Extrahepatic Biliary Dilatation Research Articles

A new classification and laparoscopic treatment of extrahepatic choledochal cyst

BackgroundPrior typing methods fail to provide predictive insights into surgical complexities for extrahepatic choledochal cyst (ECC). This study aims to establish a new classification system for ECC through clustering of imaging results. Additionally, it seeks to compare the differences among the identified ECC types and assess the levels of surgical difficulty. MethodsThe imaging data of 124 patients were automatically grouped through a K-means clustering analysis. According to the characteristics of the new grouping, corrections and interventions were carried out to establish a new classification. Demographic data, clinical presentations, surgical parameters, complications, reoperation, and prognostic indicators were analyzed according to different types. Factors contributing to prolonged surgical time were also evaluated. ResultsA new classification system of ECC: Type A (upper segment), Type B (middle segment), Type C (lower segment), and Type D (entire bile duct). The incidences of comorbidities (calculus or infection) were significantly different (P = 0.000, P = 0.002). Additionally, variations in the incidence of postoperative biliary stricture were statistically significant (P = 0.046). The operative time was significantly different between groups (P = 0.001). Age, BMI > 30, classification, and the presence of combined stones exhibit a significant association with prolonged operative time (P = 0.002, P = 0.000, P = 0.011, P = 0.011). ConclusionIn conclusion, our utilization of machine learning-driven cluster analysis has enabled the creation of a novel extrahepatic biliary dilatation typology. This classification, in conjunction with factors like age, combined stone occurrence, and obesity, significantly influences the complexity of laparoscopic choledochal cyst surgery, offering valuable insights for improved surgical treatment.

Computed tomographic features of exocrine pancreatic carcinomas in dogs and cats.

Exocrine pancreatic carcinomas are uncommon in dogs and cats, and diagnosis with diagnostic imaging can be challenging. This retrospective, multi-institutional, descriptive study was performed to evaluate the CT features of exocrine pancreatic carcinomas. The CT examinations of 18 dogs and 12 cats with exocrine pancreatic carcinomas diagnosed by cytology or histopathology were reviewed. The CT features of exocrine pancreatic carcinomas included a well-defined mass in 28/30 (93%) with contrast enhancement in 27/30 (90%), commonly heterogeneous 22/30 (73%); often with a nonenhancing fluid to soft tissue attenuating center 12/30 (40%). The right lobe of the pancreas was the most common location, 14/30 (47%), then the left lobe, 10/30 (33%), and the body, 6/30 (20%). Extrahepatic biliary duct dilation was present in six animals; 5/6 (83%) of the masses were located in the right pancreatic lobe. Additional findings included peripancreatic fat-stranding 17/30 (57%), lymphadenopathy 16/30 (57%), peripancreatic soft tissue nodules 12/30 (40%), and free fluid 10/30 (33%). When comparing the imaging features of dogs and cats, there was a large overlap in imaging characteristics. There was a significant difference between the height of the masses, with dogs having larger masses (P-value.0028). Lymphadenopathy was more likely in larger masses [increased height (P-value.029)]. Cats were significantly older than dogs (P-value.0355). Pancreatic carcinomas were commonly identified as masses with heterogeneous contrast enhancement and a nonenhancing fluid to soft tissue attenuating center with concurrent peripancreatic changes (fat-stranding and/or soft tissue nodules) and lymphadenopathy.

Atypical localization of metastatic melanoma in the gastrointestinal tract.

Although most melanomas are diagnosed in early stages of the disease, a significant percentage show metastases from the beginning or during follow-up. The most common route is lymphatic, while hematogenous spread is less frequent. The most common site for metastases is the skin itself, although they can also occur in other organs, including the digestive tract, where the small intestine is the most frequently affected organ (2). We present the case of a 48-year-old woman with a history of superficial spreading melanoma of the cervical region, surgically treated 7 years prior and in remission, who presented with painless jaundice of 48 hours duration, without fever or other infectious focus. Upon arrival at the Emergency Department, she had a direct bilirubin of 7.9 mg/dL and a CRP of 140 mg/L. An abdominal ultrasound was performed, revealing biliary tract dilation without apparent cause, with secondary, apparently tumoral involvement of the gallbladder. During hospitalization, the study was completed with an abdominal CT scan, visualizing hyperdense lesions with a polypoid appearance in the gallbladder, in addition to the aforementioned intra- and extrahepatic biliary tract dilation. The cause was then identified as a hyperdense mass in the duodenum affecting the papilla, along with two other small hypervascular polypoid lesions in the second portion of the duodenum. With these findings, an ERCP was performed, revealing a papilla with a "pregnant" appearance, compatible with ampulloma, in addition to duodenal pseudopolyps of nonspecific appearance. The anatomopathological analysis was compatible with duodenal metastases from melanoma. Melanoma is the cancer with the highest mortality rate, and the gastrointestinal tract is a typical location for metastases, only surpassed by the skin itself and the lungs, especially the small intestine, although not the duodenum, where they are found in only 12% of cases (4). In this case, the diagnosis was complicated by the endoscopic appearance of the lesion, as the characteristic features are pigmented, blackish lesions typical of melanoma. As seen in the image, these lesions had an erythematous and pseudo-inflammatory appearance, so for diagnosis and treatment, it was essential to consider the history of the disease to perform an adequate anatomopathological diagnosis.

