Description

Bladder exstrophy (EK-stroh-fee) is a rare condition present at birth. During pregnancy, the bladder of a baby is not born, also called a fetus, ways out of the stomach area. This area is also called the abdomen. The exposed bladder cannot store the urine or on the job as it should be. This causes the baby to leakage of urine after it is born.

Bladder exstrophy can also affect the genitals, stomach muscles, the bones of the pelvis, intestines, and reproductive organs. The cause of the disease is not clear, but the genes may play a role.

Bladder exstrophy can be seen on a routine ultrasound during pregnancy. But sometimes, the condition may not be seen until the baby is born. A baby born with bladder exstrophy needs surgery to close the bladder and the repair of other affected parts of the body as needed.

Symptoms

Bladder exstrophy can involve a wide spectrum of symptoms. The symptoms depend on which parts of the body are affected along with the bladder and stomach area, and the severity of the effects. Bladder exstrophy is the most common in a larger group of conditions present at birth called the bladder exstrophy-epispadias complex (EEC). Children with EEC have one of the following:

  • Epispadias. This is the least serious of the EEC. With an epispadias (ep-ih-SPAY-dee-us), the tube through which urine leaves the body does not develop fully. This tube is called the urethra.
  • Bladder exstrophy.This condition causes the bladder to form on the outside of the body. The bladder is also the other way around. Usually, bladder exstrophy is in the organs of the urinary tract, as well as the digestive and reproductive systems. The changes in the abdominal wall, bladder, genitals, and the bones of the pelvis that can happen. So what can be changes in the final part of the large intestine, called the rectum and the opening at the end of the rectum, the anus. Children with bladder exstrophy also has a condition that causes urine to flow the wrong way. This is called vesicoureteral reflux. The urine flows backward from the bladder into the tubes called ureters that connect the kidneys. Children with bladder exstrophy have epispadias as well.
  • Cloacal exstrophy.Cloacal exstrophy (kloe-AY-kul EK-stroh-fee) is the most severe form of the EEC. In this condition, the bladder and the intestine are exposed. The anus does not open and the intestine may be short. The penis or vagina may be divided. The bones of the pelvis are affected. The kidneys, the spine and the spinal cord may also be affected. Most children with cloacal exstrophy have spinal conditions, including spina bifida. Children who are born with protruding abdominal organs, probably, also have cloacal exstrophy or bladder exstrophy.

Bladder exstrophy. This condition causes the bladder to form on the outside of the body. The bladder is also the other way around. Usually, bladder exstrophy is in the organs of the urinary tract, as well as the digestive and reproductive systems. The changes in the abdominal wall, bladder, genitals, and the bones of the pelvis that can happen. So what can be changes in the final part of the large intestine, called the rectum and the opening at the end of the rectum, the anus.

Children with bladder exstrophy also has a condition that causes urine to flow the wrong way. This is called vesicoureteral reflux. The urine flows backward from the bladder into the tubes called ureters that connect the kidneys. Children with bladder exstrophy have epispadias as well.

Cloacal exstrophy. Cloacal exstrophy (kloe-AY-kul EK-stroh-fee) is the most severe form of the EEC. In this condition, the bladder and the intestine are exposed. The anus does not open and the intestine may be short. The penis or vagina may be divided. The bones of the pelvis are affected.

The kidneys, the spine and the spinal cord may also be affected. Most children with cloacal exstrophy have spinal conditions, including spina bifida. Children who are born with protruding abdominal organs, probably, also have cloacal exstrophy or bladder exstrophy.

Causes

The cause of the bladder exstrophy is not known. But the researchers believe that genetic factors may play a role.

What is known is that during pregnancy, a tissue that is called the cloaca (klo-AH-kuh) usually covers the wall of the baby before it is born bottom of the stomach. Later, it will be replaced by the muscles of the stomach. But if the sewer bursts before the muscles of the stomach form, bladder exstrophy may develop.

Risk factors

The factors that increase the risk of bladder exstrophy include:

  • The history of the family. The firstborn of the children, children of a father with bladder exstrophy or siblings of a child with bladder exstrophy have a greater chance of being born with the disease.
  • Of the race. Bladder exstrophy is more common in infants white than in Black or Hispanic babies.
  • The male sex. More boys than girls are born with bladder exstrophy.
  • Use of assisted reproduction in order to become pregnant. Children born through fertility treatments is known as assisted reproductive technologies have a higher risk of bladder exstrophy. These treatments include in vitro fertilization.

