Small-Intestinal Variceal Hemorrhage: Successful Treatment with Transjugular Intrahepatic Portosystemic Shunt

Abstract

Purpose: A 48 y/o female with a history of alcoholic cirrhosis and portal hypertension presented with 2 episodes of large hematochezia with severe epigastric and periumbilical abdominal pain. Labs revealed an acute decline in hemoglobin (Hgb) from 11 to 8 gm/dL. An abdominal CT obtained to evaluate her abdominal pain revealed changes suggestive of portal hypertension including multiple collaterals, small intestinal (SI) varices, splenorenal shunting, and a recanalized paraumbilical vein. Intraluminal blood in the distal SI was noted, thought to be in the region of a SI varix and potentially the etiology of her symptoms. Fluid resuscitation, blood transfusion, IV proton pump inhibitor and octreotide therapy were initated. However, she continued to have persistent hematochezia with a further drop in Hgb to 6.3 gm/dL. Emergent EGD did not demonstrate any lesion as a source of bleeding. Subsequent CT angiography showed pooling of contrast in the distal loop of ileum with large SI varices. Attempts at angiographically controlling bleeding were unsuccessful. Given the findings of portal enteropathy with large SI varices, emergent therapeutic TIPS was performed for acute control of SI bleeding. Post-TIPS HVPG was documented as 7 mmHg. Subsequently, her bleeding stopped and her Hgb improved. Overall, she received 10 units of packed RBC, 6 units of FFP and 4 units of platelet transfusion. Portal hypertension, usually a complication of cirrhosis, presents as ascites, splenomegaly, and bleeding from esophageal, gastric, and ectopic varices. Ectopic enteric varices including duodenal, mesenteric, anorectal, peristomal, and rarely ileal varices are an unusual cause of GI bleeding accounting for 1-5% of all variceal bleeding. While the incidence of bleeding is low, this may result in severe, life threatening hemorrhage with a mortality approaching 40%. Therapeutic options for variceal bleeding include endoscopic sclerotherapy, vasoactive drugs, segmental bowel resection, portosystemic shunt surgery, oversewing of varices, and TIPS procedures with or without focal variceal embolization. Endoscopic management is limited as very often visualization of SI varices is not achieved because of their distal location and relatively small size. Unless the underlying portal hypertension is treated, suture ligation of varices as well as sclerotherapy have high rebleeding rates. Surgical portocaval shunting is effective with low rebleeding rates, but is only recommended in patients who can tolerate a major surgical procedure. TIPS can effectively reduce the portocaval gradient, and consequently, the risks of bleeding decrease drastically. This is especially indicated for patients that are poor surgical candidates.