THU369 A Rare Case Of IgG4-related Autoimmune Pancreatitis And Diabetes Mellitus

Abstract Disclosure: M. Zahid: None. J. Shakil: None. Introduction: IgG4 related disease (IgG4-RD) is a rare multiorgan inflammatory condition characterized by dense lymphoplasmacytic infiltrates, presence of IgG4+ plasma cells and fibrosis. Here we present a case of young male with painless jaundice and hyperglycemia diagnosed with IgG4 related autoimmune pancreatitis and DM. Clinical Case: 46-year-old male with no significant history presented to outpatient clinic for pruritis and dark-colored urine of 2-week onset. He reported 17-pounds weight loss in the preceding month. While undergoing outpatient workup, patient developed epigastric pain prompting visit to the hospital. On examination, patient was afebrile, tachycardiac, icteric and had epigastric tenderness. Labs revealed normal electrolytes and renal function. LFTs were significant for total protein 7.6 g/dl (n=6.3-8.3 g/dl), AST 77 U/L (n = 10-50 U/L), ALT 127 U/L (n = 5-50 U/L), alkaline phosphatase 458 U/L (n = 40-129 U/L) and direct bilirubin >10.0 (n = 0-0.3 mg/dl). Lipase was 99 U/L (n - 13-60 U/L), blood glucose was 292 mg/dl and A1C was 6.5% (n = 4.0-5.6%). US abdomen revealed diffuse gallbladder wall thickening with moderate sludge and intrahepatic and extrahepatic distal biliary obstruction and dilatation. MRCP showed common bile duct and intrahepatic biliary ductal dilation. Pancreas was diffusely irregular with areas of hemorrhagic pancreatitis. ERCP showed dilatation of upper and middle third of main bile duct, left and right hepatic ducts and all intrahepatic branches with a stricture in distal bile duct. Biliary sphincterotomy and placement of temporary plastic biliary stent was performed and ampullary biopsies were taken. IgG4 level was checked due to suspicion for IgG4-RD and was 293 mg/dl (n: 1-123 mg/dl). Pathology revealed mucosa with epithelial reactive changes. CD138 stain showed several mature plasma cells within the inflammatory infiltrate. In addition, immunostain for IgG4 shows several IgG4 positive plasma cells forming small clusters confirming diagnosis of IgG4-RD. Post procedure, his abdominal pain and LFTs started improving. Patient was started on prednisone for IgG4-RD and insulin for persistent hyperglycemia. His prednisone dose was later tapered after starting methotrexate. Patient’s most recent LFTs have normalized except mildly elevated ALT and A1C improved to 6.2%. Conclusion: Autoimmune pancreatitis and cholangiopathy are the pancreatobiliary manifestation of IgG4-RD and most often present as painless jaundice mimicking pancreatic cancer. Endocrine insufficiency usually manifests as diabetes mellitus/pre-diabetes and may also lead to autoimmune hypophysitis and Reidel’s fibrosis. Other organ systems that may also get involved include lacrimal and salivary glands, lungs, kidneys, nervous system, and lymphatic/vascular system. Early recognition and ongoing surveillance are key to appropriate management of this condition. Presentation: Thursday, June 15, 2023

An Unusual Variant of Anomalous Pancreaticobiliary Junction.

A 27-year-old previously healthy African American female presented to the Emergency Department with an acute onset of epigastric abdominal pain and nausea. Laboratory studies proved unremarkable. CT scan demonstrated intrahepatic and extrahepatic biliary ductal dilation with possible stones within the common bile duct. The patient was discharged with a surgery follow-up appointment. Laparoscopic cholecystectomy with intraoperative cholangiography was performed 3weeks later due to concern for choledocholithiasis. The intraoperative cholangiogram showed multiple abnormalities, concerning for an infectious or inflammatory process. Magnetic resonance cholangiopancreatography (MRCP) demonstrated a suspected anomalous pancreaticobiliary junction and cystic lesion near the pancreatic head. Endoscopic retrograde cholangiopancreatography (ERCP) for cholangioscopy showed normal-appearing pancreaticobiliary mucosa with 3 tributaries directly from the pancreas entering the bile duct and an ansa orientation to the pancreatic duct. Biopsies of the mucosa were benign. Annual MRCP and MRI to assess for findings concerning for neoplasm given the anomalous pancreaticobiliary junction were recommended.

A160 A SARCOMATOID CARCINOMA OF THE COMMON BILE DUCT PRESENTING AS PAINLESS JAUNDICE AND EXTRAHEPATIC BILIARY OBSTRUCTION

Abstract Background Sarcomatoid carcinomas are extremely rare tumors made of epithelial and mesenchymal elements. They have been found in various organs, but presence in the common bile duct (CBD) has only been reported a handful of times. Insight regarding the clinical history, histopathology, treatment, and prognosis is limited. The majority of CBD sarcomatoid carcinomas have occurred in elderly women, including this case. Surgical resection is the mainstay of treatment and the roles for chemotherapy and radiation therapy are undetermined. Prognosis is variable, but generally poor. Purpose Additional information regarding sarcomatoid carcinomas of the CBD will aid in establishing a timely diagnosis and may alter treatment options and prognosis. We aim to add to the limited literature surrounding this rare CBD neoplasm. Method A 71-year-old female presented with painless jaundice, decreased appetite, and weight loss. Initial investigations showed an alkaline phosphatase (ALP) of 3075 U/L, aspartate transaminase (AST) of 507 U/L, alanine aminotransferase (ALT) of 298 U/L, total bilirubin of 325.5 µmol/L, and direct bilirubin of 254.1 µmol/L. Initial computed tomography (CT) scan done showed marked intra- and extrahepatic biliary ductal dilation with appropriate tapering and the presence of a distal CBD hyperdensity. Tissue biopsy obtained by endoscopic retrograde cholangiopancreatography (ERCP) was suspicious for a malignant peripheral nerve sheath tumor. The differential also included synovial sarcoma and sarcomatoid mesothelioma. Result(s) After multidisciplinary discussion involving hepatobiliary surgery, medical oncology, and radiation oncology, pancreaticoduodenectomy was performed; there was no role for neoadjuvant/adjuvant chemotherapy/radiation therapy. Final pathology revealed a well circumscribed mass with a narrow attachment to the posterior CBD, measuring 5.6 x 3.2 x 2.8 cm. Immunohistochemistry showed mixed differentiation with sarcomatoid, squamous, and glandular components, consistent with a sarcomatoid carcinoma. H3K27me3 expression was lost in neoplastic cells. Immunostaining showed strong expression of vimentin and weak expression of CD34, calretinin, CK5 and EMA. Post-operative course was complicated by pancreaticojejunal leak, surgical wound infection, myocardial injury, and esophageal stricturing. 14-weeks post-pancreaticoduodenectomy the patient was found to have C.difficile infection and a perforated viscus, exact location of which was not visible on imaging. Emergent laparotomy revealed a gastroduodenal leak and diffuse small bowel ischemia. The patient passed away shortly after emergent surgery. Conclusion(s) This case illustrates a rare presentation of sarcomatoid carcinoma within the CBD and highlights some of the diagnostic challenges, limited management strategies, complications, and potential poor prognosis of the disease. Further research is required to guide diagnosis and management, and ultimately improve patient outcomes. Please acknowledge all funding agencies by checking the applicable boxes below None Disclosure of Interest None Declared

