1343 A Rare Case of Lemmel Syndrome

Abstract

INTRODUCTION: Lemmel syndrome is a rare condition in which a periampullary duodenal diverticulum compresses the distal common bile duct (CBD) resulting in obstructive jaundice in the absence of choledocholithiasis or tumor. We present a rare case of Lemmel syndrome in an elderly patient below. CASE DESCRIPTION/METHODS: An 83-year-old woman with a past medical history of Crohn's disease and pancreatic cysts was transferred to our facility for management of biliary obstruction. She had noted a four-month history of progressive early satiety, post-prandial right upper quadrant abdominal pain, post-prandial emesis, poor oral intake, and significant weight loss. On presentation, labs were significant for WBC of 11.6 k/mm3, AST of 242 U/L, ALT of 1677 U/L, alkaline phosphatase of 1347 U/L, total bilirubin of 3.4 mg/dL, and lipase of 260 U/L. She underwent ERCP and was found to have a large periampullary diverticulum containing the major papilla. Cholangiogram revealed a tortuous bile duct and severe intra- and extrahepatic biliary dilation. A sphincterotomy was performed and multiple balloon sweeps were done with no stone extraction. A 10 F × 8 cm fully covered metal biliary stent (FCSEMS) was placed in the bile duct and she had improvement in her appetite, abdominal pain, and liver function tests. On follow up ERCP, the bile duct maintained a straightened position. DISCUSSION: Lemmel syndrome is characterized by obstructive jaundice caused by periampullary duodenal diverticulum (PAD) compressing the intra-pancreatic part of the CBD with resultant upstream dilatation of the extra- and intra-hepatic bile ducts. PAD is a pseudo-diverticula consisting of an extraluminal out-pouching of duodenal mucosa and lacks a muscularis layer. PAD develops within a 2-3 cm radius from the ampulla of Vater. Periampullary duodenal diverticuli are mostly asymptomatic and majority are diagnosed incidentally. When inflamed, they can cause pancreaticobiliary complications such as obstructive jaundice, cholangitis, pancreatitis, and stone formation. They are diagnosed on imaging such as CT scan, MRCP, and barium studies, which can show lateral compression of the distal CBD by the diverticulum. ERCP with sphincterotomy and biliary stent placement has shown to reduce the risk of morbidity and mortality, although diverticulectomy remains standard of care for PAD. Lemmel syndrome is rare but should be considered in the differential diagnosis for obstructive jaundice in patients with a periampullary duodenal diverticulum.