Bladder Exstrophy-Epispadias-Cloacal Exstrophy Complex
National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Bladder Exstrophy-Epispadias-Cloacal Exstrophy Complex is not the name you expected.
Bladder exstrophy-epispadias-cloacal exstrophy complex is a spectrum of anomalies involving the urinary tract, genital tract, musculoskeletal system and sometimes the intestinal tract. In classic bladder exstrophy, most anomalies are related to defects of the abdominal wall, bladder, genitalia, pelvic bones, rectum and anus.
Bladder exstrophy is a rare developmental abnormality that is present at birth (congenital) in which the bladder and related structures are turned inside out. The rear portion of the bladder wall (posterior vesical wall) turns outward (exstrophy) through an opening in the abdominal wall and urine is excreted through this opening. The extent of the exstrophy depends on how large the opening is. The mildest form is when there is a defect or opening in the tube that carries urine out of the body from the bladder (urethra) and is termed epispadias.
The most severe form is when there is a defect in the urethra, bladder and bowel (cloacal exstrophy). Classic bladder exstrophy is when there is a defect in the urethra and bladder and is intermediate in severity. The underlying cause of this complex is not known. The physical characteristics are the result of a developmental abnormality during embryonic growth in which the cloacal membrane is not replaced by tissue that will form the abdominal muscles.
Association For Bladder Exstrophy Community
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Birth Defect Research for Children, Inc.
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Genetic and Rare Diseases (GARD) Information Center
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NIH/National Kidney and Urologic Diseases Information Clearinghouse
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National Association for Continence
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Urology Care Foundation
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- Website: http://www.urologyhealth.org/
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".
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Last Updated: 8/17/2007
Copyright 2007 National Organization for Rare Disorders, Inc.
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