PRIMARY SCLEROSING CHOLANGITIS AND AUTOIMMUNE HEPATITIS: OVERLAPPING CHALLENGES IN DIAGNOSIS AND TREATMENT

Abstract Overlap syndromes (OS) describe the simultaneous presentation of more than one immune-mediated liver disease in a singular patient, and present unique challenges both in diagnosis and treatment. Here we report a case of primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) OS, ultimately treated with Vancomycin. A 30-year-old female with known ulcerative pancolitis (UC) and alcoholic cirrhosis in the setting of AIH presented to the ED with severe abdominal pain, bloody diarrhea, and bilious emesis. Initial labs showed ESR 48 mm/hr, CRP 32.97 mg/L, fecal calprotectin 1131.2 µg/mg, AST 45 IU/L, and total protein 9.1 g/dL. Clostridium Difficile and stool studies were negative. Patient was admitted with concern for acute UC flare. CT scan showed mild worsening of intrahepatic and extrahepatic biliary ductal dilatation suspicious for PSC, and a cirrhotic liver with signs of portal hypertension and splenomegaly (Fig. 1a). Endoscopy revealed multiple non-bleeding duodenal ulcers and grade II esophageal varices. Colonoscopy found moderately active UC Mayo score 2, worsened from prior exam. MRCP was significant for cirrhotic liver morphology with nodular surface contour, and intrahepatic and extrahepatic biliary dilatation with beading and irregularity of the intrahepatic biliary tree and cystic duct, consistent with PSC (Fig. 1b). She was diagnosed with PSC-AIH OS and underwent treatment with Prednisone, Azathioprine, and Ustekinumab with minimal improvement. Only after the addition of Vancomycin was there normalization of biochemical markers and clinical status. Autoimmune liver disease is generally categorized into hepatic (AIH) or cholestatic (PSC and PBC) predominance. PSC is known to be associated with IBD, specifically UC. It is hypothesized that PSC evolves in UC patients due to uptake into the enterohepatic circulation of proinflammatory cells from the colon; these agents typically concentrate in the biliary system where they ultimately cause the biliary ductal damage frequently seen with PSC. By targeting enteric bacteria, Vancomycin decreases the absorption of these inflammatory biliary toxins. For PSC patients with UC and co-occurring AIH, the addition of Vancomycin could significantly reduce mortality and disease burden. Several case reports and one pilot study have investigated Vancomycin’s role in the treatment of PSC. However, it remains a vastly underutilized therapy, and its role specifically in PSC-AIH OS has not been previously reported. There remains an evident need for a large double blind randomized placebo-controlled clinical trial to further support the addition of Vancomycin to the standard treatment regimen for PSC, and to establish clear parameters on effective dosage and length of treatment. In the meantime, clinicians should be aware of the possible use of Vancomycin to treat patients with PSC +/- AIH and IBD. Figure 1a: CT abdomen pelvis with IV contrast highlighting stigmata of cirrhosis with portal hypertension and splenomegaly. Image also shows worsening of intrahepatic and extrahepatic biliary ductal dilatation in the setting of PSC given the patient’s underlying history of ulcerative colitis. Figure 1b: MRCP showing beading and irregularity of the intrahepatic biliary tree and cystic duct, in keeping with known history of primary sclerosing cholangitis. This image also highlights the dilation of the intrahepatic and extrahepatic biliary system.

Type IVA Choledochal Cyst in Adult: A Case Report

Choledochal cyst is a congenital disease of the biliary tract with extrahepatic and/or intrahepatic biliary tree dilatation. There is a female majority (4:1). Controlling biliary sepsis and curing pancreatitis are prerequisites of surgery. Here, a 35-year-old female patient, came with complaints of epigastric and right upper abdominal pain for three days. On examination per abdomen was soft, non distended, right hypochondriac tenderness was present, no guarding, bowel sounds were audible. Magnetic Resonance Cholangiopancreatography (MRCP) was done which showed fusiform dilatation of Common Bile Duct (CBD), Common Hepatic Duct (CHD), right and left hepatic duct, along with mild dilatation of central intrahepatic biliary radicals (4.8-5 mm)- suggestive of choledochal cyst type IVA. Hepatico-duodenostomy was done. The entire extrahepatic biliary tree should be removed when the choledochal cyst is diagnosed whether or not symptoms are present. Despite being congenital, the condition is typically discovered in adulthood as well. The treatment of choledochal cysts has evolved. The current standard of care is cyst excision and hepaticojejunostomy

Concurrence of novel mutations causing Gilbert’s and Dubin–Johnson syndrome with poor clinical outcomes in a Han Chinese family

