Bladder Exstrophy

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What Is Bladder Exstrophy?

Bladder exstrophy is a rare congenital (present at birth) defect that affects about one in 50,000 children. It can affect both boys and girls but is slightly more common in boys. This condition can affect multiple structures within the genitourinary (urinary and reproductive) system. This includes:

Bladder: The child is born with their bladder outside of the body, protruding through the abdominal wall below the belly button. The bladder is open instead of closed, so there is no place for urine to collect.

External genitalia: Boys may have a short, small penis, and girls may have a narrow vaginal opening and wide labia.

Intestines: When the baby’s intestines are exposed, it is called cloacal exstrophy. This is the most severe form of bladder exstrophy. It can cause short intestines and an anus that is not completely open.

Urethra: Bladder exstrophy can also cause a condition called epispadias, in which the urethra (the tube that carries urine out of the body) is not fully developed or is in the wrong location, typically on the side or top of the penis instead of the tip in boys.

Additionally, bladder exstrophy can cause other structural issues including:

• Abnormally shaped abdominal muscles.
• Umbilical hernia (intestines that bulge through the abdominal muscles).
• Widened pubic bones.

Bladder exstrophy can cause a series of problems affecting function and quality of life. Children with bladder exstrophy have no control over their bladder and will remain incontinent unless surgery is performed to close the bladder and correct malformations of the genitourinary system. Children with bladder exstrophy also require specialty care throughout their lives due to the high risk of associated issues. These include issues related to fertility, pregnancy, sexual function and kidney and bladder function.

Surgical Repair

Surgical treatment of bladder exstrophy varies in technique and the age at which it is performed. The consistent goals of surgery are to close the bladder, reconstruct the genitalia and establish continence.

Traditionally in the U.S., surgery has been performed using either a staged approach or all procedures performed at one time. These traditional approaches can involve multiple reconstructive surgeries, including an osteotomy to reshape the pelvic bone. Recovery time from surgery can take several weeks.

The Kelly Technique

UH Rainbow offers the Kelly technique for bladder exstrophy, a soft-tissue mobilization technique not previously available in the U.S. With this approach, the baby’s bladder is closed within the first few days of life.

The next stage is performed when the child is 12 to 18 months old. This operation combines reconstruction of the bladder neck, which connects the bladder to the urethra, and an epispadias repair, which reconstructs the penis in male children. The Kelly procedure aims to create a sphincter at the bladder neck to control the flow of urine out of the bladder, increases bladder capacity, and improves the function, size and appearance of external genitalia. After the procedure, bladder control and function will develop over the course of a few years.

Every bladder exstrophy patient at UH Rainbow has the opportunity to undergo the Kelly procedure to give them the best chance of achieving full urinary control as they get older. This approach offers improved continence rates compared to staged repairs. Since the technique focuses on soft tissue reconstruction instead of osteotomy to reshape the pelvis, it also means shorter hospital stays and faster recovery times.

Follow-Up Care

Children will require continuous follow-up care with their medical team after bladder exstrophy repair. This helps monitor their progress, identify any issues, and check for any complications such as urinary tract infections (UTIs) or bladder stones.

Follow-up care includes:

• Regular check-ups with pediatric urologists and other specialists.
• Urodynamic studies to evaluate the function of the bladder, urethra and sphincters.
• Imaging tests to monitor bladder function and kidney health.

Children with bladder exstrophy will require continued support and access to specialists throughout their childhoods and into adulthood. The program at UH Rainbow brings together pediatric and adult specialists to provide care for future issues that may arise due to bladder exstrophy, including fertility and sexual health.