Obscure GI Hemorrhage due to Idiopathic Ileal Varices in a 14-Year-Old Boy

Abstract

Background: Ectopic varices (EV) are a rare cause of obscure GI bleeding, and are frequently fatal due to the rapid bleeding and difficulty localizing the source. Typically, EV are associated with portal hypertension from liver disease or disruption of portal circulation, prior surgeries, and tumors. Idiopathic small bowel varices are rare, particularly in the pediatric population, and are often difficult to diagnose due to their location. Bleeding from these lesions can be rapid and fatal Recognition of this condition is thus important to reduce morbidity. We present a rare case of recurrent GI. bleeding due to idiopathic ileal varices in 14-year-old boy. Case: An otherwise healthy 14 -year-old boy was referred to our institution for assessment of overt obscure GI hemorrhage. The patient had been hospitalized on two prior occasions for gastrointestinal hemorrhage, with subsequent Hgb levels as low as 5.0 g/dL. He had undergone a colonoscopy and upper endoscopy during each prior hospitalization, which were unremarkable, with the exception of tortuous, dilated rectal veins. Prior CT of the abdomen and pelvis, barium enema, and Meckel's scan were unremarkable. A Capsule endoscopy was also reportedly unremarkable. Prior to this episode, the patient had been healthy, with only a history of lactose intolerance, but had experienced a similar episode of overt obscure GI hemorrhage 5 years prior, for which imaging and endoscopic assessment were also negative. We performed an anterograde double-balloon enteroscopy (DBE) to the distal ileum, which was unremarkable, with the exception of increased vasculature in the distal ileum. Subsequent retrograde DBE revealed small rectal varices and large varices 15 cm in length in the terminal ileum near the I.C. Valve. The tattoo from the anterograde DBE was identified 20 cm proximal to these varices. Evaluation for coagulopathy, portal hypertension, and liver disease were unremarkable. The venous phase of an angiography revealed large ileal varices. Review of the previous capsule endoscopy images revealed distal ileal varices. Exploratory laparotomy revealed multiple large ileal varices extending into the ileocolonic region, which were resected. No evidence of portal hypertension was detected. Pathology revealed arteriovenous malformation. The patient has experienced no further bleeding over the past 12 months. Discussion: We present a rare case of ileal varices in a juvenile. While this is uncommon, recognition of small bowel varices is essential to avoid misdiagnosis. Since treatment consensus has not been established, early recognition of this entity and the underlying etiology of ectopic varices may facilitate therapeutic decision-making.