Dual-hereditary jaundice (Dubin–Johnson syndrome (DJS) and Gilbert’s syndrome (GS)) is a rare clinical entity resulting from defects of the ATP binding cassette subfamily C member 2 (ABCC2) and UDP glucuronosyltransferase family 1 member A1 (UGT1A1) genes with autosomal recessive inheritance. In this study, we aimed to investigate the mutation profiles and characterize the phenotypes in a Han Chinese family with DJS and GS. Genetic screening for variants in the ABCC2 and UGT1A1, immunohistochemistry for expression of ABCC2, and histopathological examination were carried out. The proband and his brother had unconjugated and conjugated hyperbilirubinemia after birth. The proband’s sister had only conjugated hyperbilirubinemia after birth. The proband developed into pleural effusions and ascites, pericardial thickening, intrahepatic and extrahepatic biliary duct dilatation, and enlarged gallbladder at age 50. Hepatocellular carcinoma occurred in the proband’s brother at age 46. Seven compound defects of the ABCC2 gene [c.2414delG, p.(Ile1489Gly), p.(Thr1490Pro), and p.(Ile1491Gln)] and the UGT1A1 gene (c.-3279T>G, p.(Gly71Arg), and p.(Pro451Leu)) were identified in family members. Accumulation of pigment in hepatocytes characteristic of that in DJS was present in the proband and his brother. Expression of ABCC2 protein was markedly diminished in the patient’s liver. Our results show a different genetic profile of DJS and GS in a Han Chinese family, indicating a more complex pattern of dual-hereditary jaundice among different populations. The present study illuminates the underpinnings of DJS and GS and extends the mutation profiles and phenotypes of these two syndromes in dual-hereditary jaundice.

Immune Checkpoint Inhibitor–Mediated Hepatobiliary Toxicity: What the Surgeon Should Know

INTRODUCTION: Immunotherapy with immune checkpoint inhibitors (ICIs) has revolutionized cancer treatment. However, immune-related adverse effects are increasingly recognized. Hepatobiliary toxicity can manifest radiographically as inflammatory and neoplastic changes that mimic surgically treated disease. The aim of this study is to investigate pitfalls of ICI-mediated hepatobiliary toxicity that require treatment with steroids, and not operation. METHODS: Patients evaluated in a hepatobiliary clinic for elevated liver function test while receiving ICI therapy for various malignancies were analyzed. Cross-sectional imaging and ultrasounds were evaluated for anatomic abnormality. RESULTS: Among 44 consecutive patients evaluated for elevated liver function test while receiving immunotherapy, 5 patients (11%) had imaging that showed anatomic abnormality. MRI and/or CT showed intra- and extrahepatic biliary ductal dilation in 3 patients, including 1 patient with imaging concerning for acute and chronic cholecystitis; hepatic pseudotumor in 1 patient, and abnormal liver enhancement in 1 patient (Figure). Among the 5 patients, 1 patient underwent operation for a presumed diagnosis of cholecystitis and choledocholithiasis. However, cholecystectomy was aborted due to findings of obscured anatomy and dense inflammation surrounding the hepatoduodenal ligament. All 5 patients received treatment with steroids with improvement in liver function test.FigureCONCLUSION: Anatomic abnormality involving the liver and biliary tree develops in a minority of patients receiving cancer treatment with ICIs. In patients who develop biliary ductal dilation and/or thickened gallbladder wall on ICI therapy, surgeons should have a heightened suspicion of an immune-related adverse event.

S1841 The Curious Case of CA 19-9 in Mirizzi Syndrome

Introduction: Mirizzi Syndrome (MS) is a rare complication of gallstone disease, caused by an obstructing stone in the cystic duct or the gallbladder neck, leading to obstruction of the common hepatic duct. This case discusses the utility of EUS in improving diagnostic yield of MS and the importance of cancer screening in these patients. Case Description/Methods: A 44-year-old male with a history of gastritis presented with right upper quadrant (RUQ) abdominal pain, jaundice, and scleral icterus for one week. On exam, the patient was afebrile with mild RUQ abdominal tenderness. Labs showed a total bilirubin of 5.4 mg/dL, AST of 66 U/L, ALT of 228 U/L, and a total alkaline phosphatase of 159 U/L. Ultrasound demonstrated intra- and extrahepatic biliary dilation with suggestion of a shadowing stone within the common bile duct (CBD). The gallbladder wall was thickened with trace pericholecystic fluid. MRCP showed a 15 mm calculus presumed to be in the CBD, with a 16 mm CBD dilation. ERCP showed a filling defect in the midportion of the CBD, and a CBD stent was placed. Due to these findings, a CA 19-9 antigen was drawn, measuring 3038 U/mL. EUS was then performed, which visualized a cystic duct stone partially extending into the CBD– a finding consistent with MS. An enlarged lymph node was visualized in the porta hepatis region. FNA of the lymph node as well as CBD brushings from the ERCP were both negative for malignant cells. The patient was discharged and underwent a cholecystectomy two weeks later. Surgical findings were consistent with MS and a 20 mm stone was removed with the gallbladder. The intraoperative frozen section of the gallbladder was negative for malignant cells. (Figure) Discussion: Initial evaluation for MS should include an ultrasound or CT scan, followed by MRCP. The pooled sensitivity of MRCP for detecting CBD stones is around 87%. While MRCP is often used to diagnose MS, the anatomy may not be well visualized due to the proximity of the cystic duct to the CBD. EUS has a pooled sensitivity of 97% in detecting a CBD stone and can better visualize the biliary tract anatomy, aiding in diagnosis. Diagnosis of MS prior to surgery can be helpful in avoiding biliary tract injuries. MS evaluation should also include screening for malignancy, as MS has an association with gallbladder cancer. While elevated CA 19-9 levels are concerning for malignancy, they can also be seen in chronic benign biliary obstruction. It is important to obtain a CA 19-9 level when suspecting MS, both before and after stone removal.Figure 1.: A. MRI: A T2-weighted image showing a 15 mm filling defect (white arrow) within the cystic duct, partially extending into the common bile duct, consistent with an obstructing stone. Intrahepatic and extrahepatic biliary dilation is also seen. B. Endoscopic ultrasound: An ovoid, hyperechoic structure, measuring 11 mm x 15 mm, with shadowing suggestive of a gallstone at the level of the cystic duct and compression of the common bile duct. C. Endoscopic ultrasound: An irregularly contracted gallbladder (black arrow) with adjacent dilated common bile duct. D. Endoscopic ultrasound: Fine needle aspiration of an enlarged lymph node with well defined margins in the porta hepatis region, measuring 20 mm x 15 mm in maximal cross-sectional diameter. Color Doppler imaging was utilized prior to needle puncture to confirm a lack of significant vascular structures within the needle path.