Complications

Bladder exstrophy can lead to other health problems called complications.

Without surgery

Without treatment, children with bladder exstrophy will not be able to retain the urine. This is called urinary incontinence. These children are also at risk of having problems with sexual function later in life. They have an increased risk of bladder cancer as well.

After the surgery

The surgery may reduce the risk of some complications. The success of the surgery depends on the severity of the condition. Many children who have surgical repair are able to retain the urine. Children with bladder exstrophy can walk with your feet turned slightly outward. This is due to the separation of the pelvic bones. Walking gets better with age.

The long-term complications

People who are born with bladder exstrophy can go to regular sexual function. That includes being able to have children. But pregnancy is often a high-risk to a pregnant person who had bladder exstrophy and to the unborn baby. A planned cesarean delivery, also known as C-section, may be necessary.

Diagnosis

The diagnosis involves the steps of a professional of the health care need to find the bladder exstrophy. But the condition is often found by chance during a routine pregnancy ultrasound. Other imaging tests such as magnetic resonance imaging can help confirm the finding. The signs of bladder exstrophy seen during the imaging tests are:

  • A bladder that is not fill or empty correctly.
  • An umbilical cord that is placed low on the stomach area.
  • Pubic bones — part of the hip bones to form the pelvis — that are separated.
  • Genitals that are smaller than is usual.

Sometimes, the condition may not be seen until after the baby is born. In a newborn, the health professionals looking for:

  • Size of the portion of the bladder is open and exposed to the air.
  • The position of the testes.
  • Intestine bulging through the abdominal wall, also called inguinal hernia.
  • Anatomy of the area around the navel.
  • The position of the anus.
  • How much of the pubic bones are separated, and the ease with the pelvis moves.

The Mayo Clinic has a state-of-the-art fetal care center that will help in the prenatal diagnosis and management of infants with surgical conditions, including the EEC. The Mayo Clinic's fetal care center has access to some of the most advanced fetal imaging, including high-resolution ultrasound and fetal MRI. Parents of infants with EEC can meet all your care team before their children are born.

Treatment

The treatment for bladder exstrophy is a surgery after birth. You may hear it called reconstructive surgery. The goals of the surgery are:

  • Provide sufficient space for the storage of urine.
  • Create external sexual organs that look and work acceptably.
  • Set the control of the bladder, also called continence.
  • Helps the kidneys to work as well as possible.

If the health professionals point of bladder exstrophy during pregnancy, in general, plans are made for the surgery immediately after birth. If the condition is found after the baby is born, the baby receives care in a special hospital unit. The baby may need to be transferred to a center that has experience in the treatment of bladder exstrophy. In any situation, health professionals cover the newborn of the bladder with a clear plastic dressing to protect it.

There are two main ways of bladder exstrophy surgery. It is not clear if a shape is much better than the other. The research is in progress to refine the surgeries, and the study of their long-term results. The two types of surgery are:

  • Complete repair.This surgery is called a complete primary repair of bladder exstrophy. A surgeon closes the bladder and stomach area, and repair of the urethra and external sex organs. This is only the surgery can be performed immediately after birth. Or can you do when the baby is around 2 to 3 months of age. The majority of the surgery for newborns includes the repair of the bones of the pelvis. But surgeons may choose not to do this repair if the baby is less than 72 hours of age, the pelvis, the separation is small, and the baby's bones are flexible.
  • Staged repair.The full name of this approach is modern staged repair of bladder exstrophy. Staged repair involves three surgeries. Often, the first surgery is performed within 72 hours after birth. The second is performed at the age of 6 to 12 months. And the last is 4 to 5 years. The first surgery closes the bladder and the stomach area. The second surgical repair of the urethra and the sexual organs. Then, when the child is old enough to start training the surgeons of repair of a group of muscles that are involved in the control of the bladder. This group of muscles is called the neck of the bladder.

