Exstrophy of the Bladder and Epispadias

What is exstrophy of the bladder?

Exstrophy of the bladder 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.

What is epispadias?

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.

Who is affected by exstrophy of the bladder?

According to the American Urologic Association, this is a rare disorder that occurs in about one in every 30,000 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:

• 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

How is exstrophy of the bladder diagnosed?

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. Your child's doctor may order other diagnostic procedures.

What is the treatment for exstrophy of the bladder?

Specific treatment for exstrophy of the bladder and epispadias will be determined by your child's doctor based on:

• 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

After a diagnosis is made, your child will be referred to a surgeon for surgical repair. There are usually three stages to the surgical repair that start when your child is as young as 48 hours old. The first stage involves internalization of the bladder and closing the abdomen. The second stage may be done as early as 6 months of age and involves repairing the epispadias and other genital abnormalities. The final surgery is done at around age 4-5, when the bladder is large enough and the child is psychologically ready to be dry. This final surgery involves reconstruction of the urinary tract including the bladder, and other structures of the urinary tract.