Literature DB >> 8379331

Megacystis and megacolon in an infant with Ehlers-Danlos syndrome.

T Sato1, H Ito, S Miyazaki, S Komine, Y 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.

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Year:  1993        PMID: 8379331     DOI: 10.1111/j.1442-200x.1993.tb03071.x

Source DB:  PubMed          Journal:  Acta Paediatr Jpn        ISSN: 0374-5600


  2 in total

Review 1.  Visceroptosis of the bowel in the hypermobility type of Ehlers-Danlos syndrome: presentation of a rare manifestation and review of the literature.

Authors:  Eyal Reinstein; Mark Pimentel; Mitchel Pariani; Stephen Nemec; Thomas Sokol; David L Rimoin
Journal:  Eur J Med Genet       Date:  2012-07-07       Impact factor: 2.708

Review 2.  Urogenital and pelvic complications in the Ehlers-Danlos syndromes and associated hypermobility spectrum disorders: A scoping review.

Authors:  Elizabeth Gilliam; Jodi D Hoffman; Gloria Yeh
Journal:  Clin Genet       Date:  2019-09-01       Impact factor: 4.438

  2 in total

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