Bladder exstrophy occurs when the bladder does not develop normally and is exposed through an opening on the abdominal wall. Urine produced by the kidneys drains into this open area and is not stored normally in the bladder. The disorder may occur in varying degrees from a mild to a severe defect that involves the genitalia and pelvis.
Bladder exstrophy is a rare disorder that occurs during fetal development. It occurs in about 1 in every 30,000 births and is more common in males. The cause of exstrophy of the bladder is unknown.
Exstrophy of the bladder can usually be diagnosed by fetal ultrasound before an infant is born. This can be an overwhelming diagnosis for expectant parents. At the Children’s Hospital of Philadelphia, we encourage families to meet with our exstrophy team in the Division of Urology before the baby is even born. During the prenatal visit, our team can counsel families on the diagnosis and what to expect after birth with regard to surgical procedures, continence and adjustment for the child over time. If bladder exstrophy is not seen prenatally, the bladder defect is easily visible after birth.
Complete primary repair of exstrophy (CPRE) is the preferred surgical approach at CHOP. CPRE allows us to achieve the goals of surgical correction in one operation: bladder closure, bladder neck reconstruction, epispadias repair and pelvic osteotomy (closing the pelvic bones). Closing the bladder early allows the bladder to cycle (fill and empty urine) which optimizes bladder growth and development. Additional surgery may be needed to help improve continence as the child ages, or to complete the genital reconstruction.
Our long-term goals for children with exstrophy are to optimize daytime and nighttime continence, volitional voiding and normal renal function, and to have a cosmetically acceptable genitalia. Our dedicated team consisting of two urologists, an orthopedic surgeon, and a psychologist have had years of experience working together to ensure the best surgical and social outcomes for your child.
Reviewed by: Division of Urology
Date: March 2011