Lemmel’s Syndrome: Usual Presentation of an Unusual Diagnosis

Abstract

Lemmel's syndrome causes obstructive jaundice in the absence of stones or tumors. The most common cause is the presence of periampullary diverticula which arise within 2-3 cm from the ampulla of Vater. Diverticula may be extramural or intramural. Despite current practice of obtaining imaging studies such as ultrasound, CT, and MRI, endoscopic retrograde cholangiopancreatography (ERCP) is the gold standard diagnostic test. Lemmel's syndrome should be considered when pancreaticobiliary disease is suspected. We present a case in which our patient presented with abdominal pain, fever, and transaminitis who underwent ERCP which was successful in diagnosis of Lemmel's syndrome and its treatment. Although rare, it is imperative for physicians to recognize this syndrome in order to deliver prompt care.

Lemmel’s Syndrome Secondary to Common Bile Duct Compression by an Inflamed Duodenal Diverticulum

“…Lemmel’s syndrome is the extrinsic compression of the distal common bile duct by a periampullary diverticulum, causing obstructive jaundice in the absence of choledocholithiasis or tumors of the pancreaticobiliary system [ 1 , 2 ]. First described in 1934 by Dr. Gerhard Lemmel, Lemmel’s syndrome mimics other pancreaticobiliary diseases, making diagnosis very challenging [ 1 ].…”

“…About 75% of duodenal diverticula are found in the descending duodenum, adjacent to the ampulla of vater [ 4 ]. Periampullary diverticula can be extramural or intramural, and most are extraluminal giving it ample opportunity to compress the common bile duct [ 2 , 3 ].…”

“…Up to 5% can become symptomatic and/or lead to complications such as hemorrhage, fistula, and perforation. Extrinsic compression of the common bile duct can give rise to complications such as obstructive jaundice, acute pancreatitis, and bacterial overgrowth leading to cholangitis [ 2 , 3 ].…”

“…Although noninvasive testing, such as CT scans, can support diagnosis, endoscopic retrograde cholangiopancreatography (ERCP) or endoscopic ultrasound (EUS) remain the preferred diagnostic tool [ 1 , 2 ]. Given the rarity, it is important for clinicians to utilize history and physical exams, aided by ERCP, in order to promptly recognize and treat Lemmel’s syndrome [ 1 ].…”

Evaluation of the Effectiveness of the use of Pantoprazole and the Risks

“…PPIs are authenticated by FDA for treatment of erosive esophagitis associated with GERD, and for treatment of hyper secretory conditions like Zollinger-Ellison syndrome. And also they have various off-label uses to treat H pylori bacteria, to prevent peptic ulcer re-bleeding, NSAID induced ulcers and stress ulcer prophylaxis [3] PPIs are marketed 40 years ago and till now their safety and efficacy are impeccable. But emerging literature causes concern for PPI safety over long term usage.…”