Complete Primary Repair of Bladder Exstrophy (CRPE)

Complete primary repair of bladder exstrophy (CPRE) is a surgical option used to treat bladder exstrophy, a rare condition present at birth in which the bladder develops outside the body instead of inside. The external genitalia are also not fully formed and appear on the front (anterior) surface of the body. Surgical reconstruction is required to close the bladder, position it inside the body, and bring the external genitalia together in the correct position.

In some centers, bladder exstrophy repair is performed in stages: the bladder is closed during the newborn period, and the bladder neck, urethral, and genital repair is done later.

At Boston Children’s, however, CPRE is performed as a single-stage procedure, in which the bladder closure, pelvic bone approximation, and genital reconstruction are all completed in one operation.

During the CPRE procedure, a team of surgeons works carefully to repair the parts of the body affected by bladder exstrophy.

To complete the repair, surgeons will:

• Move the bladder inside the body. In bladder exstrophy, the bladder is open and exposed to the outside world. Surgeons place it back inside the body to protect it and help it hold urine.
• Bring the pelvic bones closer together. Children with bladder exstrophy often have pelvic bones that are too far apart. Surgeons cut and move these bones together to support the bladder and lower belly.
• Repair the affected muscles and soft tissues. These tissues help hold everything in place and protect the bladder. Fixing them also helps the body function more normally.
• Reshape the genitals. Surgeons work to improve how the genitals look and function, helping with urination and overall development.

CPRE is a complex surgery that can take several hours, and your child will be admitted to the hospital afterward to heal and recover. The care team will give you detailed instructions and support during this time.

After CPRE surgery, your child will remain in the hospital for seven to 10 days. During this time, they will receive IV fluids, pain management (often through an epidural catheter), and have several small medical devices in place to support healing. These include:

• A catheter, to drain urine from the bladder
• Small stents that keep the ureters and urethra open during recovery
• A spica cast to limit movement of the abdomen and lower extremities

Your child may experience uncomfortable bladder spasms after CPRE. The care team will provide medications to help manage discomfort. As your child recovers, gentle movement will be encouraged.

Once your child is discharged, they’ll continue healing at home and may need help with postoperative care. Most patients go home with the spica cast still in place. Your child’s care team will maintain close communication, guiding you through recovery and helping your child safely return to their regular activities.

CPRE is performed by experts in our Bladder Exstrophy Program, which brings together specialists with deep experience in this complex, single-stage repair. Our team participates in a multi-institutional collaboration (MIBEC) that has helped refine the technique to improve both bladder function and appearance. CPRE is our preferred surgical approach for girls with bladder exstrophy.

The surgery is performed by a highly specialized team that includes pediatric urologists, orthopedic surgeons, and anesthesiologists. These experts work together before, during, and after the operation the procedure to ensure the best possible outcome.

We use a family-centered, individualized care approach to make sure your child is ready for surgery — and that you stay informed and feel supported throughout the entire process.