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Treatment for exstrophy of the bladder begins at birth, and care encompasses a range of surgical procedures and tests. Major goals of treatment are to:
There are two main approaches to the repair of bladder exstrophy:
Complete primary repair of exstrophy (CPRE), in which both the bladder is closed and the epispadias is repaired (entire urethra is closed into a tube) at the same time. Children who undergo CPRE will likely require additional surgery during later years to manage urinary incontinence and vesicoureteral reflux (VUR). This procedure is usually performed at approximately 6 to 8 weeks of life. Delaying CPRE beyond the first 2 to 3 days of life may have several advantages for the child and the family unit, including:
The delay presents an opportunity for normal bonding between the baby and parents before the initial reconstructive surgery and lengthy recovery period that follows.
The time between birth and initial repair allows for growth, development and additional maturation of some organs and systems prior to the complex surgery and makes the anesthesia and surgery safer.
CRPE, also known as the “primary closure technique,” is the preferred approach at Boston Children’s.
Modern staged repair of exstrophy (MSRE) involves three surgeries for reconstruction of the bladder:
The initial repair: The bladder and abdominal wall 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 usually performed in the first 2 or 3 days of life.
The second stage: In boys, the proximal part of the urethra close to the bladder is closed along with the bladder. In girls, the urethra is usually closed along its entire length, extending all the way from the bladder to the surface of the skin between the labia as it normally should be. The second stage repair occurs around 6 to 12 months of age.
The third stage. This stage involves bladder neck reconstruction with bilateral ureteral reimplantation. This stage is performed when the bladder has grown sufficiently to hold an appropriate volume of urine. This procedure is usually performed between the ages of 6 to 10 years of age.
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