Bladder Exstrophy and Epispadias | Diagnosis and Treatment

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How is bladder exstrophy diagnosed?

Bladder exstrophy may be diagnosed during a fetal ultrasound or other imaging techniques such as magnetic resonance imaging (MRI) or immediately after birth during a physical examination.

Prenatal care is an important aspect of health for every expectant mother and fetus. The prenatal diagnosis of bladder exstrophy allows time for learning about the condition and preparing for the anticipated care.

Boston Children’s offers a variety of resources for families, including the Advanced Fetal Care Center (AFCC). The AFCC provides support through prenatal counseling, by partnering with you to develop a treatment plan and follow-up care for your baby. The team includes a maternal fetal-medicine specialist who monitors you and your baby and a pediatric urology doctor who will develop a treatment plan for your baby.

Bladder extrophy in boys

Bladder extrophy in girls

How is bladder exstrophy treated?

Treatment for bladder exstrophy begins at birth. The primary goal when caring for a child with bladder exstrophy is to:

  • preserve normal kidney function
  • develop adequate bladder function and promote urinary continence
  • provide acceptable appearance and function of the external genitalia
  • ensure that your child has a typical and normal childhood

There are two main approaches to the repair of bladder exstrophy:

Complete primary repair of exstrophy (CPRE)

CPRE is a procedure 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 six-to-eight weeks of life. Delaying CPRE beyond the first, 2-to-3 days of life may have several advantages for the child. 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.

Modern staged repair of exstrophy (MSRE)

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-to-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.

What is the long-term outlook for bladder exstrophy?

After initial treatment for bladder exstrophy, lifelong follow-up care is necessary. Care is ongoing and can involve a range of surgical procedures and tests. Every child is different, and our doctors and nurses will work with you to develop the best plan for your baby. In many cases, additional surgery is required during childhood, adolescence and young adulthood.

Boston Children’s is committed to providing the care that your child needs. As your child grows, their bladder and kidneys grow with them. To ensure that your child’s kidneys and bladder are functioning properly, follow-up testing, such as a renal ultrasound, may be necessary.

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