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, exstrophy of the bladder and rectum, imperforate anus, and spinal defects.
Cloacal exstrophy can usually be diagnosed by fetal ultrasound before an infant is born. Upon birth, a physical exam will confirm the diagnosis.
Why Choose Us?
The Division of Urology at Children's Hospital of Philadelphia (CHOP) is a world leader in pediatric urology, offering specialized care for a wide range of urologic disorders in children and adolescents.
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:
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. 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 you have access to the supports you need.
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.
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.