[Ehlers-Danlos syndrome revealed by sigmoid perforation].
We report a case of colonic perforation revealing Ehlers -Danlos syndrome type IV in a male adult. This syndrome is a heritable disorder of collagen synthesis. Its prognosis is severe resulting in vascular rupture or bowel perforation. In his surgical strategy, the surgeon has to cope with the recurrent feature of the colonic perforation. In order to prevent other perforations, a therapeutic scheme has been set up according to literature.
Bedda
S
S
Service de chirurgie digestive, CHI Poissy, 20, rue Armagis, 78100 Saint-Germain-en-Laye, France. sbedda@yahoo.fr <sbedda@yahoo.fr>
Radovanovic
A
A
Fajardy
A
A
Bataille
N
N
Montariol
T
T
D000328:Adult; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000175:diagnosis*; D006801:Humans; D007416:Intestinal Perforation / Q000209:etiology* / Q000601:surgery; D008297:Male; D011379:Prognosis; D012008:Recurrence; D012810:Sigmoid Diseases / Q000209:etiology* / Q000601:surgery