S1752 Infiltration of the Biliary Tract With Cytomegalovirus

Introduction: Cytomegalovirus (CMV) is known to affect immunosuppressed patients. CMV causes infections in post-transplant and HIV/AIDS patients but also those with advanced age or hematologic malignancies. This case presents CMV infiltration of the biliary tract in an elderly patient with 2 hematologic malignancies. Case Description/Methods: A 74-year-old male with tobacco use and chronic alcohol abuse was undergoing evaluation for anemia and thrombocytopenia. EGD and colonoscopy were unremarkable. Bone marrow biopsy revealed myelodysplastic syndrome (MDS) and B cell lymphoma. He subsequently developed jaundice. Labs revealed a total bilirubin of 10.4, ALP 1077, AST 107 and ALT 131. Viral hepatitis panel and HIV were negative. MRCP showed a dilated CBD and a filling defect within the CHD extending to the left hepatic duct with contour irregularity and narrowing in the left hepatic duct giving a beaded appearance. ERCP demonstrated a dilated CBD and CHD in addition to mild left and right hepatic duct dilatation with no definitive strictures or filling defects. A plastic bile duct stent was placed due to poor drainage of contrast. His LFTs continued to remain elevated. Liver biopsy revealed moderate cholestasis, sinusoidal dilatation, mild portal inflammation, biliary metaplasia, and stage 2 fibrosis. Repeat MRCP showed worsening intrahepatic and extrahepatic biliary dilatation with no further filling defect. ERCP with cholangioscopy was then performed. Bile duct mucosa had diffuse superficial granular like appearance in the common duct and main hepatic branches, which did not resemble typical previous biliary stent inflammatory changes. Biopsies of the bile duct were obtained. A fully covered metal stent was placed for further biliary drainage due to persistent poor contrast drainage. Bile duct pathology showed chronic active inflammation with CMV viral inclusions in the hepatic hilum and left hepatic takeoff. CMV PCR from the biliary specimen was positive. Blood CMV IgG was positive. Blood CMV PCR and CMV IgM were negative. His liver enzymes showed improvement after a fully covered stent was placed. He was started on a 2 week course of valganciclovir, with serial blood CMV PCR monitoring while on Rituximab for his B cell lymphoma. Discussion: This patient’s immunosuppression secondary to MDS, B cell lymphoma and advanced age increased his risk of a CMV infection with bile duct involvement. Cholangioscopy can aid in viewing subtle bile duct irregularities with biopsies to assess for CMV involvement.

S2814 Diverting Disaster: EUS-Guided Trans-Gastric Biliary Drainage

Introduction: Lumen-apposing metal stents (LAMS) are a relatively new intervention that can be used for high risk surgical cases, such as malignant obstructions not amenable to ERCP. There are few case reports using LAMS with Endoscopic Ultrasound (EUS) to achieve drainage and palliative therapy in these situations, especially using a gastric over duodenal approach. In this case, we add to the growing body of literature supporting a transgastric approach for biliary drainage using LAMS. Case Description/Methods: A 73 year-old male was admitted due to concerns for obstructive jaundice. He presented with pruritis, nausea, vomiting, copious nasogastric tube bilious output and a total bilirubin of 19.8mg/dL. MRCP revealed a distal CBD stricture measuring 12 mm in length, marked intrahepatic and extrahepatic biliary dilatation and an 8mm dilated pancreatic duct. EGD revealed a submucosal mass within the duodenal bulb causing gallbladder impingement (a). EUS was then completed and revealed similar findings to MRCP including an atrophic pancreas and severely dilated gallbladder (b). The ampullary mass, noted on CT, was also identified under EUS and fine needle biopsy was performed. A 10mm x 10mm LAMS was placed under endoscopic and fluoroscopic visualization within the flanges in the gallbladder and the antrum of the stomach (c) and resolved the patient’s symptoms the next day. Surgical pathology demonstrated pancreatic ductal carcinoma of the major and minor papillae, pancreatic head and uncinate process. Discussion: Interventions for obstructive jaundice include ERCP with biliary stenting, percutaneous transhepatic biliary drainage, EUS with LAMS placement, as well as surgery. While ERCP is the first line option, the patient’s distorted ampullary anatomy would have made cannulation and stenting challenging and high risk. Percutaneous drainage would expose the patient to risk of dislodging, obstructing, leaking, and infection. Seen below, LAMS has a relatively large diameter which allows for immediate biliary drainage, proven by the patient’s rapid relief of symptoms. Since a duodenal approach is more commonly used, current literature displays only a small number of cases showcasing the technical success of gastric approach for CBD access via LAMS, for instance, in the setting of duodenal stenosis. Our case demonstrates the versatility of the Axios stent with another successful alternative approach and proves a transgastric approach with LAMS is a viable alternative to surgery for malignant obstructive jaundice.Figure 1.: Image a: Dilated minor and major papillae with evidence of duodenal obstruction. b: EUS view of the dilated gallbladder. c: Biliary drainage from gallbladder after LAMS placement.

