Idiopathic Ileocolonic Varices: Combined GI-Surgery Approach for Management of a Rare Condition

Abstract

SymbolConclusion: A 37-year-old Hispanic female presented with history of recurrent episodes of hematochezia for 9 years requiring repeated hospital admissions. Physical examination was normal except for internal hemorrhoids. While initial hemoglobin was 11.3, it quickly trended down to 9.7. Upper endoscopy did not reveal findings suggestive of portal hypertension or any other bleeding source. Colonoscopy revealed engorged veins in the ascending colon and terminal ileum; there was no active bleeding. CT of the abdomen showed mild, segmental colonic wall thickening of the right colon that may represent submucosal venous engorgement. Liver was normal. CT angiography was unremarkable with no evidence of mesenteric vein or portal vein thrombosis. Capsule endoscopy was positive for large submucosal veins with edematous folds in the distal ileum. While work-up was ongoing, patient’s hematochezia recurred. Urgent tagged red cell scan showed abnormal activity in the right lower quadrant suspicious for the terminal ileum as the origin of the activity. Patient was taken to the operating room. An enterostomy was made in the terminal ileum and intraoperative endoscopy was performed to improve the visualization of the abnormal vasculature and thus aid the surgeon in demarcating the extent of surgical resection. The colectomy specimen revealed markedly dilated congested vessels with thrombus formation and adjacent hemorrhage, involving submucosa, muscle layer and subserosa. Varices of the colon are rare. Incidence in one autopsy series was 0.007%. Possible causes include congenital vascular anomaly, portal hypertension, and compromise of mesenteric vein circulation (thrombosis, extrinsic pressure, tumors, adhesions, kinking or twisting, and passive congestion). Liver disease with portal hypertension and portal/mesenteric thrombosis are the most common etiological factors. Idiopathic ileocolonic varices are extremely rare. According to the literature, only 38 cases have been reported to date. Diagnosis requires the exclusion of all other possible causes mentioned above. Age at diagnosis varies from 14 to 81 years. Several reported cases have shown an associated familial aggregation; some authors have suggested a possible autosomal recessive mode of inheritance. Sixty-seven percent of documented cases involved the entire colon. Thirteen cases required at least a partial resection of the bowel. However, conservative management was deemed prudent in most cases. This video demonstrates a need for coordination between surgery and gastroenterology departments to accurately assess the resection margin intraoperatively. Our patient’s lower GI bleed resolved. There has been no recurrence following the surgery.Symbol