R. Jori et al., PERFORATED SMALL-BOWEL DIVERTICULUM AS CA USE OF ACUTE ABDOMEN IN EHLERS-DANLOS SYNDROME, Helvetica chirurgica acta, 60(1-2), 1993, pp. 57-60
Based on a rare case of perforated small-bowel diverticulum combined w
ith Ehlers-Danlos syndrome (EDS) we investigated in a retrospective st
udy several swiss hospitals for symptomatic small-bowel diverticulosis
aiming at any clue on EDS. We came up with 15 more cases, 5 of them w
ith perforation, yet none showed any sign of EDS. A review of the lite
rature on either small-bowel diverticulosis or the intestinal complica
tions of EDS amounted to 131 cases of perforation and 11 cases of symp
tomatic 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-sp
ecific histopathological changes of the skin could not be shown in the
specimen of the intestinum, it seems quite reasonable to suggest an i
ncreased incidence of diverticulosis in patients with EDS, since the w
eakness of the connective tissue allows an easy protrusion of the muco
sa through the intestinal wall.