S1780 Immunoglobulin G4-Associated Cholangitis Mimicking Cholangiocarcinoma in Elderly

Introduction: Sclerosing cholangitis is a diffuse inflammation and fibrosis that progressively leads to stenosis and destruction of the bile ducts. It includes 3 types: primary sclerosing cholangitis (PSC), secondary cholangitis, and IgG4 sclerosing cholangitis (IgG4-SC). Usually they all present with cholestatic symptoms like pruritus and abdominal pain along with elevated ALP and GGT as well as bilirubin, however IgG4-SC has better outcomes, treatment response to steroids and less recurrence. We hereby presenting a case of IgG4-SC presenting as cholangiocarcinoma. Case Description/Methods: An 80 year-old male with a past medical history of bladder cancer was referred to the gastroenterology for EUS after blood work showed (ALT 320, AST 297, Bilirubin 1.4, GGT 2032), and CT abdomen with IV contrast revealed intra and extrahepatic biliary dilatation with an abrupt cutoff in mid CBD, suspicious for either a primary CBD mass or pancreatic mass (Figure). EUS showed an irregular mass in the CBD wall, suspicious for cholangiocarcinoma (Figure). The ERCP showed malignant appearing stricture of the extrahepatic bile duct consistent with cholangiocarcinoma, Bismuth 2 vs early 3B (Figure). Exploratory laparotomy was done and portal node biopsy came back benign; for which a repeat ERCP/Spyglass with direct visualization cholangioscopy, was done and showed diffusely dilated main bile duct with sludge and pus, single diffuse stenosis in right hepatic duct sludge with lining malignant appearing abnormal mucosa (nodularity and dilated vessels). IgG4 was elevated (931) consistent with IgG4 cholangitis (Figure). Biopsy and cytology brush showed no signs of dysplasia or neoplasia. Patient was then started on prednisone for a month and scheduled to repeat ERCP/Spyglass after. Discussion: IgG4-RD is a fibrous-inflammatory process related to immunomodulation, therefore IgG4-SC often co-presents with auto-immune pancreatitis, dacryoadenitis and sialadenitis. IgG4-SC is extremely challenging, as it’s frequently misdiagnosed on imaging as a pseudotumor, and can progress to liver cirrhosis if left untreated, however it comes with great response to steroids. In 2021, Mendoza criteria (tortuous vessels, irregular nodulations, raised intraductal lesions, irregular surface, and friability) was published and found to be accurate to differentiate malignant and benign causes of biliary stricture, with overall diagnostic accuracy of 77%. Our case didn’t meet the 5 criterias of Mendoza, which is consistent with IgG4-SC.Figure 1.: Top: CT of abdomen and ERCP. Bottom: Exploratory laparotomy.

S1860 Diffuse Large B Cell Lymphoma Presenting as Acute Recurrent Pancreatitis

Introduction: Extra-nodal Non-Hodgkin’s lymphomas are mostly made of gastrointestinal sites1. Primary pancreatic lymphomas account for < 1% of all extra-nodal lymphomas and are extremely rare. Diagnosis is often missed or delayed due to the prevalence of chronic pancreatitis or pancreatic adenocarcinoma as thought to be the main causes of any pancreatic stricture or mass seen when diagnosing a patient. We present a case of pancreatic lymphoma presenting as acute recurrent pancreatitis. Case Description/Methods: A 51-year-old male with past medical history of rheumatoid arthritis and nicotine use presented with jaundice, abdominal pain, and fatigue and 30-pound weight loss. MRCP showed intra- and extra-hepatic biliary dilation, showing double duct sign. CT suggested acute pancreatitis. ERCP was done showing a single localized biliary malignant appearing stricture in the lower third of the main bile duct with upstream dilation. EUS showed a 19 mm by 19 mm mass in the head of pancreas and 8 mm dilation of the common bile duct. EUS fine needle biopsy showed chronic pancreatitis with dense fibrosis and benign peri-pancreatic lymph nodes. PET CT showed activity in the pancreas and retroperitoneal lymph nodes. Tumor markers (AFP, CEA, CA 19-9) were within normal limits. (Figure) Patient had no known risk factors for chronic pancreatitis therefore, the acute presentation and biopsy results in congruent with the clinical history led us to repeat EUS one month later. This showed a 20 mm by 15 mm irregular mass in the head of the pancreas with upstream pancreatic duct dilation. This time, pathology was sent for flow cytometry and came back positive for lymphoid infiltrate with a preponderance of B-cells surrounded by dense fibrosis and was positive for CD 19, 20, 22 and 10. By this point, malignancy other than adenocarcinoma was suspected and IR guided biopsy of retroperitoneal lymph nodes confirmed a diagnosis of diffuse large B cell lymphoma. Patient started chemotherapy with a 70% chance of being cured. ERCP one month later showed the stricture had resolved and the stent was removed. Discussion: Keeping a broad differential in mind for pancreatic masses is essential to prevent delayed diagnoses. With that in mind, although rare, pancreatic lymphoma should always be in the differential and physicians should consider sending for flow cytometry to avoid missing this diagnosis. When patients present with recurrent pancreatitis and no risk factors, lymphoma should be kept in the differential.Figure 1.: a) CT showing inflammatory reaction of the pancreas with bile duct dilation; b) Bile duct stricture on ERCP; c) EUS image showing common bile duct stent.

