Cloacal Exstrophy

What is cloacal exstrophy?

When a baby is born with certain inner abdominal organs and structures exposed on the outside of the abdomen, along with some malformation of these organs, this is called cloacal exstrophy.

Some common characteristics of cloacal exstrophy are:

A large portion of the intestines may lie outside the body, protruding through the umbilical cord area (omphalocele)
On either side of the exposed intestines are the two halves of the bladder
In males the penis may be flat and short, and each penile half may be separated
In females the clitoris may be separated into a right and left half

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Cloacal exstrophy is also known as OEIS

When a baby is born with cloacal exstrophy, part of the intestines may be outside of the body (called omphalocele) and a portion of the small bowel called the terminal ileum may also stick out further, called prolapse. The small bowel (prolapse) comes out into the opened beginning part of the colon called the cecum. This means that poop does not come out of the anal opening (hindgut). The bladder may be outside of the body and in two pieces (called hemibladder). Urine (pee) drains directly onto the bladder plates through the ureters.

OEIS stand for a combination of conditions that are often present together:

Omphalocele – Some of the abdominal organs protrude through the umbilical cord area. The omphalocele may be small – involving only some intestines – or may be large and contain other abdominal organs such as the liver.
Exstrophy of the bladder and rectum – The bladder is open and separated into two parts. The colon and rectum may also be divided.
Imperforate anus/anorectal malformation – The anal open may be in the incorrect location or be missing entirely.
Spinal defects – These may be minor or major and may include spina bifida, which is the incomplete closure of a section of the spine and membranes surrounding the spinal cord.

When and why does it occur?

Cloacal exstrophy is a very rare congenital defect that happens early in pregnancy, while a baby is still developing in the uterus. It is believed to occur in approximately 1 of 250,000 births.

There is no one specific cause of cloacal exstrophy. Some patients may have a family history of cloacal exstrophy indicating a genetic component. Others have no family history. No environmental factors have been identified as contributing to cloacal exstrophy.

How is this condition diagnosed?

Often, cloacal exstrophy is diagnosed prenatally via sonogram; however, sometimes this condition is not discovered until a baby is born.

Your doctor will order several tests to better understand your baby’s specific type of malformation and develop a plan.

These tests may include:

Abdominal X-rays
Abdominal ultrasound to evaluate the kidneys, urinary structures and how urine (pee) is draining
Spinal ultrasound or spinal MRI to evaluate for tethered spinal cord
Echocardiogram (ECHO) to look for heart defects

What is the treatment for cloacal exstrophy?

Children's Mercy is an American College of Surgeons Verified Center

Cloacal exstrophy requires a series of surgeries, also known as ‘staged reconstruction’. These will be planned and prioritized based on the types and severity of malformations listed below.

Priority is to make sure:
- Poop can exit the body safely
- Urine can exit the body safely
- Organs such as intestines which may be exposed and visible are protected

What are some of the surgeries for cloacal exstrophy?

During primary omphalocele surgery, the abdomen is closed (midline closure) with the intestines inside. Part of the colon is directed to a hole in the abdomen to allow poop to exit the body (colostomy). The two halves of the hemibladders are moved closer together.

Abdominal repair: The first stage of surgery may also be called omphalocele closure. The abdominal wall may not be able to close completely and may be closed in stages. Often when the abdominal wall is closed, the two sides of the bladder are brought closer together. The end of the colon is located and a colostomy is made which lets poop exit your baby’s body. During this surgery, the urology team evaluates if urine can exit the body safely as well as moves the open bladder halves closer together (called bladder exstrophy) During this stage, the urology team also makes sure that there is a way to safely urinate and monitor the urinary system.

Epithelial closure happens when skin and scar tissue grows over the omphalocele. This is an option when the omphalocele has too many organs within it to bring normal skin across.

After complete closure, the bladder will be tucked into the abdomen and will empty through an opening in the tummy wall (vesicostomy). There is a hole on the side of the abdomen that allows poop to exit the body (colostomy).

Osteotomies: Once your child has healed, the second stage of surgery involves working on the bladder (and sometimes the abdominal wall if it is not completely closed). The bladder is brought together by a urologist and tucked into the abdomen. The orthopedic surgery team helps to bring the pelvic bones closer together (by performing a procedure called an osteotomy) as they can be further apart. Children who need osteotomies may be placed in traction or a spica cast for a few weeks after surgical repair.

Pull-through procedure: Many families wonder if their child will have the ability to poop out of their bottom instead of having a colostomy bag. This reconstruction is usually done later. The surgery involves having poop come out of the bottom instead of into a colostomy bag. This surgery depends on the amount of colon and the thickness of your child’s stool. Our team can help assess if your child can have this surgery.

Spinal surgery: Depending on the spinal development of your child, spinal surgery may be required. A neurosurgeon performs this surgery.

Additional surgeries: Children are followed life-long to ensure the health of the bladder, kidneys, bowel, spine, heart and reproductive organs. There will be additional testing to see how these organs are doing during your child’s growth and development. Additional treatment, surgeries and procedures are commonly recommended to help improve your child’s quality of life and independence.

What are cloacal exstrophy care options?

Because cloaca exstrophy involves multiple body systems, children do best when their care is provided by a coordinated team of experts in colorectal surgery, gynecology, urology, orthopedic surgery, neurosurgery and radiology, such as is available at the Children's Mercy Comprehensive Colorectal Center.