Bladder exstrophy repair is surgery to repair a birth defect in which the bladder is inside out and sticks out of the abdominal wall. The pelvic bones are also separated.

Bladder exstrophy repair involves two surgeries: One to repair the bladder and another to attach the pelvic bones to each other.

The first surgery separates the exposed bladder from the abdomen wall and closes the bladder. The bladder neck and urethra are repaired. A flexible, hollow tube called a catheter is placed to drain urine from the bladder through the abdominal wall. A second catheter is left in the urethra to promote healing.

The second surgery, pelvic bone surgery, may be done along with the bladder repair. Or, it may be delayed for weeks or months.

The surgery is recommended for children who are born with bladder exstrophy. Bladder exstrophy occurs more often in boys and is often associated with other birth defects.

Surgery is necessary to:

• Allow the child to develop normal urinary control
• Avoid future problems with sexual function
• Improve the child's physical appearance (genitals will look more normal)
• Prevent infection that could harm the kidneys

Sometimes the bladder is too small at birth, so the surgery will be delayed till the bladder has grown. In this case, the newborn is sent home on antibiotics and the bladder, which is outside the abdomen, must be kept moist.

It can take months for the bladder to grow to the right size. The infant will be followed closely by a medical team to determine when the surgery should take place.

Risks with this procedure may include:

• Chronic urinary tract infections
• Erectile/sexual dysfunction
• Inadequate urinary control (incontinence)
• Kidney problems
• Need for future surgeries

Risks for any anesthesia are:

• Breathing problems
• Reactions to medications

Risks for any surgery are:

• Bleeding
• Infection

Most bladder exstrophy repairs are done when your child is only a few days old, before they leave the hospital. In this case, the hospital staff will prepare your child for the surgery.

If the surgery was not done when your child was a newborn, your child’s doctor may ask for the following when it is time to do the surgery:

• Urine test (urine culture and urine analysis) to check your child’s urine for infection and kidney function
• Blood tests (complete blood count, electrolytes, and kidney tests)
• Record of urine output
• X-ray of your child’s lower stomach and bones
• Renal ultrasound to check your child’s kidneys

Always tell your child’s doctor or nurse:

• What drugs your child is taking
• Even drugs or herbs you bought without a prescription

During the days before the surgery:

• 10 days before the surgery, your child may be asked to stop taking aspirin, ibuprofen, warfarin (Coumadin), and any other drugs that make it hard for your blood to clot.
• Ask your doctor which drugs your child should still take on the day of the surgery.

On the day of the surgery:

• Your child will usually be asked not to drink or eat anything for several hours before the surgery.
• Give the drugs your child’s doctor told you to give with a small sip of water.
• Your child’s doctor or nurse will tell you when to arrive.

After pelvic bone surgery, the child will need to be in a lower body cast or sling for 4 to 6 weeks. This helps the bones heal.

After the bladder surgery, your child will have a tube that drains the bladder through the stomach wall (suprapubic catheter) for 3 to 4 weeks.

The child will also need pain management, wound care, and antibiotics. The doctors and nurses will teach you about these things before you leave the hospital.

Due to the high risk of infection, your child will need to have a urine analysis and urine culture checked at every well-child visit, and at the first signs of an illness. Some children take antibiotics on a regular basis to prevent infection.

Urinary control usually happens after the neck of the bladder is repaired. This surgery is not always successful, and the child may need to repeat the surgery later on.

A few children, even with repeat surgery, will not have control of their urine and must use intermittent catheterization to have urinary control.

Gearhart JP, Mathews R. Exstrophy-Epispadias Complex. In: Wein AJ, ed. Wein: Campbell-Walsh Urology. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 119.

Hammer G, Hall S, Davis PJ. Anesthesia for general abdominal, thoracic, urologic, and bariatric surgery. In: Motoyama E, Davis P, eds. Smith’s Anesthesia for Infants and Children. 7th ed. Philadelphia, Pa: Mosby Saunders Elsevier; 2005:chap 19.