Ileal Varices Causing Massive Gastrointestinal Hemorrhage in a Cirrhotic Patient

Abstract

Purpose: Introduction: Esophageal variceal hemorrhage is a common complication of portal hypertension, but rarely ectopic varices may present a diagnostic challenge. Bleeding from non-stomal jejunal and ileal varices is exceedingly rare; we report a case of recurrent massive gastrointestinal hemorrhage from ileal varices in a patient with alcohol related cirrhosis. Case: A 53-year-old male with a history of alcoholic cirrhosis presented with bright red blood per rectum. His previous surgical history included the repair of an umbilical hernia 4 years ago. He was hypotensive with a hemoglobin of 4 gm/dl. He had multiple previous admissions for obscure gastrointestinal bleeding over the previous 6 months. Prior workup included multiple upper endoscopies showing grade 1 esophageal varices and mild portal hypertensive gastropathy with no active bleeding, an unremarkable colonoscopy and a negative nuclear bleeding scan. A capsule endoscopy showed active bleeding in the distal ileum, followed by single balloon enteroscopy from below showing multiple terminal ileal arteriovenous malformations which were treated with Argon plasma coagulation. He was treated as an out patient with subcutaneous octreotide and a transplant evaluation was started. During this hospitalization, a visceral angiogram was done and was negative. The patient required continuous transfusion of blood products to stabilize the active bleeding. A computed tomography (CT) scan of the liver showed collateral vessels arising from the paraumbilical vein adjacent to the distal small bowel. A venogram further defined this as a cluster of ileal varices. Subsequently, the ileal varices were coil embolized and a transjugular intrahepatic portosystemic shunt (TIPS) was inserted. His bleeding stopped after this. He received a total of 63 units of PRBCs, 48 units of FFPs, 70 cryoprecipitate and 56 units of platelets. His liver function declined and his MELD score was 24. He underwent orthotopic liver transplantation 4 days after TIPS. Intra-operatively he was noted to have multiple adhesions around the distal small bowel. His post operative course was unremarkable, with no recurrence of bleeding. Conclusion: Ileal varices represent a rare form of ectopic varices in patients with portal hypertension and their formation may be the result of collateral circulation through postoperative adhesion between the ileum and the abdominal wall. Diagnosis is a challenge, and endoscopy is rarely definitive. A high index of suspicion and the utilization of imaging modalities including CT, angiography, and nuclear studies can assist in establishing the diagnosis and prompt treatment.