Abstract

INTRODUCTION: Duodenal varices (DVs) represent 17% of ectopic varices (EVs), have a 4-fold increased bleeding risk when compared to esophago-gastric varices (EGVs), and mortality rate up to 40%. There are no guidelines on the ideal management of DVs. We report a case of massive hemorrhage secondary to DV managed successfully with transjugular intrahepatic portosystemic shunt (TIPS). CASE DESCRIPTION/METHODS: A 69-year-old gentleman presented with hematemesis, hematochezia, dizziness and near-syncope. He was diagnosed with cryptogenic cirrhosis, non-alcoholic steatohepatitis in the past after negative autoimmune and infectious work-up. Prior doppler ultrasound showed elevated portal flow velocity; esophagogastroduodenoscopy (EGD) a month prior revealed portal hypertensive gastropathy (PHG) but no EGVs, and normal duodenum. Given prior hepatic encephalopathy, TIPS was not performed. He had abdominal surgeries in the past. He was hemodynamically unstable with benign abdominal exam. Labs revealed acute blood loss anemia with Hgb 9.1 (baseline 13), thrombocytopenia and coagulopathy. Following initial resuscitation with fluids, blood products, octreotide and pantoprazole infusion, urgent EGD was performed. There were no EGVs, but features of diffuse PHG, as well as a compressible mucosal bulge with a nipple sign representing a varix in the second portion of the duodenum were seen. Given the insufficient evidence to support endoscopic therapy in ectopic varices, interventional radiology was consulted. CT angiogram demonstrated patent portosystemic veins. TIPS was performed with a decrease in portal gradient from 16 to 2 mmHg. Rifaximin, lactulose and zinc were commenced. He remained hemodynamically stable following this and did not require further intervention. A year later, he remains free of further bleeding episodes or hepatic encephalopathy. DISCUSSION: Bleeding DVs are rare and potentially life-threatening. Diagnosis should be suspected in all cases of gastrointestinal hemorrhage, particularly in the absence of EGV or another source. Standard management has not yet been established. TIPS is a relatively safe and effective means of controlling acute DV bleeding. EVs may re-bleed despite a reduction of portosystemic pressure gradient < 12 mmHg or by 25–50% of baseline; hence the “12 mmHg rule” does not apply. Our patient recovered successfully following TIPS without needing additional therapy. Reducing the pressure gradient to very low values can result in successful outcomes with TIPS in DVs.

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