What is Bladder Exstrophy?

Bladder exstrophy is a birth defect and is a condition in which the bladder forms from the inside out. You can find more information below.

What is bladder exstrophy?

Bladder exstrophy is a rare birth defect in which the bladder develops outside the fetus. In exposed persons, the bladder cannot store urine normally and may cause leakage of urine (incontinence) .

Problems caused by bladder exstrophy vary in severity. It can include defects in the bladder, genitals, and pelvic bones, as well as defects in the intestines and reproductive organs.

Whether the unborn baby has such a problem can be detected during a routine ultrasound during pregnancy. However, sometimes the defect does not appear until the baby is born. Babies born with this disease need surgery to correct the defects.

What causes bladder exstrophy?

Doctors aren’t sure what develops bladder exstrophy. Researchers think a combination of genetic and environmental factors likely play a role.

What is known is that as the fetus grows, a structure called the cloaca , where the reproductive, urinary and digestive systems come together, does not develop properly in infants who develop bladder exstrophy. Defects in the cloaca may vary depending on the condition of the fetus when the developmental error occurs.

Who is at risk?

Factors that increase the risk of bladder exstrophy include:

  • Family history: If the mother, father, siblings or close relatives have this disease, there is always the possibility of passing it on to the child.
  • Skin colour: Bladder exstrophy is more common in whites than in other races.
  • Gender: More boys than girls are born with this birth defect.
  • Use of assisted reproduction: Children born with assisted reproductive technology such as IVF are at higher risk than others.
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What are the symptoms of bladder exstrophy?

Bladder exstrophy is most common in the larger group of birth defects called the bladder exstrophy-epispadias complex (MEEK) . If your child has MEEK , they will have one of the following:

  • Epispadias: This is the least serious form in which the tube to pass urine is not fully developed.
  • Bladder exstrophy: This defect causes the bladder to form outside of the body. The bladder is inverted. Often this will involve the urinary tract as well as the digestive and reproductive systems. Opening defects may occur in the abdominal wall, bladder, genitals, pelvic bones, the last part of the large intestine (rectum) and the end of the rectum (anus). Children with bladder exstrophy have vesicoureteral reflux. This causes the urine to flow incorrectly. Also, children with this condition have epispadias .
  • Cloacal ecstasy: Cloacal ecstasy is the most serious form of MEEK. In this case, the rectum, bladder, and genitals are not completely separated as the fetus develops. These organs may not form correctly and the pelvic bones are also affected. The kidneys, spine, and spinal cord may also be affected. Most children with this condition have spinal abnormalities , including spina bifida . Children born with protruding abdominal organs likely have cloacal ecstasy or bladder ecstasy.

How is bladder exstrophy diagnosed?

Bladder exstrophy is found incidentally during a routine pregnancy ultrasound. It can be diagnosed more precisely before birth with an ultrasound or MRI-scan .

Signs the doctor will look for in images include:

  • Bladder that is not filled or emptied correctly
  • Umbilical cord located on the abdomen
  • Separation of the pubic bones
  • Smaller-than-normal genitalia
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Sometimes the disease is not visible until the baby is born. In a newborn baby, doctors look for:

  • The size of the open and exposed part of the bladder
  • Position of testicles
  • Intestinal swelling from the abdominal wall (inguinal hernia)
  • Belly circumference anatomy
  • Opening position at the end of the rectum
  • How far apart the pubic bones and how easily the pelvis moves

How is bladder exstrophy treated?

After birth, the bladder is covered with a clear plastic dressing for protection.

Children born with bladder exstrophy are treated with reconstructive surgery after birth. The general objectives of the restructuring are:

  • Provide adequate space to store urine
  • Creating external genitalia that look and function acceptable
  • Establishment of bladder control (continence)
  • protect kidney function

Surgical treatment

There are two main approaches to surgery for bladder exstrophy, but it is unclear whether one approach is significantly better than the other. Research is ongoing to improve surgeries and examine their long-term outcomes.

Two types of surgical repair include:

1- Full repair

This procedure is called complete primary repair of bladder exstrophy. Complete repair surgery is performed in a single procedure that closes the bladder and abdomen, and repairs the urethra and external sex organs. This can be done right after birth or when the baby is two to three months old. Most surgeries performed on newborns will involve repairing the pelvic bones. However, doctors may choose not to make this repair if the baby is less than 72 hours old and the baby’s bones are flexible.

2- Phased repair

The full name of this approach is the modern staged repair of bladder exstrophy. Progressive repair includes three actions. One is done within 72 hours of birth, the other at 6 to 12 months of age, and the last at 4 to 5 years of age. The first procedure closes the bladder and abdomen, and the second repairs the urethra and sex organs. Then, when the child is old enough to participate in toilet training, surgeons perform the bladder neck reconstruction.

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Surgical follow-up

Standard care after surgery includes:

  • Immobilization: After surgery, babies need to remain calm while they recover. The amount of time a child needs to be immobilized varies, but is typically four to six weeks.
  • Pain management: Doctors may insert a thin tube into the spinal canal during surgery to deliver pain medication directly to the area where the baby needs it. This allows for more consistent pain control and less use of opioid medications.

After surgery, some can achieve continence, although most do not. Children sometimes need to have a tube inserted ( catheterization ) in their bladder to drain the urine . Additional surgeries may be required as the child grows.

Bladder exstrophy complications

before surgery

Without treatment, children with this condition become incontinent. They also run the risk of sexual dysfunction and an increased risk of bladder cancer.

after surgery

Surgery can reduce complications. The success of the surgery depends on how severe the defect is. Many children who have surgical repair may hold urine. Young children with bladder exstrophy may walk with their legs slightly turned outward due to separation of their pelvic bones.

long-term complications

People born with bladder exstrophy may have normal sexual function, including the ability to have children. However, pregnancy will be at high risk for both mother and baby and a planned cesarean delivery may be required.

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