Lemmel syndrome, a rare cause of obstructive jaundice by periampullary duodenal diverticulum: Case report and review of the literature

Abstract

Lemmel syndrome is a pancreaticoduodenal disease caused by compression of the mid or distal common bile duct by a periampullary diverticulum. This condition should be considered a rare complication of a duodenal diverticulum and an unusual cause of obstructive jaundice. Because of its infrequent occurrence and non-specific clinical presentation, Lemmel syndrome can mimic other conditions. We herein report the clinical and imaging findings (computed tomography, magnetic resonance imaging) of a patient who presented with intermittent abdominal pain and jaundice. Large air-filled outpouching lesions of the duodenum compressed the biliary duct, resulting in upstream biliary ductal dilatation that led to the diagnosis of Lemmel syndrome.

Keywords: Lemmel syndrome; Obstructive jaundice; Periampullary duodenal diverticulum.

Figure 1:

The schematic representation of periampullary diverticulum (PAD). (a) The normal size of PBD which joins to the ampulla of Vater, marking the entry point of bile into the second part of the duodenum. (b) Extrinsic compression of the biliary tract by the PAD causing PBD dilatation. PBD: Principal bile duct, PAD: Periampullary duodenal diverticulum, d: duodenum. The schematic representation was reproduced with permission from “Frauenfelder G, Maraziti A, Ciccone V, Maraziti G, Caleo O, Giurazza F, et al. Computed tomography imaging in Lemmel syndrome: A report of two cases. J Clin Imaging Sci 2019;9:23.”

Figure 2:

An 82-year-old man with Lemmel syndrome who presented with intermittent abdominal pain and jaundice. (a and b) Axial and coronal contrast-enhanced computed tomography (CT) scan of the abdomen shows dilation of the intrahepatic duct (arrow in a) and common bile duct (CBD) (arrow in b) without demonstrable cholelithiasis. (c) CT image with coronal reconstruction shows a large air-filled periampullary duodenal diverticulum (asterisk) showing narrow communication with the duodenal lumen (open arrow) was located adjacent to the distal CBD (arrow in c).

Figure 3:

An 82-year-old man with Lemmel syndrome who presented with intermittent abdominal pain and jaundice. (a and b) Coronal T2-weighted magnetic resonance imaging of the abdomen shows an 8.3-cm periampullary diverticulum (asterisk) mechanically compressing the biliary duct with distal common bile duct (CBD) (arrow in a) and pancreatic ductal dilatation (open arrow in b). Occluded cholangiogram showed dilated CBD without filling defect (c).