Prophylactic colectomy in Ehlers-Danlos syndrome with colonic ectasia

An adolescent boy with clinical features of Ehlers-Danlos syndrome developed ectasia of the colon leading to intractable constipation and clinically imminent colonic perforation. Total colectomy with ileorectal anastomosis in two stages was performed eliminating not only his intractable constipation but also the risk of potentially lethal perforation. This case illustrates an approach that may reduce the high mortality rate reported in patients with Ehlers-Danlos syndrome and colonic perforation.