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Bladder exstrophy is a rare, complex birth defect involving the urinary, reproductive and intestinal tracts, as well as the musculoskeletal system. During a baby’s development in the womb, the abdominal wall and underlying organs sometimes do not fuse properly, and the infant is born with the bladder inside out and exposed on the outside of the body.
Bladder exstrophy usually involves several systems within the body, including the urinary tract, reproductive tract (external genitalia) and pelvic skeletal muscles and bones. In rare cases, intestinal tracts are involved.
Bladder exstrophy can result in weakened abdominal muscles and a shorter than average urethra and vagina or penis. It can cause a variety of complications and problems, including incontinence, catheterization, reflux, infertility and the need for repeated reconstructive surgery. It may be associated with urinary incontinence and back up of urine from the bladder to the kidneys, called vesicoureteral reflux.
The cause of bladder exstrophy is unknown. Some studies show a clustering of the condition in families, suggesting that there’s an inherited factor. Popular theories suggest overdevelopment of a normal structure known as the cloacal membrane. Overdevelopment or prolonged presence of the cloacal membrane may prevent appropriate tissue development, ingrowth and the joining together of the supportive lower abdominal wall structure. However, the chance for parents to have another child with exstrophy of the bladder is less than one percent.
Children with bladder exstrophy also have epispadias. Epispadias occurs when the urethra fails to close normally, and the inner lining of the urethra lays flat and exposed on the surface of the penis. In some cases, epispadias may be present on its own.
In isolated epispadias, the bladder is closed and covered by the lower abdominal wall muscles and skin. In some children with epispadias, there may be bony- pelvis abnormalities similar to, but not as severe as, that seen with bladder exstrophy.
The Boston Children's Hospital Bladder Exstrophy Program is dedicated to providing exceptional pediatric care.
We provide a team of health care professionals that include physicians, nurses, social workers and child life specialists who will care and support you and your child at every step.
Our nurses are available to answer any questions whether you have a child living with bladder exstrophy or you have a baby recently diagnosed.
We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”