Recurrent ascending cholangitis with acute pancreatitis and pancreatic atrophy caused by a juxtapapillary duodenal diverticulum

Abstract

Rationale: Intermittent combined pancreaticobiliary obstruction may lead to multiple episodes of ascending cholangitis and pancreatitis, usually due to choledocholithiasis or periampullary mass. However, one of the rare causes is periampullary or juxtapapillary duodenal diverticulum. Although duodenal diverticula are relatively common in the general population, the overwhelming majority are asymptomatic. Duodenal diverticula can cause combined pancreaticobiliary obstruction through multiple mechanisms such as stasis-induced primary choledocholithiasis, stasis-induced intradiverticular enterolith, or longstanding diverticulitis, causing stenosing fibrosing papillitis or a combination of more than one of these mechanisms. Herein, I report a case of Lemmel syndrome due to a combination of multiple mechanisms and review the available literature on the epidemiology, pathogenesis, clinical presentation, diagnostic work-up, and management of juxtapapillary duodenal diverticulum. Patient concerns: Multiple episodes of abdominal pain, jaundice, anorexia, fever, and significant unintentional weight loss. Diagnoses and interventions: Primary choledocholithiasis, recurrent ascending cholangitis, recurrent acute pancreatitis, and pancreatic atrophy due to giant juxtapapillary duodenal diverticulum, with unsuccessful endoscopic retrograde cholangiopancreatography that was completely resolved after open transduodenal sphincteroplasty and septoplasty, transampullary and transcystic common bile duct exploration and stone extraction, and duodenal diverticular inversion. Outcome: Complete resolution of combined pancreaticobiliary obstruction without recurrence for 2 years after surgery. Lessons: Surgeons should be aware of such rare syndromes to avoid misdiagnosis and delayed or inappropriate management. Furthermore, they should understand the different available operative options for cases that are refractory to endoscopic approach.

Concomitant Sigmoid Diverticulitis and Periampullary Duodenal Diverticulitis Complicated by Lemmel Syndrome: A Case Report

“… 5 The presentation can be non-specific and individual cases may require different management strategies depending on the complications that arise including choledocholithiasis, acute cholangitis, and pancreatitis. 5 , 7 - 9 Indeed, although this condition classically presents with obstructive jaundice, this is not seen in all cases, similar to our patient. 5 , 6 Although the most common pathophysiology explaining Lemmel syndrome appears to be CBD obstruction by periampullary duodenal diverticula, other proposed mechanisms include (1) Irritation of the ampulla of Vater by the periampullary duodenal diverticulum and subsequent chronic inflammation and fibrosis, (2) Sphincter of Oddi dysfunction due to the diverticulum.…”

“… 5 Lemmel syndrome is an extremely rare condition whereby periampullary duodenal diverticula, most commonly without diverticulitis, leads to obstruction of the common bile duct (CBD) due to mass effect and associated complications such as acute cholangitis and pancreatitis. 6 - 8 Here, we present an exceptionally rare case of periampullary duodenal diverticulitis complicated by Lemmel syndrome with concomitant colonic diverticulitis with colovesical fistula.…”

Juxta-papillary duodenal diverticula are associated with pyogenic liver abscesses: a case control study

“…JPDD have been associated with obstructive jaundice in the absence of choledocholithiasis, described as Lemmel’s syndrome [ 13 ]. This syndrome has also been described in patient presenting with cholangitis [ 14 ]. In our study, five (7.6%) cases with PLA had concurrent cholangitis.…”

A Rare Presentation of Duodenal Diverticulum Causing Obstructive Jaundice: Lemmel's Syndrome

“…The pancreatic atrophy observed in our patient could be explained by an isolated pancreatic obstruction producing recurring episodes of acute pancreatitis and pancreatic atrophy. This obstruction could be caused by choledocholithiasis and sludges caused by stasis, stenosing papillitis brought on by intermittent episodes of diverticulitis, or intra-diverticular enterolith [ 15 ]. In asymptomatic patients, no treatment is suggested.…”