The treatment of cloacal exstrophy is similar to that of bladder exstrophy in the aspect that the condition requires a series of surgical operations to repair and restore the malformations. The goal, as always, is to attempt to restore normal bladder and bowel function while trying to achieve a balance of appropriate reconstruction and cosmetic appearance.
Multiple surgeries are always required, which we understand can be scary to hear as new parents, but rest assured that advances in technology and surgical techniques have allowed surgeons the ability to minimize the number of surgeries a child will require. The multiple staged surgeries usually aim to achieve the following:
- The separation of the urinary and the digestive tracts is accompanied by the repair of the bladder and rescue of the colon. In addition, the intestines are put back into place, and the abdominal wall is closed.
- Is a hip readjustment to close the pelvic ring and bring the anus and the urethra closer to their usual positions. During this procedure, the accompanying spinal defect (spina bifida) is usually repaired, as well.
- Try to restore normal emptying of urine and stool through their respective pathways.
It is important to understand that successful full restoration can vary among children and depends upon many different underlying factors (ex: how much bowl the child was born with and can be rescued). Furthermore, children with cloacal exstrophy will require a lifetime of follow-up care to ensure proper kidney function and avoid bowel obstructions. Lastly, parents should recognize that there is no specific blueprint for every child with cloacal exstrophy, and some children are going to require additional surgeries and care to achieve the best possible results.
Ways to treat bladder exstrophy?
Cloacal exstrophy requires surgical repair. The patients 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 the patient’s particular situation. Surgeons create a treatment plan based on the type and the extent of your child’s condition and discuss the plan with their parents
Abdominal repair
Typically, soon after the patient is born, the surgeons repair the omphalocele by closing the bladder and creating a colostomy so the patient 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, patients remain in the hospital, where surgeons monitor the intestine as it begins to function.
Bladder repair
This may be done as a single-stage procedure where the two bladder halves are closed and placed into the bladder. The bladder repair may also be done with delayed bladder closure, occurring anywhere from 3 months to 2 years old, allowing the bladder plates to grow and the bones of the pelvis to mature to allow for a stronger osteotomy.
Osteotomies
Once a patient has healed from his first procedure and had some time to grow, doctors schedule the second stage of the repair. This primarily involves working on the bladder. The orthopedic surgeon on the team performs osteotomies to help ensure that the patient’s pelvis can best support the bladder over time. During the osteotomy, the hip bones are cut and adjusted. The patient needs to be in traction or in a spica cast for several weeks following this surgery.
Pull-through procedure
If the baby 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.
As patients age, vaginal or penile reconstruction surgery may occur to repair physical abnormalities in the genitals.