Abstract
Diverticular disease is a major cause of hospitalizations, especially in the elderly. Although diverticulosis and its complications predominately affect the colon, the formation of diverticula in the small intestine, most commonly in the duodenum, is well characterized in the literature. Although small bowel diverticula are typically asymptomatic, and diagnosed incidentally, a complication of periampullary duodenal diverticulum is Lemmel syndrome. Lemmel syndrome is an extremely rare condition whereby periampullary duodenal diverticula, most commonly without diverticulitis, leads to obstruction of the common bile duct due to mass effect and associated complications including acute cholangitis and pancreatitis. Here, we present the first case, to our knowledge, of periampullary duodenal diverticulitis complicated by Lemmel syndrome with concomitant colonic diverticulitis with colovesical fistula. Our case and literature review emphasizes that Lemmel syndrome can present with or without suggestions of obstructive jaundice and can most often be managed conservatively if caught early, except in the setting of emergent complications.
Introduction
Diverticulosis is characterized by the protrusion of blind sac-like pouches, known as diverticula, through weaker regions of the gastrointestinal (GI) wall while maintaining connection with the GI lumen. 1 Although the majority of patients with diverticulosis are asymptomatic, diverticular disease is a major cause of hospitalization and patient mortality, especially in the elderly.1,2 Specifically, diverticular disease, which affects 25% of patients, is characterized by clinically significant and symptomatic diverticulosis which includes diverticulitis (5%), diverticular hemorrhage, symptomatic uncomplicated diverticular disease, and segmental colitis associated with diverticula. 2 Although diverticulosis and its complications predominately affect the colon, the formation of diverticula in the small intestine is well characterized in the literature.
Non-Meckel’s small bowel diverticula are rare, with a reported prevalence of 0.5% to 1.9%, and most commonly affects the duodenum. 3 Although patients with small bowel diverticula are typically asymptomatic, and diagnosed incidentally, rarely complications can manifest in approximately 5% of those with duodenal diverticula including small bowel obstruction, diverticulitis, and GI bleeding.3-5 Diverticulitis of periampullary diverticula located 2 to 3 cm from the ampulla of Vater, that is, periampullary duodenal diverticulitis, is rare and scarcely reported in the literature. 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.
Case Report
A 74-year-old female with a history of sigmoid diverticulitis 9 months prior, history of C. difficile colitis, gastroesophageal reflux disease, hypertension, coronary artery disease, and a surgical history of bariatric surgery, hernia repair, hysterectomy and appendectomy, presented to the emergency department for progressive epigastric, right upper quadrant (RUQ), and left upper quadrant (LUQ), abdominal pain for 1 day. The pain was described as a discomfort that did not radiate to the back, shoulders, or jaw and was not related to meals. She reported nausea, and recent loose stools which had resolved. She denied fever, chills, emesis, hematemesis, hematochezia, melena, dysuria, sick contacts, international travel, and changes to her diet. She did not smoke or drink alcohol.
She was afebrile (36.5°C), with a normal heart rate (65 bpm), but was hypertensive (159/71 mm Hg), mildly tachypneic (20 breaths/min) and with an oxygen saturation of 98%. On physical exam, she was not jaundiced and did not have scleral icterus. There was pain on palpation of the epigastrium, as well as the RUQ and LUQ on abdominal exam. There was no pain in the left lower quadrant. The abdomen was soft and non-distended without any peritoneal signs.
Labs revealed a mild leukocytosis with a white blood cell (WBC) count of 10.1 × 109/L. Aspartate aminotransferase (AST) and alkaline phosphatase (ALKP) were slightly elevated at 67 and 144 U/L, respectively. Alanine aminotransferase (ALT) and total bilirubin (0.4 mg/dL) were normal. Urine analysis showed significant pyuria (> 100 WBC/high power field) and urine culture grew Escherichia coli and multidrug resistant Klebsiella aerogenes. Electrocardiogram (EKG) showed normal sinus rhythm and suggestions of left ventricular hypertrophy which was unchanged from previous. Computed tomography (CT) of the abdomen and pelvis was suggestive of mild/early acute on chronic sigmoid diverticulitis complicated by colovesical fistula. In addition, findings were suggestive of diverticulitis of a periampullary duodenal diverticulum with new mild dilation of the biliary tree which was to represent Lemmel syndrome (Figure 1).
Computed tomography of the abdomen and pelvis with IV contrast demonstrating periampullary duodenal diverticulitis (solid white arrow) with new mild dilation of the common bile duct (dotted white arrow) and intrahepatic bile ducts (white arrowheads).
She was started on intravenous antibiotics and remained hemodynamically stable. Colorectal surgery was consulted regarding the colovesical fistula and recommended outpatient follow-up for further management. She was discharged on an oral antibiotic regimen for periampullary duodenal and colonic diverticulitis, which also covered the urinary pathogens. Repeat CT of the abdomen and pelvis approximately 2 weeks after discharge showed colonic diverticulosis and a periampullary duodenal diverticulum without evidence of diverticulitis. At this time, the abnormal AST and ALKP had also normalized.