Complete repair. This surgery is called a complete primary repair of bladder exstrophy. A surgeon closes the bladder and stomach area, and repair of the urethra and external sex organs. This is only the surgery can be performed immediately after birth. Or can you do when the baby is around 2 to 3 months of age.

The majority of the surgery for newborns includes the repair of the bones of the pelvis. But surgeons may choose not to do this repair if the baby is less than 72 hours of age, the pelvis, the separation is small, and the baby's bones are flexible.

Staged repair. The full name of this approach is modern staged repair of bladder exstrophy. Staged repair involves three surgeries. Often, the first surgery is performed within 72 hours after birth. The second is performed at the age of 6 to 12 months. And the last is 4 to 5 years.

The first surgery closes the bladder and the stomach area. The second surgical repair of the urethra and the sexual organs. Then, when the child is old enough to start training the surgeons of repair of a group of muscles that are involved in the control of the bladder. This group of muscles is called the neck of the bladder.

Surgical follow-up

Standard of care after the surgery includes:

  • Helps to stay still, also called immobilization. After the surgery, the children need to use devices that keep the lower part of their legs even during healing. The amount of time that a child needs for his stay varies. But it is usually around 4 to 6 weeks.
  • The management of pain. Health professionals can place a thin tube into the spinal canal during surgery to deliver pain medication directly to the area where it is needed. This gives the babies consistent for pain control and decreases the need for more potent pain relievers called opioid.

After the surgery, many children earn some bladder control. If your child had a complete primary repair of the surgery, your child will probably need to bladder neck surgery to improve bladder control. Sometimes, children need to have a tube inserted into the bladder to drain urine. This is called catheterization. More surgery may be needed to improve the urination or repair of the genital area as your child grows.

Coping and support

Having a baby with a rare, serious condition present at birth such as bladder exstrophy can be very stressful. It is difficult for health professionals to predict how the success of the surgery will be. So you're facing an uncertain future for your child.

Depending on the surgery of the result and the degree of control of the bladder after surgery, your child may face social challenges. For example, your child may have problems with body image. Your child may also be at risk of mental health problems such as anxiety and depression. A social worker or a mental health professional like a therapist can offer your child and your family support to cope with these challenges.

Some health professionals recommend that all children with EEC receive early counseling. It is also recommended that these children and their families to continue receiving support for mental health in adulthood.

You can also benefit from finding a support group of parents who are dealing with this condition. It can help to talk with other people who have had experiences like yours, and to understand what is happening.

It can also be useful to keep in mind that children with bladder exstrophy typical life expectancy. They also have a good chance of a fulfilling and productive life with the work, their relationships, and their own children. If necessary, fertility treatments, such as assisted reproductive technology can help to achieve pregnancy.

Preparing for your appointment

The health care professional you chose for your medical care during your pregnancy, you may have found your baby's condition. If so, it's likely that you keep seeing this professional of the health during pregnancy. You'll also likely meet with a team of doctors, surgeons and other specialists.

Here's some information to help you prepare for your appointment, and what to expect from your health care team.

What you can do

  • Be aware of all the instructions prior to your appointment. At the time of making the appointment, ask if there is something specific that you need to do to prepare.
  • Ask a family member or friend to go with you, if possible. Sometimes it can be difficult to remember all the information that your health care team gives you. Someone who joins you can remember something that you missed or forgot.
  • Write questions to ask their health care team. This can help you to make the most of their time with their health professionals.

For the bladder exstrophy, some basic questions to ask include:

  • What is the degree of the disease? You can tell how serious is it?
  • What can I do to my baby immediately after birth?
  • How many and what types of surgeries will my child need? What are some of the complications that can occur due to a treatment or surgery?
  • Will the condition cause long-lasting effects?
  • Are there any support groups that can help my child and me?
  • What are the chances of this happening again in future pregnancies? Is there any way to prevent this from happening again?
  • Are there brochures or other printed material I can have? What sites do you recommend?

Feel free to ask questions during your appointment.

What to expect from your doctor

Your healthcare provider will likely ask questions such as:

  • Have you ever had a child with bladder exstrophy or other conditions that are present at birth?
  • Someone in your family has been born with bladder exstrophy?
  • If necessary, you are able to travel to a medical center that offers specialized care?
Symptoms and treatment of Bladder exstrophy