Exstropy
What is it?
Exstrophy of the bladder is a congenital defect in
which the bladder is open to the outside and turned inside
out, so that its inside is visible at birth and protruding
from the lower abdomen.
Who gets it?
Exstrophy is rare, occurring in about one in 40,000
births.
What causes it?
The cause of the defect is not well known, but lack of
prenatal care and nutrition has been linked to many birth
defects, including exstropy.
What are the symptoms?
Exstrophy of the bladder is the most severe bladder
abnormality. The patient has no control over urination and
is more likely to develop bladder cancer. The defect may
also cause urine reflux, which may lead to chronic bladder
infections. It can also cause a separating of the pubic
bones, which may affect gait. In males, the penis may be
shortened.
How is it diagnosed?
Exstrophy causes the bladder to protrude from the
abdomen, so it is often diagnosed at birth after the
initial examination. Frequently other congenital defects
will accompany exstrophy, so a doctor may search for other
abnormalities such as large bowel or a hernia.
What is the treatment?
Surgery is necessary and can usually produce successful
results. If possible, the surgery should be done within 48
hours of birth. If there is a delay in surgery, the doctor
may divert the urine into the bowel because the partially
repaired bladder cannot control the flow. Patients
frequently require multiple operations, including bladder
augmentation and bladder neck reconstruction.
In addition, recent research suggests bone defects
associate with exstrophy are more extensive than
previously thought. Researchers at the Children’s Center
and St. Vincent de Paul’s Hospital in Paris found that the
most pronounced differences are an outward flare of the
upper part of the hip bone and a rotation of the entire
pelvic girdle from the bottom forward. The research
broadens surgeons' understanding of those bone defects so
that more effective adjustments can be made in realigning
and repositioning bones of the pelvis.
Self-care tips
With immediate surgery, most patients will have control
of their urine and no long-term consequences. However, the
rate of infection is greater for those with congenital
bladder defects, since any abnormality in the urinary
system opens it to invasion by bacteria.
This information has been designed as a comprehensive and quick reference
guide written by our health care reviewers. The health information written
by our authors is intended to be a supplement to the care provided by your
physician. It is not intended nor implied to be a substitute for
professional medical advice.
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