?

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

    What are the components of cloacal exstrophy?

    • Omphalocele — Some of the abdominal organs protrude through an opening in the lower abdominal wall 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, or large, with most of the abdominal organs (including intestine, liver, and spleen) protruding outside of the body.

    • Cloacal exstrophy — The bladder is open in the front and the inner surface is exposed on the lower surface of the abdominal wall. The exposed bladder appears as a right and left half, with the large intestine (rectum/ hindgut) exposed and connected in the middle. This abnormal connection of the intestine and the bladder allows mixing of stool and urine.

    • Imperforate anus — The anus has not been formed, and the colon connects to the bladder as detailed above.

    • 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.

    What causes cloacal exstrophy?

    The cause of cloacal exstrophy is unknown. It does not appear that any medications or activities of the parents or expectant mother have any impact on the development of cloacal exstrophy. Based on our current knowledge, it is very unlikely that this condition can be prevented.

  • How will my physician diagnose cloacal exstrophy in my child?

    In some instances, cloacal exstrophy can be diagnosed before birth with a prenatal ultrasound and may be confirmed by magnetic resonance imaging (MRI). After birth, this diagnosis may be confirmed or determined by physical examination.

    Other diagnostic tests and procedures can include:

    • computerized tomography scan (also called a CT or CAT scan)

    • magnetic resonance imaging (MRI)

    • endoscopy— insertion of a small instrument called an endoscope to view the interior of a hollow organ, such as the rectum, urethra or vagina

    • abdominal ultrasound (also called sonography)— an imaging method used to view internal organs as they function, and to assess blood flow

  • 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.

    Staged reconstruction

    This is a multi-step treatment involving several operations over a number of years. Advances in surgical techniques have enabled pediatric surgeons to minimize the number of stages involved. Another goal of current management is to decrease the number of stomas your child will need (discussed below), and in some instances eliminate the need for a stoma altogether.

    The exact timing, nature, and outcome of these procedures 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.

    The first days

    Within the first 24 to 48 hours after your child is born, surgery will likely be performed to repair the omphalocele, to return the protruding organs to the abdomen and close the opening in the abdominal wall.

    The surgeons will also close the bladder and create a way for your child to eliminate stool. The latter is done by creating 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 a collection bag. The lower section allows mucus that is produced by the intestine to pass into a collection bag. This allows for the normal separation of the urine and stool evacuation. Your baby's digestion will not be impaired after the colostomy.

    Your child may also need to have a catheter passed intermittently in order to help eliminate urine or a surgical procedure to reroute urine flow.

    Some spinal defects may also need to be repaired. These may be treated with surgery in the first months of life.

    Going home

    The next step would be to get your child healthy enough to go home. This will involve allowing your 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.

    We support you

    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 during this time.

    Subsequent surgery

    Depending on the amount of colon your child was born with and the nerve and muscle function in your baby's bottom, surgeons may eventually create a rectum and close the stoma. If your child has a significant amount of colon and is able to form solid stool, a surgical procedure known as a "pull through" may eventually be performed; it involves opening the abdomen to connect the colon to the rectum.

    Subsequent procedures 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. Years ago, unfortunately, many of these children died at birth or within the first days of life. Today, with advances in surgery, many 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.

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