THE SURGICAL COMPLICATIONS OF EHLERS-DANLOS SYNDROME

Between January 1975 and July 1995, 44 gastrointestinal and 45 vascula r complications of Ehlers-Danlos syndrome type IV (EDS-IV) were report ed in the literature,This included 41 colon perforations, 2 paraesopha geal hernias, 22 spontaneous hemorrhages, 17 aneurysms, and 5 arterial dissections. Twenty-seven colonic perforations were treated with rese ction and diversion, 11 with total abdominal colectomy (TAG), and 3 wi th primary colon repair. Reperforation occurred in 15 resection/divers ion 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 v essel ligation, 20 with arterial reconstruction, and two with therapeu tic angiography. Eighteen patients underwent angiography, with three ( 22%) major complications and one death (5.6%). Twelve (30%) patients d ied from vascular complications of EDS-IV, seven of whom had been trea ted with arterial reconstruction (P < 0.05). This review supports trea ting 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 rec onstruction.