Cloacal Exstrophy (OEIS Sydrome)| Diagnosis & Treatment

How is cloacal exstrophy diagnosed?

In some instances, cloacal exstrophy (OEIS Syndrome) can be diagnosed before birth with a prenatal ultrasound, which may be confirmed by magnetic resonance imaging (MRI). After birth, your baby’s doctor can make or confirm this diagnosis with a physical examination.

Other diagnostic tests and procedures can include:

  • Magnetic resonance imaging (MRI)
  • Examination under anesthesia and endoscopy. This involves the 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 (sonography). This imaging method is used to view the anatomy of the 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 our team of surgeons to collaborate on the reconstructive process to minimize the number of stages involved.

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.

Abdominal repair

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 work as a team and include a pediatric urologist and a pediatric colorectal surgeon.

If possible, the surgeons will separate the bladder from the bowel, potentially close the bladder and create a way for your child to eliminate stool. Once the bowel is separated from the bladder, it is made into a tube. Usually, any other bowel that is present is put into continuity with this tubularized bowel (a procedure called a Hindgut rescue). Usually, an end stoma is created so that stool from the intestines 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 ostomy.

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.

If you are visiting us for a second opinion after the initial surgery, sometimes a hindgut rescue procedure maybe needed. Our team will do a careful examination under anesthesia with possible scoping procedures to determine if further surgery is necessary.

After treatment

The next step is to get your child healthy enough to go home. This involves 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.

The nursing staff and other health care 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. This surgery involves opening the abdomen to connect the colon to the rectum.

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