S1818 Rare Sequelae of Obstructive Jaundice: Hyperferritinemia and Bile Cast Nephropathy

Introduction: Obstructive jaundice occurs due to a physical blockage in the biliary outflow tract or external compression. While jaundice and scleral icterus are common symptoms initially, long term sequelae include severe systemic manifestations from the buildup of various compounds normally excreted in bile such as bilirubin. Case Description/Methods: A 67-year-old male smoker presented with 3 weeks of pruritus, acholic stools, dark urine, nausea, vomiting, and 20-pound weight loss. Physical exam was notable for jaundice and scleral icterus, LUQ and epigastric tenderness to palpation, but lacked hepatosplenomegaly, ascites or other stigmata of cirrhosis. Liver function tests showed AST 486 IU/L, Alk Phos 1419 IU/L, Alt 600 IU/L, and total bilirubin >30.0 mg/dL with direct bilirubin >10.0 mg/dL. Ferritin was >7500 ng/mL with decreased transferrin of 159 mcg/dL. Hepatitis panel and malignancy/autoimmune markers were negative. CMP showed BUN 74 mg/dL, and creatinine 5.1 mg/dL with 2+bilirubin on urinalysis and microscopic analysis showing pigmented renal tubular cells, bilirubin crystals, and waxy casts suggestive of ATN due to bilirubin damage and possible CKD given waxy casts (Table). CT abdomen reported severe intra and extra hepatic biliary duct dilation with CBD of 17 mm and abrupt tapering with pancreatic head fullness confirmed with MRCP. On day 2, ERCP showed a tight 15mm distal biliary stricture. A small sphincterotomy followed by balloon dilation allowed for placement of a CBD stent with excellent drainage of dark green bile. Following ERCP, patient had a drop of liver function tests and ferritin levels, gradual improvement in BUN and creatinine, and was discharged in stable condition on hospital day 6. On day 16, EUS revealed a hypoechoic area within the periampullary area with atypical cells in brushings collected during repeat ERCP (Figure). Discussion: Iron and iron binding proteins have been shown to be excreted in bile, primarily in iron excess. Our patient showed a large increase in ferritin signifying the potential role of biliary excretion of excess ferritin, with unknown ramifications. Additionally, severe biliary tree obstruction led to markedly elevated bilirubin, overwhelming renal clearance leading to severe tubular damage. With alleviation of obstruction, there was a steady decrease in ferritin and return to normal baseline renal function. Further research is needed to clarify both the consequences of excess ferritin in biliary obstruction and the significance of excess bilirubin in renal injury.Figure 1.: a) Ampulla prior to ERCP on day 2 shows no periampullary abnormality. b) Day 16 - Endoscopic Ultrasound showing narrowing of distal common bile duct with hypoechoic intraductal area. c) Bile casts and bilirubin crystals seen on microscopy. Table 1. - Patient’s Laboratory test results on and post admission Laboratory Values Reference Range Day 1 Day 5 (3 days post-ERCP) Day 21 AST 13 – 39 IU/L 486 187 18 ALT 7 – 52 IU/L 600 315 27 Alkaline Phosphatase 34 – 104 IU/L 1419 886 160 Total Bilirubin 0.3 - 1.2 mg/dL >30.0 13.0 4.4 Albumin 3.5 - 5.2 g/ 3.0 2.9 3.4 BUN 7 – 25 mg/dL 74 64 18 Creatinine 0.5 - 1.2 mg/dL 5.1 2.90 1.01 WBC 4 – 11 K/cmm 13.8 17.7 10.1 Hgb 13.7 - 17.5 g/dL 11.9 9.4 8.5 Ferritin 24 – 336 >7500 4677 976 Transferrin 300 to 360 mcg/dL 159 233 Iron 50 - 182 ug/dL 126 61 Iron Saturation 57% 19% TIBC 250 – 450 ug/dl 233 326 Urine Bilirubin Negative 2+ Negative Urobilinogen 0.0 - 2.0 EU/dL 4.0 < 2.0 PT 9.1 - 13.2 sec 26.6 12.8 PTT 23.3 - 36.6 SEC 42.0 33.0 INR 0.8 - 1.2 2.3 1.1 Alpha-1-Antitrypsin (ATT) 83 – 199 mg/dL 256 Ceruloplasmin 18– 36 mg/dL 51 Carcinoembryonic Antigen (CEA) 0.0 - 3.0 ng/mL 3.9

S367 Endoscopic Eversion and Stent-Assisted Strangulation for the Management of an Intraductal Papillary Neoplasm of the Bile Duct: A Minimally Invasive Technique for Non-Surgical Candidates

Introduction: Intraductal papillary neoplasms of the bile duct (IPNB) are uncommon lesions that may accompany risk for malignancy. Management is often surgical and may be associated with significant postoperative morbidity and mortality. Case Description/Methods: An 80-year-old female with a history of hepatocellular carcinoma (HCC) presented for management of jaundice and sepsis after microwave ablation of HCC. Computed tomography (CT) imaging demonstrated intra and extrahepatic biliary ductal dilation with a 19 mm soft tissue density in the distal common bile duct (CBD). Index labs depicted leukocytosis, hyperbilirubinemia, and elevated lactate. Endoscopic ultrasound (EUS) delineated a round, hypoechoic intraductal CBD lesion. The pancreas and ampulla were normal. Endoscopic retrograde cholangiopancreatography (ERCP) confirmed a distal CBD filling defect. Biliary sphincterotomy was performed and balloon sweeping revealed a frond-like partially protruding polypoid lesion suggestive of an IPNB. Due to non-surgical candidacy, we decided to perform a complete eversion with subsequent full prolapse of the lesion by using a biliary extraction balloon. This was followed by the placement of a biliary plastic stent to assist with strangulation of the base of the lesion. Biopsies were consistent with a papillary neoplasm with intestinal differentiation, confirming our findings of an IPNB, without accompanying high-grade dysplasia or carcinoma. Interval surveillance ERCP 3 months later revealed no protruding lesion from the major papilla, no residual filling defects on cholangiogram, and sweeping of the biliary tree uncovered no contents. Cholangioscopy demonstrated segmental mucosal irregularity with mild granular tissue in the mid/distal bile duct wall; however, cholangioscopy-guided intraductal biopsies and brushings showed benign reactive tissue and no recurrent IPNB. At 5 months of follow-up, the patient continues to do well. Discussion: IPNB is a rare biliary lesion. Radical surgical resection has been the mainstay of curative treatment. However, a few recent cases have described endoluminal radiofrequency ablation as an alternative therapy. To the best of our knowledge, this is the first reported case of balloon-assisted eversion combined with stent-assisted strangulation of an IPNB leading to spontaneous removal. In non-surgical candidates, this technique may be advantageous to minimize adverse operative outcomes, improve patient tolerance, and decrease cost. Watch the video at https://tinyurl.com/ACGAbstractS367

