How scared would a mother or father be if they saw that a newborn baby's abdomen had not formed properly and some of the organs that should be inside had come out? It's really hard to imagine. But don't worry. Although this is a very rare condition, there is a treatment for it. Let's talk about this condition called Cloacal Exstrophy in a simple way that you can understand.
What is this cloacal exstrophy?
Simply put, Cloacal Exstrophy is a birth defect that occurs when a baby's abdominal wall doesn't form properly while they're growing in the womb. Imagine, because the lower part of the abdomen doesn't close properly, the baby's bladder and part of the colon, or other organs, are covered by a small membrane and stick out of the body. This is really hard to see. This condition also affects the baby's genitals.
This is also known as a congenital urinary anomaly . This means that it is a problem that occurs while the baby is still in the womb. But don't worry, after a few corrective surgeries , starting at birth and lasting for a while, most children with this condition go on to live normal, happy lives.
How does this affect the baby? What is the OEIS complex?
Cloacal Exstrophy is also known as OEIS complex or OEIS syndrome because it is accompanied by several other problems. Let's see what they are:
What is Omphalocele?
This happens when the baby's abdominal muscles, near the belly button, are not properly joined, creating an opening. Through this opening, the bladder, large intestine, and sometimes other organs, such as the liver, protrude. But these organs don't just pop out, they are covered by a protective membrane.
What is exstrophy?
This is when the baby's bladder wall turns outwards instead of inwards. This is called bladder exstrophy . The bladder is flat instead of round, and may even be divided in two. Also, the abdominal muscles and pelvic bones, or the bones around the waist, do not connect properly. As a result, the pelvis is spread out on both sides, like a book that has been opened wide.
What is an imperforate anus?
In this case, the baby's anus, the opening through which we pass stool, has not formed properly. It may be blocked, or there may be no such opening at all. The colon is very short, and it may even be connected to the bladder.
What are spinal defects?
About three out of four babies with cloacal exstrophy also have a condition called spina bifida . This is when the bones of the spine don't close properly, causing the fluid-filled sac around the spinal cord (and possibly nerves) to protrude through the gap.
Important: A baby may have all of these, or only some of them. Doctors will carefully examine all of these.
Does this have any effect on the sexual organs?
Yes, Cloacal Exstrophy also affects the baby's genitals.
- If it's a boy: The penis may be flat, short, or divided into right and left sides.
- If it is a girl: The clitoris may be divided into two parts, one on the right and one on the left. There may also be two vaginal openings.
How common is this condition?
This is a very rare condition . Roughly speaking, it affects about one in 250,000 to one in 400,000 babies born. Also, boys are two to three times more likely to have this condition than girls.
What are the types of exstrophy-epispadius complex (EEC)?
Cloacal exstrophy is one of a group of birth defects called Exstrophy-Epispadias Complex (EEC) . In fact, it is the most severe type of the group. A baby can have just one condition in this group, or they can have several together.
Other conditions in the EEC category are:
- Epispadias: In this condition, the baby's urethra, the tube that carries urine out of the body, is not formed properly.
- Bladder exstrophy: In this condition, the baby's bladder turns inside out.
What causes cloacal exstrophy?
Doctors don't know exactly why some babies develop this condition, but they believe it happens when a tissue called the cloacal membrane tears or ruptures during the first trimester of pregnancy.
The cloaca is the area where the baby's reproductive system, digestive system, and urinary system come together. Normally, the organs of these systems are separated from each other by this cloacal membrane. So when this membrane ruptures, the abdominal wall does not fully form. The organs do not develop properly, and may even be connected to each other incorrectly. Eventually, some organs protrude through a gap in the abdomen.
What are the symptoms of this condition?
Symptoms of cloacal exstrophy can be seen immediately after birth. Your baby may experience the following:
- Organs have come out of a hole in the stomach, inside a sac-like structure.
- A sac-like structure protrudes from a gap in the back of the spine (this is Spina Bifida).
- The penis or vagina looks like it's split in two.
How do doctors detect this?
Cloacal exstrophy can often be diagnosed during a prenatal ultrasound scan during pregnancy. Your doctor may also recommend a fetal MRI scan. This can provide clearer images of your baby's organs. These scans can show things like:
- Urine does not flow properly from the bladder.
- The pubic bones are separated from each other.
- Smaller than normal size of the genitals.
- An omphalocele means that the umbilical cord is attached too low in the baby's abdomen.
After your baby is born, your doctor may order an MRI scan , an abdominal ultrasound , or other imaging scans . These can assess organ function and the severity of the disease. Using all of this information, your doctor will develop the best treatment plan for your baby.
How is Cloacal Exstrophy treated?
Every newborn with cloacal exstrophy will need surgery . This surgery involves putting the protruding organs back inside the body and closing the gaps in the abdomen and back. Your baby will likely have several surgeries over the course of several years. This is called staged reconstruction .
Birth travel treatment
Treatments like these begin as soon as the baby is born:
- Reconstructing the bladder, putting the organs back inside the abdomen, and closing the gap in the abdomen.
- Creating a stoma . This is a hole in the abdomen that allows stool to pass through from the large intestine. This stool is collected in a pouch on the outside (colostomy pouch). This colostomy pouch may be temporary, until other surgeries are performed.
- Closing the gap in the spine.
Treatment in early childhood
When the baby is a little older, that is, in early childhood, treatment is done like this:
- A bone surgery (osteotomy) is performed to cut the pelvic bones. This allows the pelvis to close more easily and provides better support for the bladder as the baby grows.
- The reproductive organs and urinary tract are being reorganized.
- An anal opening is created. If the colon is long enough, the doctor can perform a pull-through surgery to connect the colon to the anus. The stoma is then closed. The baby will no longer need a colostomy pouch.
What complications can arise from this condition?
After surgical treatment, most children with cloacal exstrophy live active, full lives . Some children may need more surgery as they grow older.
Cloacal exstrophy can cause the following risk conditions:
- Infertility.
- Kidney disease, kidney infections, kidney stones, and kidney failure.
- Sexual dysfunction, erectile dysfunction, etc.
- Urinary incontinence and fecal incontinence.
Things to ask your doctor
You can ask your baby's doctor things like:
- Is my baby at increased risk of other health conditions?
- What is the best treatment for Cloacal Exstrophy?
- How many surgeries will my baby need?
- What complications should I watch out for after surgery?
- What are the chances that my next child will also have Cloacal Exstrophy?
It's normal to feel scared and worried when you find out your baby has Cloacal Exstrophy, or OEIS syndrome. But remember, you're not alone.
The most important thing to remember
It's natural to feel overwhelmed and scared when you find out your baby has Cloacal Exstrophy. But don't worry . Your doctors and medical team are ready to help you and your baby. Your baby will need several surgeries right after birth, and then more surgeries to reshape the organs.
But the best thing is, with proper treatment, most children born with this condition live normal, happy lives . Talk to your doctor about any questions or concerns you may have. They will explain everything to you.
` Cloacal Exstrophy, Birth Defects, OEIS Complex, Omphalocele, Bladder Exstrophy, Pediatric Surgery
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