Ectopic Small Bowel Varices - A Rare Cause of Obscure Gastrointestinal Bleed

Abstract

Introduction: Ectopic varices (ECV) are a rare cause of gastrointestinal bleed (GIB) and account for about 1-5% of variceal bleeding. Their location in the small bowel (SB) is even rare and represents a clinical, diagnostic and therapeutic challenge. Here we present an interesting case of SB varices (SBV) presenting a lower GIB. Case Report: A 55 year old man with known hepatitis C, human immunodeficiency virus (HIV) and hepatocellular carcinoma (HCC) presented with recurrent melena. He had multiple prior admissions for GIB and had multiple esophagogastroduodenoscopies (EGD) and colonoscopies in the past. His hemoglobin was 5.6 gm/dl, platelet count 244,000/μl and internationalized normalized ratio (INR) was 1.7. EGD showed grade III esophageal varices with no stigmata of recent bleeding. Colonoscopy revealed active bleeding from ileocecal valve. Bleeding scan was positive for active bleed likely within small bowel. Mesenteric angiography showed no evidence of bleeding or arterio-venous malformation (attempts to show a venous phase were unsuccessful). Computerized tomography angiography identified a large ectopic varix in the small bowel as the source of bleeding. This varix was connecting the superior mesenteric vein to the right inferior epigastric vein. The patient was referred for surgical evaluation. Discussion: Portal hypertension (PHT) usually causes gastro-esophageal varices (GEV). ECV, although rare, can occur in any other part of gastrointestinal (GI) tract including small bowel and missing these lesions can have grave consequences. Bleeding associated with SBV can be life-threatening. A triad of portal hypertension, hematochezia, and prior abdominal surgery is characteristic of SBV. Diagnosis is fairly challenging and a high index of suspicion is needed. SBV should be considered as a possibility in any patient with GIB and portal hypertension, especially if no stigmata of recent bleeding from GEV is noted. Differential diagnostic modalities including radiological imaging, nuclear scans, angiography and endoscopy; and a multidisciplinary approach which includes a hepatologist, endoscopist, interventional radiologist and surgeon is usually needed for appropriate diagnosis and optimal therapy. Conclusion: ECV represents a particular challenge to clinicians as they are difficult to identify, diagnose and treat. Bleeding from ECV has significant morbidity and mortality and hence clinicians should consider ECV as a possibility in patients with PHT presenting as lower GIB especially if they have non-bleeding GEV.