Ehlers-Danlos syndrome complicated by eventration of the diaphragm, colonic perforation and jejunal perforation--a case report.

A case of a 61 year old woman with Ehlers-Danlos syndrome who developed recurrent gastrointestinal complications is reported herein. She has been followed by Kyoundo Hospital since 1965, when she originally underwent treatment for eventration of the diaphragm and a volvulus of the stomach at the age of 41. During the past 22 years she has experienced perforation of the colon three times and jejunal perforation once. This case was considered to be type II Ehlers-Danlos syndrome. We have found 12 other cases of Ehlers-Danlos syndrome with gastrointestinal complications in the Japanese literature. More thorough examination of Ehlers-Danlos syndrome cases may reveal more subclinical gastrointestinal abnormalities. We concluded from our experience and from the literature that when colonic perforation occurs in this syndrome, total colectomy and ileo-rectal anastomosis is reasonably indicated.