The surgical complications of Ehlers-Danlos syndrome

Between January 1975 and July 1995, 44 gastrointestinal and 45 vascular complications of Ehlers-Danlos syndrome type IV (EDS-IV) were reported in the literature. This included 41 colon perforations, 2 paraesophageal hernias, 22 spontaneous hemorrhages, 17 aneurysms, and 5 arterial dissections. Twenty-seven colonic perforations were treated with resection and diversion, 11 with total abdominal colectomy (TAC), and 3 with primary colon repair. Reperforation occurred in 15 resection/diversion patients versus none treated with TAC (P < 0.05). Seven patients (23.3%) died from their gastrointestinal complications. Eleven vascular complications were treated with nonoperative management, eight with vessel ligation, 20 with arterial reconstruction, and two with therapeutic angiography. Eighteen patients underwent angiography, with three (22%) major complications and one death (5.6%). Twelve (30%) patients died from vascular complications of EDS-IV, seven of whom had been treated with arterial reconstruction (P < 0.05). This review supports treating colon perforations in EDS-IV patients with TAC and end ileostomy to avoid a reperforation or an anastamotic leak. EDS-IV patients with vascular complications should be treated with nonoperative therapy and noninvasive imaging techniques when possible. Operative intervention, when required, should focus on simple vessel ligation rather than reconstruction.