Bladder exstrophy

What is bladder exstrophy?

Bladder exstrophy, also called exstrophy of the bladder, is a rare congenital (present at birth) birth defect in which the bladder is not contained inside the abdomen, but is visible on the outside of the body.

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The defect exists in less than one percent of births. The condition is more common among boys than it is among girls.

What are the different types of bladder exstrophy?

Bladder exstrophy varies widely among children with this condition. 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.

What are the signs and symptoms of bladder exstrophy?

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.

How is bladder exstrophy diagnosed?

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.

What are the causes of bladder exstrophy?

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

How is bladder exstrophy treated?

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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.