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Pediatric Bladder Exstrophy

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Summary

Bladder exstrophy, also called exstrophy of the bladder, is a rare birth defect in which the bladder is not contained inside the abdomen, but is visible on the outside of the body. The defect exists in less than one percent of births.

Cause

Bladder exstrophy occurs early in a fetus’s development, but why it occurs is not known.

Types

Bladder exstrophy varies widely among children with this condition. The condition is more common among boys than it is among girls. Children with bladder exstrophy often have other abnormalities, such as an unusually wide pelvic bone structure or problems with abdominal muscles.

  • Girls with bladder exstrophy may have a urinary opening in the wrong place, or one that’s very large or misshapen. Girls with bladder exstrophy also may have a split clitoris.
  • Boys with bladder exstrophy may have a misshapen penis, or it may be curved downward. Boys with bladder exstrophy also may have an unusually short urethra, or one that’s on the upper part of the penis, instead of on the tip.

Symptoms

Children with bladder exstrophy will have very poor bladder control and may also have problems controlling other muscles in the abdominal area, including in the digestive tract.

Tests and Diagnosis

Bladder exstrophy is an unusual condition, easily seen during a visual exam and often diagnosed before birth, during an ultrasound exam of the mother. Most of the tests performed on patients with the condition are intended to rule out other problems or complications associated with bladder exstrophy.

A baby’s bladder develops in the first trimester of pregnancy, and bladder exstrophy is usually diagnosed during an ultrasound before the child is born. If the condition is not diagnosed before delivery, it is obvious at birth.

In either case, treatments typically begin very shortly after delivery. Bladder exstrophy is diagnosed when any one of several abnormal conditions exist in a child’s bladder, urinary tract, or abdominal area.

A variety of imaging tests are used to determine the extent of the condition. Your doctor may also conduct urine tests and bladder function tests to check for infections and to determine whether the kidneys are functioning properly.

Children with bladder exstrophy could also have widened pelvic bones or other abnormalities in the abdominal region, such as an off-center belly button. Unusual bladder formation associated with bladder exstrophy sometimes includes a misshapen bladder, a short bladder neck, a short urethra in boys or a wide labia or very narrow vaginal opening in girls.

Such related conditions are usually addressed during the surgeries required to reverse bladder exstrophy. Following treatment, most children with this condition are able to have normal elimination and sexual activity.

Treatment

In almost all cases, surgery is required to treat bladder exstrophy. Many patients require several surgeries, performed over several years as they grow and develop. Under the care of an experienced urology team, most children with bladder exstrophy will have bladders and sexual organs that are fully functional and normal in appearance.

Generally, the first surgery to correct bladder exstrophy occurs when the child is just a few days old. The goal of bladder exstrophy reconstructive surgeries is to position the bladder inside the body so that it functions properly, first, and then to improve outward appearances.

Surgeries are performed by pediatric urologists, and patients are given general anesthesia. They may be given pain or anti-inflammatory medications to help in healing after a surgery. Patients may also be given antibiotics to prevent infections while healing.

Bladder reconstruction and the management and treatment of bladder exstrophy will be different for each patient. In most cases, some counseling is offered to the patient and the patient’s family, to help with the adjustment to each phase of the process.

In the initial surgery or surgeries, the bladder will be detached from the abdominal wall and if necessary, it will be separated from the bowel. If the bladder’s size or shape interferes with function, it may be necessary to reconstruct part of the bladder.

Following each surgery, the child will likely remain hospitalized for several weeks. Urine tests will be repeated to monitor the surgery site, bladder and kidneys for any signs of infection.

The final surgical procedures are usually performed when the child is old enough to maintain bladder control, generally around four years of age

Prognosis

Reconstruction done in stages, while a lengthy process, offers a hopeful prognosis for children with bladder exstrophy. About 75% of those treated for this condition will be able to exercise bladder control. The majority of those treated also have normal function of the bladder and sexual organs.

After treatment, patients treated for bladder exstrophy generally have no lifestyle restrictions and the condition has no effect on life expectancy.

Prevention

The cause of bladder exstrophy is unknown at this time, so there are no guidelines for prevention.

FAQs

Why is my child affected?

Bladder exstrophy is a rare birth defect. Although some studies have suggested the condition has a genetic component, at this time the underlying cause of bladder exstrophy is unknown. Fortunately, it is treatable, and does not affect life expectancy.

What may be affected in addition to the bladder?

While the primary concern for a child with bladder exstrophy is to establish normal bladder function (and later, normal sexual function), in many cases other areas of the abdomen are affected.

The most common problems associated with bladder exstrophy are widened pelvic bones (which may complicate reconstruction), interference between the bladder and the bowel, abnormal appearance of the penis or vagina, and problems with the muscular structure in the midsection, including the digestive tract.

When will my doctor suggest treatment begin?

In most cases, bladder reconstruction surgery can be performed within the child’s first few days of life.

How does a doctor diagnose bladder exstrophy?

Bladder exstrophy can be diagnosed visually, and is often seen on an ultrasound exam before the child is born. If the condition is not diagnosed before delivery, it is apparent at birth. After the child is born, a doctor will typically order additional imaging tests and urine tests to determine the extent of the condition and whether other bodily systems are affected.

What is the treatment for bladder exstrophy?

Bladder reconstruction requires surgery, which in most cases allows the patient to achieve normal bladder function and proper development of the sexual organs. Typically, more than one surgery is required.

Will bladder exstrophy be a lifelong disability?

The long-term outlook for children born with bladder exstrophy is very good. In the majority of cases, when pediatric urologists and surgeons familiar with the condition and related complications follow a bladder reconstruction and treatment plan, affected children establish normal bladder function and have no lifestyle restrictions. Also, this rare birth defect seems to have no affect on life expectancy. 

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