S4790 Repeat UGIB in the Setting of Portal Vein Atresia: A Unique Presentation to a Unique Diagnosis

Introduction: The most common causes of upper gastrointestinal bleeding (UGIB) include peptic ulcer disease, erosive esophagitis, arteriovenous malformations, and esophageal varices. One of the most common causes of esophageal variceal bleeding is due to portal hypertension, commonly caused by decompensated cirrhosis. Congenital Absence of Portal Vein, otherwise known as portal vein atresia, is a rare cause of portal hypertension leading to development of esophageal, gastric, and rectal varices, all of which can lead to GI bleeding. We present a rare case of a repeat UGIB caused by variceal bleeding due to portal vein atresia, presenting in the third decade of life. Case Description/Methods: The patient is a 32-year-old man with a prior medical history of B12 deficiency anemia, Iron Deficient Anemia, recent admission to outside hospital for upper gastrointestinal (GI) bleed thought to be secondary to esophageal varices from recently diagnosed Portal Vein Atresia, who presented to the emergency department with melena. Upon review of previous esophagogastroduodenoscopy (EGD), findings were notable for medium sized, non-bleeding esophageal varices. It should also be noted the patient had multiple images of the abdomen and pelvis, negative for chronic liver changes, yet notable for atretic portal vein with cavernous transformation with splenomegaly, consistent with portal vein atresia. On admission to our facility, patient's Hgb was 10.4, which was his baseline. Due to stable Hgb, the patient decided to leave against medical advice. He then presented back to our facility 2 days later with similar complaints of dark stools, with a Hgb 10.6. Due to continued dark stools, patient was agreeable to EGD, which was notable for 4 cords of grade III esophageal varices, with stigmata of recent bleeding with red wale sign, for which x1 band was placed with adequate hemostasis. Discussion: Portal Vein Atresia is a rare congenital anomaly, commonly diagnosed at an early age, with reported 30% of cases before the age of 5-year-old, and most others during childhood or adolescents. Due to its effect of portal vasculature and portal hypertension, this rare diagnosis can lead to UGIB due to esophageal variceal formation. Our case highlights the importance of a broad differential when it comes to causes of UGIB. Although peptic ulcer disease and esophagitis are at the forefront of most gastroenterologist’s minds when it comes to UGIB, more rare causes should always be on the differential so adequate treatment then subsequent follow up can be established.