Bladder exstrophy is a birth defect that affects the urinary system. It occurs when the bladder develops inside out and pushes outside the skin. Most babies with the condition need corrective surgery within the first few days of life. Children who receive treatment usually develop typically and don’t have activity restrictions.
Bladder exstrophy is a birth defect (congenital abnormality) where your bladder develops inside out. Your bladder is a small organ in the low abdomen (belly). Shaped like a balloon, it holds urine(pee) until you’re ready to go to the bathroom.
In newborns with bladder exstrophy, the skin and pelvis do not join correctly. As a result, the bladder may not close. It sticks out of the skin on the baby’s belly. The bladder may also be flat instead of round.
Bladder exstrophy affects a baby’s urinary system. Typically, when your bladder fills with urine, nerve signals tell your brain that your bladder is full. To urinate (pee), your bladder contracts (squeezes) and forces urine through your urethra and out of your body.
With bladder exstrophy, the cloaca (clo-AY-ka) doesn’t develop as it should. The cloaca is the area where the reproductive, digestive and urinary organs come together. Babies with bladder exstrophy empty urine through the opening in the abdomen instead of through the urethra.
Bladder exstrophy is one condition in a group of birth defects called bladder exstrophy-epispadias complex (BEEC). Some babies are born with one type of BEEC. Others have multiple BEEC conditions.
The other types of BEEC include:
Bladder exstrophy is rare. It affects around 1 out of every 50,000 newborns.
Experts don’t know why bladder exstrophy occurs. There is some evidence that environmental factors and genetics play a role.
Risk factors for bladder exstrophy include:
The main symptom of bladder exstrophy is the bladder protruding through an opening in the belly. Bladder exstrophy can lead to other symptoms, including:
In some cases, babies may also have inguinal or umbilical hernias. Hernias occur when part of the stomach lining (and sometimes intestines) bulges out of the abdominal wall. Hernias often need surgery to put organs in the correct place.
In a baby born with bladder exstrophy, their bladder often is sticking through the abdomen wall at birth. Providers may use MRIs or X-rays to confirm a bladder exstrophy diagnosis and check for other problems.
If a baby has epispadias, providers may detect it when the baby has frequent bladder infections or bladder control problems.
Sometimes, healthcare providers spot bladder exstrophy on an ultrasound or fetal MRI before birth. Signs that your baby may have bladder exstrophy include:
Babies born with bladder exstrophy need surgery to correct the condition.
Sometimes, babies have one surgery to close the abdomen and repair the urethra. Or babies may have surgery in three stages:
The type and number of surgeries your baby needs depend on how severe their symptoms are. Your baby’s surgeon will explain the best approach for your baby’s needs. Some children may need additional surgeries to achieve continence as they grow.
Because there is no clear cause of bladder exstrophy, parents can’t do anything to prevent it. Parents with known risk factors should speak with their healthcare provider about potential testing and treatment plans.
Most babies have good long-term outcomes if they receive surgery to correct bladder exstrophy. Some children have ongoing challenges with their bladder or kidneys, including:
Providers treat children for these issues as needed. Most children who receive treatment can do their usual activities without restrictions.
You may also want to ask your healthcare provider:
A note from Cleveland Clinic
Bladder exstrophy occurs when a baby’s bladder develops inside out and protrudes outside the skin. Babies may also have a small bladder, separated pelvic bones or undescended testicles. Usually, babies need surgery to put the bladder in its correct position and close the opening in their bellies. Depending on the severity of the condition, babies may need one surgery immediately after birth or a series of three surgeries. With timely treatment, children usually experience a high quality of life and take part in typical activities.
Last reviewed by a Cleveland Clinic medical professional on 09/09/2021.
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