Colon perforation in Ehlers-Danlos syndrome. Report of two cases and review of the literature.

Two cases of spontaneous perforation of the colon in Ehlers-Danlos syndrome type IV are reported, and eight cases previously reported in the English literature are reviewed. Emergency surgery for acute free colon perforation in patients with Ehlers-Danlos syndrome should be carried out in the following standard manner: exteriorization of the perforation or resection of the involved colon with end colostomy, and either closure of the rectal stump (Hartmann procedure) or distal mucous colostomy. The reported experience although small, indicates that a high incidence of recurrent perforation can be expected if bowel continuity is reestablished. Permanent colostomy status, therefore, appears advisable, despite the young ages of most of the patients.