Thomas Jefferson University Hospital Philadelphia, PA, United States
Gregory Habig, MD1, Daniel Garrido, MD1, Christopher Schmoyer, MD2, Leo Katz, MD1 1Thomas Jefferson University Hospital, Philadelphia, PA; 2Thomas Jefferson University Hospital, Collingswood, NJ
Introduction: Arteriovenous malformations (AVM), abnormal connections between arterial and venous vasculature, commonly cause gastrointestinal bleeding (GIB). Typically these diminutive lesions are a cause of chronic anemia, while large volume blood loss is extremely rare. We present a case of a man with a clinically significant GIB from a massive gastric AVM requiring multidisciplinary evaluation and treatment.
Case Description/Methods: A 20-year-old male with no relevant medical history presented with epigastric pain, vomiting, and anemia with hemoglobin 8.7 (12.5-15 g/dL). Computed tomography (CT) of the abdomen displayed a hyper-enhancing mass in the gastric fundus with multiple hypoattenuating foci throughout the liver. Subsequent esophagogastroduodenoscopy (EGD) revealed a large, soft submucosal mass without stigmata of bleeding in the gastric cardia concerning for a vascular lesion (Figure 1a). Magnetic resonance imaging demonstrated multiple hepatic serpiginous lesions with arterial enhancement consistent with AVM without evidence of portal hypertension (Figure 1b). Imaging of the lungs and brain did not reveal abnormal vascularity. The patient was discharged without issue but presented 2 years later with frank hematemesis. CT angiogram (CTA) revealed a 5.4cm lobulated gastric AVM supplied by the left gastric artery (Figure 1c). His course was complicated by acute blood loss anemia requiring multiple transfusions. Given the case’s complexity, a multidisciplinary discussion with surgery and interventional radiology (IR) was held before pursuing IR embolization of the left gastric artery with resolution of his symptoms. Given his presentation, clinical concern for Hereditary Hemorrhagic Telangiectasia (HHT) was high.
Discussion: Hematemesis from a massive gastric AVM is extremely rare. Arteriography is the gold standard in diagnosis as differentiation from other vascular pathology is difficult. However, the role of CTA is expanding. When observed with hepatic AVMs, the presence of HHT must be considered as similar disorders like capillary malformation-AVM syndrome and hereditary benign telangiectasia infrequently have GI involvement. Those with suspected HHT should undergo screening for remote lesions via imaging of the brain and lungs. Multiple management options exist including endoscopic intervention (clipping, cautery), intravascular intervention, and surgical resection. A multidisciplinary approach considering the lesion size and location is required.
Figure: Figure 1: a) EGD with a submucosal vascular lesion and adjacent gastritis b) MRI showing diffuse hypodensities (white arrows) concerning for AVMs c) 5.4cm lobulated vascular structure supplied by a dilated left gastric artery (black arrow).
Disclosures:
Gregory Habig indicated no relevant financial relationships.
Daniel Garrido indicated no relevant financial relationships.
Christopher Schmoyer indicated no relevant financial relationships.
Leo Katz indicated no relevant financial relationships.
Gregory Habig, MD1, Daniel Garrido, MD1, Christopher Schmoyer, MD2, Leo Katz, MD1. P0467 - Hematemesis From a Large Gastric AVM: An Uncommon Presentation of HHT, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.