Exstrophy of the Bladder
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 the child’s body. Because the bladder and other structures are outside 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 opening where urine is released from, is in an abnormal location. In males, an epispadias means the opening is usually on the topside of the penis and not the tip. This is different from hypospadias where the opening is 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 and is more common in males. 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.
What is the treatment for exstrophy of the bladder?
Specific treatment for exstrophy of the bladder and epispadias is determined based upon:
- The child’s age, overall health and medical history.
- The extent of the disorder.
- The child’s tolerance for specific medications, procedures or therapies.
- Expectations for the course of the disorder.
- The patient’s family’s opinion or preference.
After a diagnosis is made, the child will be seen by both an orthopaedist and a urologist for surgical repair, as specialties are usually involved in the primary surgery. There are typically multiple stages to the surgical repair that are required and begin when the child is as young as 48 hours old. Often the first stage involves internalization of the bladder and closing the abdomen, as well as repairing the pelvic bones. Other future repairs include repairing the epispadias and other genital abnormalities, as well as possible surgeries to reconstruct the urinary tract should the child be experiencing incontinence.
Patient and Family Resources
The Association for the Bladder Exstrophy Community (ABC), established in June 1991 (formerly known as the Association for Bladder Exstrophy Children), is an international support group network comprising individuals with bladder exstrophy, local parent-exstrophy support groups and health care providers who work with patients and families living with bladder exstrophy. We encourage you to log on to: http://bladderexstrophy.com/ for more information
Learn more about surgical procedures associated with bladder exstrophy: