Endoscopic management of pancreatic and biliary duct stenoses due to a giant pseudoaneurysm in a patient clinically suggestive of Loeys-Dietz syndrome

Loeys-Dietz syndrome is a rare connective tissue disorder characterized by the formation of aggressive arterial aneurysms. There are a few reports of Loeys-Dietz syndrome with pseudoaneurysms causing simultaneous pancreatic and biliary stenoses. Herein, we report the case of a 42-year-old man with Loeys-Dietz syndrome who presented with acute pancreatitis and liver dysfunction caused by a giant pancreaticoduodenal artery pseudoaneurysm compressing the main pancreatic and bile ducts. To minimize the risk of pseudoaneurysm rupture during endoscopic intervention, transcatheter arterial embolization was performed, followed by endoscopic intervention. Although initial clinical improvement was observed after endoscopic stent placement, a fistula between the pancreatic duct and the thrombosed pseudoaneurysm was detected at 4 months but spontaneously closed with continued stenting. Despite persistent ductal stenosis requiring long-term stent management, the fistula had closed 1 year after the initial stent placement. To the best of our knowledge, this is the first report describing a structured treatment strategy for pancreaticobiliary obstruction caused by a Loeys-Dietz syndrome-related pseudoaneurysm. This case highlights the importance of a stepwise interventional radiology-first approach and careful follow-up for the management of complex vascular compressive syndromes.