Bladder Exstrophy

What Is Bladder Exstrophy?

Bladder Exstrophy

What Is Bladder Exstrophy?

 

Introduction

Sometimes problems happen with the way the bladder forms. The bladder muscles don’t develop properly for children with bladder exstrophy. Under normal conditions, the bladder is located in your lower belly. Bladder exstrophy is a rare birth defect in which the bladder develops outside the fetus. The exposed bladder can’t store urine or function normally, resulting in urine leakage (incontinence). Problems caused by bladder exstrophy vary in severity. They can include defects in the bladder, genitals, and pelvic bones, as well as defects in the intestines and reproductive organs. Bladder exstrophy may be spotted on a routine ultrasound during pregnancy. Sometimes, though, the defect isn’t visible until the baby is born. Babies born with bladder exstrophy will need surgery to correct the defects.

Bladder exstrophy is a birth defect. It’s a condition where the bladder and parts around it from inside out. The skin, muscle, and pelvic (hip) bones at the lower part of the belly or abdomen are not joined. As a result, the inside of the bladder pokes outside the belly. Instead of its normal round shape, the bladder is flat. During a baby’s development in the womb, the abdominal wall and underlying organs sometimes don’t fuse properly, and the infant is born with the bladder inside out and exposed on the outside of the body. Bladder exstrophy is rare. On average, it occurs in about 1 out of every 50,000 live births. It is slightly more common in males than females.

 

What Causes Bladder Exstrophy?

What Causes Bladder Exstrophy

Doctors aren’t sure what causes bladder exstrophy to develop. Researchers think that a combination of genetic and environmental factors likely plays a role. Bladder exstrophy is not caused by anything a parent did or didn’t do during pregnancy. There is no clear cause for this condition. It is thought to happen during the 11th week of pregnancy, as the organs develop. Some experts believe that the bladder defect occurs at the times the tissues in the lower wall of the belly or abdomen develop. At the same time, the developing muscles and pelvic bones are affected too. A temporary tissue called the cloacal membrane covers the lower belly wall and is replaced by maturing and developing abdominal muscles. If the cloacal membrane bursts before the abdominal muscles fully form, this may result in an “exstrophied” bladder. Another tissue called the urorectal septum helps to separate the developing bladder from the bowels or intestines.

Whether the child is born with epispadias (a rare birth defect of the penis, where the urethra ends in an opening on the top side of the penis. For girls, the urethra develops too far upfront), classic bladder exstrophy or cloacal exstrophy (a severe birth defect where the abdominal organs of the bladder and intestines are exposed. The penis or vagina is split, and the anus may be sealed) depends on when the cloacal membrane bursts and if the bladder and intestines are separated by the urorectal septum.

Factors that increase the risk of bladder exstrophy include:

  • Family history: Firstborn children, children of a parent with bladder exstrophy, or siblings of a child with bladder exstrophy have an increased chance of being born with the condition.
  • Race: Bladder exstrophy is more common in whites than in other races.
  • Sex: More boys than girls are born with bladder exstrophy.
  • Use of assisted reproduction: Children born through assisted reproductive technology, such as IVF, have a higher risk of bladder exstrophy.

 

Is Bladder Exstrophy Genetic?

Is Bladder Exstrophy Genetic

Family history plays a role. Children born to a parent with bladder exstrophy have about a 1 in 70 chance of having it. If a family has one child with this condition, they have a 1 in 100 chance of having another child with it. Fertility treatments may play a role. Recent studies suggest that children born with assisted fertility are seven times more likely to have bladder exstrophy. The underlying cause of this error in development is not known. Unfortunately, theories on genetic and family history risk factors are mostly speculative or have limited research and evidence to back them up.

 

Bladder Exstrophy Symptoms

Doctors often identify BE during a routine pregnancy ultrasound. In other cases, the defect is not seen until after a baby is born. The main symptom of BE is leaking urine from the open bladder. A child with BE will have bladder control problems and will struggle with controlling the muscles of the abdomen and digestive tract. Other symptoms will vary from child to child, but may include:

  • The urethra not forming completely:A rare congenital condition called epispadias can affect the genitals. In boys with this condition, the urethra may be open on top of the penis rather than the tip. In girls, the opening is positioned higher than it normally would be.
  • Wider-than-normal pubic bones:The pubic bones usually join to protect the bladder, urethra, and abdominal muscles. In children with BE, these bones are not joined, leaving the hips outward.
  • Abnormal genitalia development:Boys with BE may have a penis that is shorter and curved, while testicles are not where they usually are. Some boys with this condition may have hernias. Hernias are bulges seen in the groin that may cause pain and discomfort, especially with coughing and lifting. In girls, the clitoris and labia minora may be separated and the vagina and urethra may be shorter. The uterus, fallopian tubes, and ovaries are generally unaffected.

