Cloacal exstrophy

What abnormalities are seen in cloacal exstrophy?

Cloacal exstrophy is a very rare condition that affects the digestive, urinary and reproductive organs. A baby with cloacal exstrophy may be born with all or part of an organ, such as the intestines or bladder, located on the outside of the body.

They may have something called an omphalocele, where intestines protrude out in a sac where the belly button would normally be.

Babies with cloacal exstrophy are also born without an anal opening and have an abnormal pelvis shape and external genitals.

Many babies born with cloacal exstrophy also have spinal defects, such as spina bifida.

What are some of the problems that babies and children with cloacal exstrophy can encounter?

• Abdominal and pelvic organs being outside of the body can cause problems with heat and fluid loss.

• The bladder is exposed, split in half, and connected to the intestines when normally they should be separate. They would not be able to pee normally and urine and stool may mix.

• Without an anal opening, the baby would not be able to poop from the anus and will need an ostomy to manage their stooling, until later when an anal opening is created if feasible.

• Babies with cloacal exstrophy can be born with a shorter than normal colon and may have trouble absorbing adequate fluid and electrolytes.

• The abdominal wall defect and abnormal pelvic shape will affect motor development.

• Babies and children with cloacal exstrophy are at risk for urinary problems such as urinary tract infections, problems emptying urine causing back up of urine into kidneys, or incontinence (unable to control bladder). They can also have problems with bowel control, such as constipation and stool accidents.

• Many children with cloacal exstrophy also have problems growing and need special nutritional attention.

How is cloacal exstrophy diagnosed?

Sometimes cloacal exstrophy is detected during a prenatal ultrasound. You may need an MRI while you are still pregnant to obtain more information about your child’s condition. MRI is considered safe during pregnancy for both mom and baby. These tests help our teams prepare to care for your child as soon as they are born.

Once your child is born, physical examination by specialists will provide more information about their anatomy. But additional testing to check baby’s heart, spine, and kidneys will be needed.

What causes cloacal exstrophy?

Cloacal exstrophy results from abnormal development of the lower abdominal wall and pelvis as well as development of pelvic organs, but the cause is currently unknown. Doctors and scientists are conducting research to better understand how and why this condition happens.

How is cloacal exstrophy treated?

All children with cloacal exstrophy will need surgery to repair and/or reconstruct their organs. At Children’s Health, your child’s team of experts, including urologists, colorectal surgeons and gastroenterologists, will work together to create a treatment plan designed especially for them.

After birth, your child will stay in our highly advanced Level IV neonatal intensive care unit for around-the-clock care and support. Typically, within 48 hours of their birth, our specialized surgeons will perform surgery with the goal of returning their organs to the inside of their body and close up the opening in their abdomen. They may also separate any organs that are entwined and create a way for your child’s body to get rid of urine and stool.

Most children with cloacal exstrophy will need more surgeries over the first years of their life to reconstruct their pelvis and organs in stages. We perform as few surgeries as possible to help your child gain the ability to eat, drink, urinate and stool normally in the future. We’ll help you know the timeline of your child’s care and help you prepare for each step.

While your child is healing from surgery at the hospital, we’ll teach you how to care for their special needs, such as an ostomy bag (bag that collects stool and/or urine). We are here to support you so that when your child is strong enough, you are ready to welcome them home.

Your child will need multidisciplinary care from birth through adulthood. Our team of experts at the Colorectal and Pelvic Center, including colorectal surgeons, urologists, gastroenterologists, gynecologists, psychologists, and dieticians will be there to support you and your child.