Discussion
Lemmel syndrome is an exceptionally unique entity that was first described in 1934 and has been scarcely reported in the literature since then. 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.5,6,8 No standardized diagnostic imaging recommendations have been made but CT of the abdomen and pelvis may have the best sensitivity but may lack specificity in differentiating between duodenal diverticulum and a mass located at the head of the pancreas, prompting further imaging such as Magnetic Resonance Cholangiopancreatography (MRCP) or Endoscopic Ultrasound with Fine Needle Biopsy (EUS-FNB).5,8 Similarly, there are no standardized recommendations regarding management, but this may involve conservative treatment (antibiotics, hydration, high-fiber diet, diverticular lavage) in the setting of mild cases, to endoscopic sphincterotomy with or without stenting, or surgical management with diverticulectomy, in more complicated emergent cases.5,6,8
Although periampullary duodenal diverticulum causing Lemmel syndrome is rare, our case, which involved periampullary duodenal diverticulitis as the etiology is even more unique. An extensive literature search for published case reports of periampullary duodenal diverticulitis causing Lemmel syndrome was conducted in PubMed/Medline and Embase since inception. This search yielded only 4 relevant articles consisting of 5 cases.6-9 All 5 cases were reported after 2010 which may signify increased awareness of Lemmel syndrome. Adding our case to this, the average age (standard deviation; SD) of patients was 53.2 ± 25.7 years old, where the large SD was due to 2 cases being less than 30 at the time of diagnosis (Table 1). There was also a prominent female predominance (5/6 cases). As expected, 5 cases presented with abdominal pain where at least 4 of these were localized to the epigastric region and/or RUQ. Interestingly, only 2 cases had reported jaundice and/or scleral icterus, 2 cases had no such finding, and for the remaining 2 cases, this information was not provided. Similarly, only 3 cases had an elevated total bilirubin, where for 2 of them, the elevation was mild and less than 3 mg/dL. The AST, ALKP, inflammatory markers, and ALT were elevated in 5, 4,4, and 3 patients, respectively.
Summary of Cases of Patients With Periampullary Duodenal Diverticulitis Complicated by Lemmel Syndrome.
Hx: history; Dx: diagnostic; Tx: treatment; NS: not stated; WBC: white blood cell; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; AST: aspartate aminotransferase; ALKP: alkaline phosphatase; ALT: alanine aminotransferase; CBD: common bile duct (CBD stone = choledocholithiasis); NG: nasogastric; IV: intravenous; CT: computed tomography; RUQ: right upper quadrant, LUQ: left upper quadrant; PBC: primary biliary cirrhosis; HTN: hypertension; ERCP: endoscopic retrograde cholangiopancreatography.
Two cases, including ours, did not demonstrate prominent evidence of obstructive jaundice. Our patient sought medical care immediately due to the pain related to the diverticulitis, which likely explains the lack of signs of obstructive jaundice on presentation. The most common complication of Lemmel syndrome was acute pancreatitis affecting 50% of cases, followed by acute cholangitis which was diagnosed in 33% of cases. Our patient did not have any complications of Lemmel syndrome and also did not present with obstructive jaundice suggesting that the duodenal diverticulitis was most likely caught and managed early preventing significant obstruction of the CBD, obstructive cholestasis, and associated complications. In addition, our patient is the only reported case with concomitant colonic diverticulitis. Although CT of the abdomen and pelvis was utilized as the initial diagnostic modality for the majority (5/6) of patients, many cases involved further imaging modalities such as MRCP. Nearly all (5/6) cases were managed only conservatively, with 66% of patients having resolution/improvement of the periampullary duodenal diverticulitis on follow-up imaging.
The major strength of our case was that the periampullary duodenal diverticulitis complicated by Lemmel syndrome was diagnosed and treated expeditiously, which may have prevented associated complications. Another strength was that our paper was formatted according to the CARE guidelines for case reports. 10 However, 1 limitation of our case is that we did not obtain subsequent MRCP while the patient was hospitalized, although we did plan for this in the outpatient setting. Nevertheless, follow-up CT showed resolution after management suggesting against a pancreatic mass.
In conclusion, although small bowel diverticula are typically asymptomatic, and diagnosed incidentally, a complication of periampullary duodenal diverticula, and more rarely diverticulitis, is Lemmel syndrome.3-5 Here, we presented the first case, to our knowledge, of periampullary duodenal diverticulitis complicated by Lemmel syndrome with concomitant colonic diverticulitis with colovesical fistula. Our case and literature review emphasizes that Lemmel syndrome can present with or without suggestions of obstructive jaundice and can most often be managed conservatively if caught early, except in the setting of emergent complications.
Footnotes
Author Contributions
B.S.: contributed to conceptualization, data extraction, formal analysis, writing—original draft, and writing—review & editing final manuscript. S.J. contributed to data extraction and writing—review & editing final manuscript. J.N.F. contributed to writing—review & editing final manuscript. V.K.-D. contributed to writing—review & editing final manuscript. A.V. contributed to writing—review & editing final manuscript. W.M. contributed to writing—review & editing final manuscript and supervision.
Data Availability Statement
Most data generated or analyzed during this study are included in this article. Further inquiries can be directed to the corresponding author.
Declaration of Conflicting Interests
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding
The author(s) received no financial support for the research, authorship, and/or publication of this article.
Ethics Approval
Our institution does not require ethical approval for reporting individual cases.
Informed Consent
All patient information has been de-identified. Written informed consent was obtained from the patient for their de-identified information to be published.
Submission Declaration and Verification
This manuscript has not been published previously or in a different language. In addition, this manuscript is not simultaneously being submitted for publication elsewhere and is not currently under consideration at a different journal. The publication of this manuscript is approved by all authors. If this manuscript is accepted, it will not be published elsewhere in the same form, in English or in any other language, including electronically without the written consent of the copyright-holder.
