Cloacal Exstrophy

What is cloacal exstrophy?

Cloacal exstrophy affects the formation of the lower abdominal wall of the developing fetus. Because the wall doesn’t form properly, the baby’s bladder and part of the large intestines (colon) are enclosed in a membrane on the outside of the body after birth. The condition also affects a child’s genitals.

Cloacal exstrophy is a type of birth defect or congenital urinary anomaly. This means the condition occurs while a baby is developing in the womb and is present at birth. After a series of corrective surgeries that begin shortly after birth, most children with cloacal exstrophy do well and lead full lives.

How does cloacal exstrophy affect a child?

Cloacal exstrophy is also known as OEIS complex or OEIS syndrome because it causes these problems:

• Omphalocele: An opening (omphalocele) in abdominal muscles near the belly button allows the bladder and large intestine (and sometimes other organs) to protrude outside the body. The organs are inside a membrane.
• Exstrophy: The bladder wall turns outward (bladder exstrophy) and is flat instead of round. It may be split in half. The abdominal muscles and pelvic bones don’t join correctly, causing the pelvis to splay open like a book.
• Imperforate anus: The opening to the anus, the last part of the digestive system where poop comes out, is blocked or nonexistent. The large intestine (colon) is too short and may connect to the bladder.
• Spinal defects: As many as 3 out of 4 infants with cloacal exstrophy also have spina bifida. The spinal cord doesn’t close, allowing a sac of fluid (and sometimes nerves) to bulge through the opening.

How does cloacal exstrophy affect a child’s genitals?

Cloacal exstrophy also affects a child’s genitals.

• The penis in the male reproductive system may be flat, short or split into a right and left half.
• The clitoris in the female reproductive system is split into a right and left half. There may be two vaginal openings.

How common is cloacal exstrophy?

Cloacal exstrophy is rare, occurring in approximately 1 in 250,000 to 400,000 births. Male babies are two to three times more likely to have this condition.

What are the types of exstrophy-epispadias complex (EEC)?

Cloacal exstrophy is one condition in a group of birth defects called exstrophy-epispadias complex (EEC). It is the most severe type. A baby may have one condition or several.

Other EEC conditions include:

• Epispadias: A baby’s urethra (the tube that carries urine out of the body) doesn’t form properly.
• Bladder exstrophy: A baby’s bladder develops inside out.

What causes cloacal exstrophy?

Healthcare providers don’t know why some babies develop this condition. Something happens during the first trimester of pregnancy that causes a tissue known as the cloacal membrane to tear or rupture.

The cloaca (clo-AY-ka) is the area where the reproductive, digestive and urinary systems come together. The cloacal membrane typically separates the organs in these systems. When there’s a rupture, the abdominal wall doesn’t form completely. Organs don’t develop as they should or may connect incorrectly. Some organs poke through an abdominal opening.

What are cloacal exstrophy symptoms?

The signs of cloacal exstrophy are evident at birth. Your baby may have:

• Organs in a pouch poking through an opening in their belly.
• A sac protruding through an opening in their back (spina bifida).
• Penis or vagina that is split.

How is cloacal exstrophy diagnosed?

A prenatal ultrasound often detects cloacal exstrophy during pregnancy. You may also get a fetal MRI, which provides more detailed images of fetal organs. These tests may reveal:

• The bladder isn’t emptying properly.
• Separated pubic bones (the bones that form the pelvis).
• Small genitalia.
• Umbilical cord that connects too low on the fetus's belly due to the omphalocele.

After birth, your baby may get an MRI, abdominal ultrasound or other imaging scans to assess organ function and disease severity. This information helps your provider develop the best treatment plan for your child.

How is cloacal exstrophy managed or treated?

All newborns with cloacal exstrophy need surgery to put their organs into their proper place and close abdominal and spinal openings. Your child will need a series of surgeries over many years. This is known as staged reconstruction.

Treatments soon after birth may include:

• Fixing the bladder, placing organs back into the abdominal cavity and closing the opening in the abdomen.
• Creating a stoma, an opening in the stomach that allows the large intestine to empty stool into a pouch outside the body. A colostomy pouch may be temporary until other surgeries can take place.
• Closing the hole in the spinal cord.

Treatments during early childhood may include:

• Cutting the pelvic bones (bone surgery or osteotomy). This allows the pelvis to close more easily and support the bladder as your child grows.
• Reconstructing the reproductive organs and urinary tract.
• Creating an anal opening. If there’s enough large intestine, your surgeon may perform a procedure called pull-through surgery to connect the large intestine to the rectum. The surgeon then closes the stoma. Your child will no longer need a colostomy pouch.

What are the complications of cloacal exstrophy?

After surgical treatments, most children with cloacal exstrophy live active, full lives. Some children will need additional surgeries as they mature.

Cloacal exstrophy increases the risk of:

• Infertility.
• Kidney disease, kidney infections, kidney stones and kidney failure.
• Sexual dysfunction, including erectile dysfunction.
• Urinary incontinence and fecal (bowel) incontinence.

What should I ask my provider?

You may want to ask your child’s healthcare provider:

• Does my child have a higher risk of any other health conditions?
• What is the best treatment for cloacal exstrophy?
• How many surgeries will my child need?
• What complications do I need to watch for after surgery?
• What are the chances that another child of mine will have cloacal exstrophy?

A note from Cleveland Clinic

Learning that your child has cloacal exstrophy, or OEIS syndrome, can make you feel scared and worried for your child's future. Your healthcare team will help plan several surgeries for your child soon after birth, with additional corrective surgeries to reconstruct organs will take place later on. With appropriate treatment, most children born with cloacal exstrophy enjoy full lives. Talk to your healthcare provider about any concerns you have.