 Exstrophy of the Bladder and Epispadias
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Exstrophy of the bladder, sometimes known as classic exstrophy, is a complex combination of disorders that occurs during fetal development. The disorder usually involves many systems in the body, including the urinary tract, skeletal muscles and bones, and the digestive system. Bladder exstrophy means that the bladder is essentially inside out and exposed on the outside of the abdomen. Because the bladder and other structures are exposed to the outside of the body, urine constantly trickles onto the skin causing local irritation.
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Epispadias is usually seen with exstrophy of the bladder. Epispadias occurs when the urethral opening, which is the hollow tube that drains urine from the bladder to the outside of the body, is in an abnormal location. In males, the opening is usually on the topside of the penis and not the tip. This is different than hypospadias where the opening is usually underneath the penis. In girls, the urethral opening may be positioned further up the urethra and may be bigger and longer than normal. Often in girls, the opening extends to the bladder.
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This is a rare disorder that occurs in about one in every 40,000 births. It is more common in males and varies in severity. The cause of exstrophy of the bladder is unknown. Although some reports show a clustering of exstrophy of the bladder in families, suggesting an inherited factor. However, the chance for parents to have another child with exstrophy of the bladder is small (1 percent or less). The disorder may occur in varying degrees from mild to severe. In many cases, exstrophy of the bladder is associated with the following:
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- widened pubic bones
- outwardly rotated legs and feet
- triangle-shaped defect in the abdomen and visibility of the membrane of the bladder which is usually bright pink
- abnormally-shaped abdominal muscles
- displacement of the umbilicus (belly button), usually above the defect
- umbilical hernia may be present (section of intestine protrudes through a weakness in the abdominal muscles)
- short, small penis with urethral opening along top of penis (epispadias)
- narrow vaginal opening, wide labia, and short urethra
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Exstrophy of the bladder can usually be diagnosed by fetal ultrasound before an infant is born. After the infant is born, exstrophy can be determined by physical examination. Other diagnostic procedures may include:
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- renal ultrasound - a non-invasive test in which a transducer is passed over the kidney producing sound waves which bounce off of the kidney, transmitting a picture of the organ on a video screen. The test is used to determine the size and shape of the kidney, and to detect a mass, kidney stone, cyst, or other obstruction or abnormalities.
- renal scan - a specialized scan that may include injections of a radioactive substance. A scan is then performed at different intervals to determine the blood flow through the renal vessels and urine flow through the kidneys.
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Specific treatment for exstrophy of the bladder and epispadias will be determined by your child's physician based on:
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- your child's age, overall health, and medical history
- the extent of the disorder
- your child's tolerance for specific medications, procedures, or therapies
- expectations for the course of the disorder
- your opinion or preference
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After a diagnosis is made, your child will be referred to a pediatric urologist for surgical repair. Formerly, exstrophy of the bladder was treated in a planned three-stage operation, the final stage of which couldn't be performed until the child was 3 years of age. Now, with advances in surgical techniques, surgery in most children can be performed in one stage shortly after birth. The operation consists of internalization of the bladder, closing of the abdomen, repair of the penis in boys or the external genitalia in girls and any structures that need repair in the urinary tract. This all-in-one approach has resulted in improved continence and, in some patients, it has not been necessary to correct vesicoureteral reflux or incontinence, which are associated with bladder exstrophy. The results of total reconstruction in the newborn period have been excellent, but patients should expect the possibility of further surgery as the child gets older.
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Researchers at Children's Hospital Boston's Center for Genitourinary Tissue Reconstruction are looking into ways to apply tissue engineering techniques to treat children with bladder exstrophy and/or epispadias. Typically, surgeons use the patient's intestinal or stomach tissue to shape and enlarge the bladder in children with exstrophy. The incompatibility of intestinal tissue and bladder tissue can sometimes lead to problems that can include metabolic disorders, kidney stones and cancer risks. Engineered tissue, however, is viable tissue that is grown in the laboratory using the patients' own cells. Doctors would take a piece of the child's bladder, less than the size of a postage stamp, retrieve the cells and seed them onto scaffolds designed to accommodate the various layers of the bladder. Using this technique, doctors can grow enough of the patients own bladder tissue to enlarge or replace the bladder.
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