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Megacystis and megacolon in an infant with Ehlers-Danlos syndrome
Authors:TADASHI SATO  HIROYUKI ITO  SUMIO MIYAZAKI  SHINICHIRO KOMINE  YUTAKA HAYASHIDA
Abstract:We report a 14 month old male infant with Ehlers-Danlos syndrome who became ‘anuric’ due to an acutely dilated urinary bladder. Although the patient was also found to have megacolon, no diverticulum was seen in his gastrointestinal tract or urinary bladder. In order to decompress the urinary bladder and colonic wall, we put an indwelling urinary catheter in place for 2 months, and carried out daily glycerin enema for 3 months. All urological and gastrointestinal symptoms subsided with this intensive medical treatment. The diagnosis of megacystis and megacolon was made very early in life for this patient. This may indicate that the striking extension of gastrointestinal and bladder wall may lead to the development of diverticula of gastrointestinal and urinary tracts in later life.
Keywords:Ehlers-Danlos syndrome  megacolon  megacystis
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