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Centro del Grupo de atención y apoyo a la extrofia de vejiga

Acerca de la extrofia vesical

Acerca de la extrofia vesical

Bladder exstrophy
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What causes bladder exstrophy?

While the exact cause isn't known, the most popular theory suggests overdevelopment of the cloacal membrane, a temporary membrane that gives way during normal fetal development. Overdevelopment of the cloacal membrane prevents appropriate tissue development and growth of the lower abdominal wall structures, which results in "herniation" of the open bladder and urethra on the front lower abdomen.

Bladder exstrophy can sometimes also result in:

a widened pubic bone
abnormally-shaped and weakened abdominal muscles
a shorter than average urethra and vagina or penis

¿Cómo se diagnostica?

Bladder exstrophy is diagnosed either immediately at birth or during a fetal ultrasound. A prenatal diagnosis is best because it provides time for parents to learn more about it and prepare for their baby. They are able to schedule induction of labor, schedule pre-operative evaluation and surgery, and receive continuous care with familiar and experienced clinicians.

¿Cuál es el tratamiento?

The complex surgical repair of bladder exstrophy is performed with either the staged approach or the recently re-popularized complete primary repair technique (CPRE).

Staged Approach

The staged approach consists of three surgical components:

The bladder, posterior urethra and abdominal wall are closed during the newborn period.
The urethral closure (epispadias repair) is done at 6 months to 1 year of age.
The bladder neck reconstruction for urinary continence and bilateral ureteral re-implantation (for vesicoureteral reflux -- the abnormal flow of urine from the bladder back into the ureters), are performed at 4 to 5 years of age, when the child has adequate bladder capacity, and is willing and able to participate in a voiding program after the surgery.

Although there are numerous successful outcomes with a staged approach, complete primary repair of exstrophy (CPRE) is the preferred treatment at Children's Hospital Boston.

CPRE

This technique combines the goals of staged reconstruction into a single operation, so complete surgical repair is done sooner and normal bladder function can begin earlier. This allows for optimal bladder growth and development.

After surgical repair, the Urology team at Children's and the parents and child all share the responsibility of lifelong follow-up care, paying careful attention to the child's development, growth, and overall health, particularly bladder and kidney function.

Vesicoureteral reflux, upper urinary tract deterioration, and urinary tract infections are some complications that can arise and may require further medical care.

After surgery

Vesicoureteral reflux, upper urinary tract deterioration, and urinary tract infections are some complications that can arise and may require further medical care.

What is the typical outcome?

The most important goals in the treatment of bladder exstrophy are:

to preserve normal kidney function
to develop adequate bladder function, including urinary continence
to provide acceptable appearance and function of the external genitalia

Although further surgery beyond the initial repair is sometimes required to meet these goals, the majority of patients with bladder exstrophy do achieve urinary continence and adequate bladder function, as well as normal sexual function and fertility potential.

For more information on bladder exstrophy, please view our health topic.


					Children's Hospital Boston es el principal hospital de enseñanza pediátrica de la Escuela de Medicina de Harvard.

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