[Perforated small intestine diverticulum as a cause of acute abdomen in Ehlers-Danlos syndrome]

Abstract

Based on a rare case of perforated small-bowel diverticulum combined with Ehlers-Danlos syndrome (EDS) we investigated in a retrospective study several swiss hospitals for symptomatic small-bowel diverticulosis aiming at any clue on EDS. We came up with 15 more cases, 5 of them with perforation, yet none showed any sign of EDS. A review of the literature on either small-bowel diverticulosis or the intestinal complications of EDS amounted to 131 cases of perforation and 11 cases of symptomatic small-bowel diverticulosis in combination with EDS, 2 of them suffering from perforation. Regarding the rarity of these diseases the number of patients presenting both is surprising. Although the EDS-specific histopathological changes of the skin could not be shown in the specimen of the intestinum, it seems quite reasonable to suggest an increased incidence of diverticulosis in patients with EDS, since the weakness of the connective tissue allows an easy protrusion of the mucosa through the intestinal wall.