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- $Unique_ID{BRK03727}
- $Pretitle{}
- $Title{Exstrophy of the Bladder}
- $Subject{Exstrophy of the Bladder Ectopia Vesicae}
- $Volume{}
- $Log{}
-
- 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.
-
-