Dina Halegoua-De Marzio, MD, is an attending gastroenterologist and transplant hepatologist in practice at Thomas Jefferson University Hospital in Philadelphia, PA. Halegoua-DeMarzio will oversee the Case Conference blog, working with the fellows at Thomas Jefferson for the most interesting and engaging cases of the month.

Disclosure: Halegoua-DeMarzio reports she was an consultant for Intercept Pharma.

BLOG: A young adult with life-threatening hematemesis

Kunjal Gandhi, MD, is a second-year gastroenterology fellow at Thomas Jefferson University. She received her undergraduate and medical degree from University of Florida in Gainesville, Florida, and completed Internal Medicine residency at Johns Hopkins Hospital in Baltimore. She plans to pursue a career in academic inflammatory bowel disease.

Case presentation:

A 24 year-old healthy male presented to a local hospital with one-day history of bright red hematemesis and melena. He underwent emergent esophagogastroduodenoscopy (EGD) and was found to have a proximal gastric bleeding source. The underlying lesion was felt to be a Mallory-Weiss tear and endoclipping was performed.

Kunjal Gandhi

Over the next 4 days, he continued to have recurrent massive hematemesis requiring a total of 12 units packed red blood cells. He underwent three additional EGDs, all of which showed proximal gastric bleeding. There was concern for possible gastric arteriovenous malformation (AVM) on subsequent endoscopic exams. Further endoclipping and argon photocoagulation was performed with only temporary hemostasis. At this point, the patient was transferred to our institution for further management.

He underwent an emergent EGD shortly after arrival and was found to have active bleeding from the fundus. Many attempts at clearing the clot to visualize the underlying lesion were unsuccessful. He then underwent emergent angiography by Interventional Radiology. He was found to have a 2-inch arterial malformation between the cranial medial aspect of the spleen and the stomach, which was predominantly supplied by two splenic artery branches along with a small supply from the left gastric artery (Image 1).  

 

Since there was no active extravasation at the time of the study, focal embolization was not performed. A computed tomography angiogram was performed next to further characterize the vascular malformation (Images 2 and 3).  

 

Due to ongoing bleeding and failure of endoscopic and percutaneous management, the patient was taken to the operating room the following day. Exploratory laparotomy was performed with partial gastrectomy with resection of the gastric malformation. He did well post-operatively with no further signs of bleeding. He was discharged home 4 days later.

Discussion:

AVMs are defined as direct vascular connections between the arterial and venous components. They are commonly acquired with aging or occur secondary to other chronic medical conditions such as aortic stenosis and end-stage renal disease. They are scattered throughout the gastrointestinal tract, with the right colon being the most common location, followed by the small bowel. Bleeding is thought to occur due to abnormally high pressures in the venous side leading to rupture of the venule. When found by endoscopy, they can easily be treated with direct endoscopic therapies such as argon photocoagulation and use of endoclips.

AVMs are rare in the stomach and also rare in young adults, as in our patient. His vascular malformation was more likely representative of a congenital lesion, rather than acquired. Congenital lesions are suspected to form due to failure of embryonic vascular plexus to fully differentiate and develop a mature capillary bed. With aging, they can grow to impressive sizes. As the vessels erode through the gastrointestinal mucosa, massive hemorrhage ensues. In some instances congenital AVMs are thought to be the origin of Dieulafoy's lesions. Given the advancement of endoscopic therapies, most can be managed by gastroenterologists. Radiologic embolization and surgical resection can be necessary for larger malformations.

This case highlights the rare finding of a congenital vascular malformation in the stomach as a cause for massive life-threatening bleeding. The case also highlights the infrequent failure of endoscopic therapy in management of upper GI bleeding and importance of early rescue surgery.