umbilical hernia

  • Belly button displacement or umbilical hernia:Umbilical hernias usually don’t cause pain, but they appear as a bulge near the belly button. They are generally present at birth and can be seen when the child is straining for a bowel movement or coughing.
  • Vesicoureteral reflux, or VUR:VUR causes urine to travel back up the kidneys. This condition may develop after surgery to repair and close the bladder.
  • Bladder neck and sphincter that aren’t complete: The bladder neck is the lower part of the bladder. The sphincter is a ring of muscles around the bladder neck. These parts control urine flow when they open and close. If they aren’t complete, your child’s bladder cannot hold in urine.
  • Small bladder: The bladder may hold less urine than normal. This may cause frequent peeing (urination) or leaking.
  • Backflow of urine: Normally, the flow of urine is one way, from the kidneys down tubes called ureters into the bladder. In vesicoureteral reflux, urine can also flow back from the bladder to the kidneys.
  • Spinal lipoma: Some children with a more severe form of exstrophy (cloacal exstrophy) may have a fatty growth (lipoma) on their spinal cord. This may cause problems with their legs, bladder, and rectum. Cloacal exstrophy also affects the genitals and bowel.
  • Kidney problems: Some children may have double kidneys or kidneys, not in the usual place.
  • Bowel problems: Children with cloacal exstrophy may have bowels that are large, not well-developed, and without a rectum. They usually need surgery to make an opening from the intestines to outside the belly wall so poop (stool) can come out. This surgery is called a colostomy.

 

Bladder Exstrophy and Fertility

Bladder Exstrophy and Fertility

Most of the male and female patients with EEC have adequate sexual function. Nearly three fourth of the male patients are capable of ejaculating with varying force. Urinary diversion in some series seems to result in better ejaculatory hence fertility outcome. Recent series have shown equally good results with complete reconstruction. Male exstrophy patients are reported to have more psychosexual disturbances as compared to females. Most of the female patients have normal fertility but contrary to this most of their counterpart male patients have significantly suboptimal fertility.

Although the penile length is short, most male patients are reported to have normal libido and erections adequate for sexual intercourse. A significant number of patients do not ejaculate normally and suffer from varying degrees of seminal fluid abnormality. Most of the females with EEC have normal sexual desire and are adequately sexually active. The associated problems which may adversely affect sexual activity are leakage of urine during sexual intercourse, reduction or lack of clitoral sensation, and genital prolapse. In contrast to the males, most of the females have normal fertility.

 

Bladder Exstrophy Treatment

After delivery, the bladder is covered with a clear plastic dressing to protect it. Children born with bladder exstrophy are treated with reconstructive surgery after birth. The overall goals of reconstruction are to:

  • Provide enough space for urine storage
  • Create outer sex organs (external genitalia) that look and function acceptably
  • Establish bladder control (continence)
  • Preserve kidney function

There are two main approaches to surgery, though it’s not clear whether one approach is significantly better than the other. Research is ongoing to refine the surgeries and study their long-term outcomes. The two types of surgical repair include:

  • Complete 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 the abdomen and repairs the urethra and outer sex organs. This can be done soon after birth, or when the baby is around two to three months old.

Most surgery for newborns will include repair to the pelvic bones. However, doctors may choose not to do this repair if the baby is less than 72 hours old, the pelvic separation is small and the infant’s bones are flexible.

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

 

Surgical follow-up

Standard care after surgery includes:

  • Immobilization:After surgery, infants need to stay in traction while they heal. The amount of time a child needs to be immobilized varies, but is typically around four to six weeks.
  • Pain management:Doctors can place a thin tube into the spinal canal during surgery to deliver pain medications directly to the area it’s needed. This allows for more consistent pain control and less use of opioid medications.

 

What Drinks Are Good for Your Bladder?

Drink plenty of liquids, especially water

  • Drink enough fluids, especially water, one should try to drink six to eight, 8-ounce glasses of fluid each day. Water is the best fluid for bladder health. At least half of fluid intake should be water. Some people need to drink less water because of certain conditions, such as kidney failure or heart disease.
  • Limit alcohol and caffeine. Cutting down on alcohol and caffeinated foods and drinks—such as coffee, tea, chocolate, and most sodas—may help.
  • Fruit juice: Try switching to something with less acid, such as apple or pear juice, and dilute it with water.
  • Drinks with artificial sweeteners, such as aspartame and saccharin, may irritate the bladder.
  • Chamomile and peppermint teas can also help improve bladder infections.

Hopefully, this article gave you some information regarding Bladder Exstrophy.

 

 

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