Definition

Bladder exstrophy repair is surgery to repair a birth defect of the bladder. The bladder is inside out. It is fused with the abdominal wall and is exposed. The pelvic bones are also separated.

Alternative Names

Bladder birth defect repair; Everted bladder repair; Exposed bladder repair; Repair of bladder exstrophy

Description

Bladder exstrophy repair involves two surgeries. The first surgery is to repair the bladder. The second one is to attach the pelvic bones to each other.

The first surgery separates the exposed bladder from the abdomen wall. The bladder is then closed. The bladder neck and urethra are repaired. A flexible, hollow tube called a catheter is placed to drain urine from the bladder. This is placed 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. It may also be delayed for weeks or months.

A third surgery may be needed if there is a bowel defect or any problems with the first two repairs.

Why the Procedure Is Performed

The surgery is recommended for children who are born with bladder exstrophy. This defect occurs more often in boys and is often linked to 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. In this case, the surgery will be delayed until the bladder has grown. These newborns are sent home on antibiotics. 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. The team decides when the surgery should take place.

Risks

Risks of anesthesia and surgery in general are:

• Reactions to medicines
• Breathing problems
• Bleeding, blood clots
• Infection

Risks with this procedure may include:

• Chronic urinary tract infections
• Sexual/erectile dysfunction
• Kidney problems
• Need for future surgeries
• Poor urinary control (incontinence)

Before the Procedure

Most bladder exstrophy repairs are done when your child is only a few days old, before leaving 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 may need the following tests at the time of surgery:

• Urine test (urine culture and urinalysis) to check your child's urine for infection and to test kidney function
• Blood tests (complete blood count, electrolytes, and kidney tests)
• Record of urine output
• X-ray of pelvis
• Ultrasound of the kidneys

Always tell your child's health care provider what medicines your child is taking. Also let them know about the medicines or herbs you bought without a prescription that your child takes.

Ten days before the surgery, your child may be asked to stop taking aspirin, ibuprofen, warfarin (Coumadin), and any other medicines. These medicines make it hard for the blood to clot. Ask the provider 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 provider told you to give with a small sip of water.
• Your child's provider will tell you when to arrive.

After the Procedure

After pelvic bone surgery, your 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 abdominal wall (suprapubic catheter). This will be in place for 3 to 4 weeks.

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

Due to the high risk for infection, your child will need to have a urinalysis and urine culture at every well-child visit. At the first signs of an illness, these tests may be repeated. Some children take antibiotics on a regular basis to prevent infection.

Outlook (Prognosis)

Urinary control most often happens after the neck of the bladder is repaired. This surgery is not always successful. The child may need to repeat the surgery later on.

Even with repeat surgery, a few children will not have control of their urine. They may need catheterization.

References

Elder JS. Anomalies of the bladder. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 556.

Gearhart JP, Di Carlo HN. Exstrophy-epispadias complex. In: Partin AW, Dmochowski RR, Kavoussi LR, Peters CA, eds. Campbell-Walsh-Wein Urology. 12th ed. Philadelphia, PA: Elsevier; 2021:chap 31.

Weiss DA, Canning DA, Borer JG, Kryger JV, Roth E, Mitchell ME. Bladder and cloacal exstrophy. In: Holcomb GW, Murphy JP, St. Peter SD eds. Holcomb and Ashcraft’s Pediatric Surgery. 7th ed. Philadelphia, PA: Elsevier; 2020:chap 58.