Hereditary Colonic Varices‐Image of the Month

Abstract

A 17-year-old adolescent boy presented with significant hematochezia, orthostatic hypotension and anemia, with normal coagulation studies. He required transfusion of 4 units of packed red blood cells. Colonoscopy (Figure) revealed findings throughout the colon and in the terminal ileum. Small-bowel enteroscopy results were negative; arteriography findings were normal, without evidence of portal hypertension. The episode resolved without sequelae. He had a similar episode several months later, not requiring transfusion, and a previous episode at age 2 years. His mother had a diagnosis of angiodysplasia of the ileum and right colon, requiring ileocecal resection at age 42 years; his maternal grandfather had a lifelong history of severe gastrointestinal bleeding, necessitating transfusions, but had not undergone colonoscopy.Figure: Colonic varices in the descending colon.The endoscopic images demonstrate colonic varices, which may be hereditary in patients without portal hypertension. The varices are tortuous in appearance with a purplish discoloration. The mechanism of inheritance and etiology of varix formation are unknown (1). Patients may be asymptomatic for years between bleeding episodes. Identification of varices during an episode of active bleeding may be difficult because of variceal decompression caused by decreased intravascular volume or excessive air insufflation at colonoscopy. Identification of an actively bleeding varix is infrequent at colonoscopy (1). The rectosigmoid colon, descending colon and cecum are the sites most frequently involved. Ileal involvement may also occur. Although some patients can be managed conservatively, in patients with recurrent bleeding and extensive involvement, surgical resection remains the mainstay of therapy. Argon plasma coagulation has been effective in a single case of an adult with localized colonic varices who was not a surgical candidate because of metastatic disease (2). Submitted by: Marsha Kay Robert Wyllie Department of Pediatric Gastroenterology and Nutrition The Cleveland Clinic Foundation Cleveland, Ohio