Treatment for bladder exstrophy starts at birth: the protruding bladder is covered with a transparent plastic dressing to protect it. The primary treatment for Bladder Exstrophy (BEX) is reconstructive surgery and/or multiple surgeries.
The primary goals of the reconstruction surgery are to:
- Maintain normal renal function
- Improve enough bladder function and capacity, and ultimately gain urinary continence
- Provide acceptable physical appearance of the abdominal walls and the external genitalia
- Ensure your child has a typical and normal childhood
- Support and comfort the child throughout the physical and mental traumatic time period
- Contribute to fertility
There are two different and important methods for surgery; both are of the same caliber of effectiveness. The treatment plan is dependent on the training and the preference of the surgeon. Long-term results are generally quite similar to each other.
- Modern staged repair of exstrophy (staged repair)
- Complete primary repair of exstrophy (complete repair)
Staged repair
This includes three surgeries for the reconstruction of the bladder:
Initial repair
The bladder and abdominal walls are closed, the belly button is reconstructed, and an osteotomy is sometimes performed (the pelvic bones are reformed to aid in the repair). The initial repair is generally performed in the first 2 to 3 days of life.
Second stage
In boys, the proximal part of the urethra closest to the bladder is closed along with the bladder. In girls, the urethra is usually closed along its entire length, expanding all the way from the bladder to the face of the skin between the labia, which it normally should be. The second stage repair takes place between 6 to 12 months of age.
Third stage
The third stage includes bladder neck reconstruction with bilateral ureteral reimplantation. This part is performed when the bladder has grown enough to hold the appropriate volume of urine. This procedure is generally done between the ages of 6 to 10 years.
Complete repair
In the surgical treatment of bladder exstrophy, the complete repair method has gained more popularity in the last couple of years. This is a technique in which both the bladder is closed, and the epispadias is repaired (the entire urethra is closed into a tube) at the same time. Children who undergo a Complete Repair will most likely need additional surgeries in later years to manage urinary incontinence and vesicoureteral reflux. This procedure is usually performed at approximately 6 to 8 weeks of life. Postponing this repair after the first 2 to 3 days of life may have several benefits for the child, for instance, giving some time for important bonding between the newborn and his/her parents before the initial reconstructive surgery. This bonding is also important for the long recovery time that follows the procedure.
Most surgeries for infants involve the correction of the pelvic bones. Nevertheless, surgeons may select not to do this repair if the baby is less than 72 hours old, the pelvic separation is small, and the infant’s bones are flexible.
The time between birth and initial repair supplies growth, development, and additional maturation of some organs and organ systems before the complex surgery. Waiting also more strongly secures the success of the surgery and the safety of the anesthesia.
After surgery, your child will be admitted to the hospital for approximately three weeks to allow him to heal.
After initial treatment for bladder exstrophy, lifelong follow-up care is necessary. Care is constant and can involve a range of surgical procedures and tests. Every patient is different, and doctors and nurses will work with you to develop the best plan for your baby. Regardless of the type of surgery, in many cases, additional surgery is required during childhood, adolescence, and young adulthood.