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$Title{Exstrophy of the Bladder}
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Copyright (C) 1986 National Organization for Rare Disorders, Inc.

195:
Exstrophy of the Bladder

** IMPORTANT **
It is possible the main title of the article (Exstrophy of the Bladder)
is not the name you expected.  Please check the SYNONYMS listing to find the
alternate names and disorder subdivisions covered by this article.

Synonyms

     Ectopia Vesicae

General Discussion

** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only.  It should not be used for diagnostic or treatment
purposes.  If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.


Exstrophy (the turning inside out) of the Bladder is a developmental
abnormality marked by the absence of a portion of the lower abdominal wall
and the posterior vesical (bladder) wall.  The posterior vesical (bladder)
wall turns outward through the opening.  Urine is excreted through this
opening in the abdominal wall.

Symptoms

Exstrophy of the Bladder is characterized by incontinence (inability to hold
back urine).  The pubic arch is open and the ischia (parts of the hip bone)
are widely separated and connected by a fibrous band.  The connection between
the ureter (tube from the kidney to the bladder) and the bladder is
constricted, and the ureters are dilated (wider than usual).  If this
condition is not corrected, urine will start flowing back into the ureters,
and pyelonephritis (inflammation of the kidneys) and renal (kidney) failure
are possible.

Causes

Exstrophy of the Bladder is a congenital disorder caused by defective
development.  It may possibly be due to rupture of the bladder during fetal
life, or transposition of the earliest embryologic form of the organ
resulting from a change in position of the vitelline duct (a duct in earliest
embryologic development).

Affected Population

Exstrophy of the Bladder is a congenital abnormality which occurs in males 7
times more often than in females.

Therapies:  Standard

Exstrophy of the Bladder is corrected by surgically implanting the ureter
into the sigmoid part of the large intestine (ureterosigmoidostomy) with or
without a colostomy (surgical creation of an opening between the colon and
the surface of the abdominal wall near the implantation).  Another common
procedure to correct this disorder is ileal (to the ileum) or colon loop
urinary diversion; i.e., the diversion of the urinary flow by surgically
connecting the ureter to a loop of the ileum (the last part of the small
intestine), or to the colon (the part of the large intestine before the
rectum).

Reconstruction of the genitalia when necessary is usually begun by the
age of two years.  The prognosis for maintenance of normal kidney function is
relatively good.

Therapies:  Investigational

This disease entry is based upon medical information available through
December 1988.  Since NORD's resources are limited, it is not possible to
keep every entry in the Rare Disease Database completely current and
accurate.  Please check with the agencies listed in the Resources section for
the most current information about this disorder.

Resources

For more information on Exstrophy of the Bladder, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     (203) 746-6518

     National Support Group for Exstrophy of the Bladder
     5075 Medhurst Street
     Solon, OH  44139
     (216) 248-6851

     Simon Foundation
     P.O. Box 815
     Wilmette, IL  60091
     (321) 864-3913

     H.I.P.(Help for Incontinent People)
     P.O. Box 544
     Union, SC  27379

     National Kidney and Urologic Diseases Information Clearinghouse
     Box NKUDIC
     Bethesda, MD  20892
     (301) 468-6345

References

THE MERCK MANUAL, 15th ed., Robert Berkow, M.D., ed in chief, published by
Merck, Sharp & Dohme Research Labs, Rahway, NH, 1987.  Pp. 1957.

THE CECIL TEXTBOOK OF MEDICINE, 18th Ed.:  James B. Wyngaarden and Lloyd
H. Smith, Jr., Eds; W.B. Saunders Co., 1988.  Pp. 2107, 650.