S3531 A Rare Cause of Obstructive Jaundice: Afferent Limb Syndrome

Introduction: Afferent limb syndrome (ALS) is a rare but potentially life-threatening complication of upper gastrointestinal (GI) surgery and reconstruction causing distal obstruction of the biliopancreatic limb with subsequent accumulation of bile, pancreatic fluid, and other small bowel secretions upstream afferent limb dilation. It is imperative to recognize this complication postoperatively as buildup in the small bowel can result in bowel perforation requiring emergency surgery. We present a patient who developed ALS after upper gastrointestinal reconstruction with esophagojejunostomy for the treatment of gastric adenocarcinoma. Case Description/Methods: An 84 year-old female with a past medical history of gastric adenocarcinoma with total gastrectomy and esophagojejunostomy presented to the emergency department with a subacute history of upper abdominal pain and new-onset jaundice. Initial labs were notable for elevated liver chemistries including alkaline phosphate level of 435 U/L (normal 39-117 U/L), alanine transaminase of 62 U/L (normal 6-45 U/L), and aspartate transaminase of 36 U/L (normal 10-25 U/L). Abdominal ultrasound demonstrated markedly dilated common bile and intrahepatic ducts and gallbladder distention without stones. CT scan of the abdomen confirmed the ultrasound findings, but also identified a dilated duodenum with an abrupt transition point in the proximal jejunum suggestive of ALS shown in Figure A. The patient underwent open laparotomy which demonstrated multiple tumor implants. A large implant at the site of the previous jejunojejunostomy was identified as the cause of afferent limb obstruction. A palliative bypass was performed. The patient had a complicated post-operative course. Ultimately, due to the extensive disease burden and ongoing patient discomfort, the patient and their family decided to pursue hospice care. Discussion: ALS is a post-operative complication of upper GI tract surgery that involves mechanical obstruction of the biliopancreatic limb. Patients often present with jaundice. CT scan is the preferred diagnostic modality. First-line treatment involves surgery to relieve the obstruction with revision of the prior anastomosis. Although rare, this diagnosis should be considered in appropriate patients.Figure 1.: A: CT scan with markedly dilated intrahepatic and extrahepatic biliary ductal dilation, distended gallbladder, and markedly dilated duodenum with abrupt transition point in proximal jejunum.

S3439 Lemmel Syndrome Due to Periampullary Diverticular Food Impaction

Introduction: Lemmel Syndrome is a rare cause of mechanical biliary obstruction attributed to periampullary duodenal diverticula (PAD). It was first described in 1934 as obstructive jaundice in the absence of biliary stones or malignancy. The majority of PAD are asymptomatic, however, about 5% have symptomatic disease. We present a symptomatic case. Case Description/Methods: An 80-year-old woman with history of cholecystectomy, coronary artery disease, hypertension, and diabetes was transferred from a free-standing emergency room for acute pancreatitis. She had a one day history of postprandial epigastric pain with radiation to the back. Labs on presentation included lipase above 4000 U/L, BUN 27 mg/dL, AST 316 U/L, ALT 422 U/L, ALP 292 IU/L, total bilirubin 0.6 mg/dL. Abdominal ultrasound revealed 12mm common bile duct (CBD) with intrahepatic ductal dilatation. MRCP showed intra and extrahepatic biliary ductal dilatation with dilated pancreatic duct and a 3.6 cm fluid filled periampullary diverticulum. ERCP showed a large periampullary duodenal diverticulum with food impaction causing obstruction of the ampullary orifice which was removed with forceps. Cholangiogram revealed dilation of pancreatic, common bile, and intrahepatic ducts without any filling defects. Patient improved and was discharged home. (Figure) Discussion: The incidence of duodenal diverticula is approximately 17% with PAD being the most common type. PADs are pseudo-diverticula most commonly found in the second part of the duodenum and they pose a cannulation challenge during ERCP with an increased risk of complications. PAD in the setting of biliary obstruction is known as Lemmel Syndrome. It can cause biliary obstruction by sphincter of Oddi dysfunction, direct obstruction, or via external compression by fluid or material-filled PAD. In our case, there was biliary compression due to a food-filled PAD. Cross sectional imaging is critical for diagnosis, however, ERCP is the gold standard. Several therapeutic modalities including endoscopic extraction of entrapped food or stones, extracorporeal shock wave lithotripsy, and laparoscopic diverticulectomy have been used. Our case required endoscopic extraction of diverticular food debris and adds to the limited published cases to date. Lemmel Syndrome requires a high index of suspicion when a PAD is found in the setting of biliary obstruction.Figure 1.: A. MRCP image showing a large periampullary diverticulum packed with debris. B. ERCP image of food impacted inside periampullary diverticulum. C. MRCP image demonstrating a dilated common bile duct in absence of choledocholithiasis.

S1741 Gelatinous Ascites and a Double Duct Sign: A Case of Pancreatic Adenocarcinoma

Introduction: Pseudomyxoma peritonei (PMP) is a rare disorder characterized by the accumulation of gelatinous material within the abdomen and pelvis. While usually appendiceal in origin, it can be seen with mucinous tumors arising from the ovary, GI tract, urachus, and pancreas. We describe a case of pancreatic adenocarcinoma diagnosed due to the clinical syndrome precipitated by pseudomyxoma peritonei. Case Description/Methods: A 59-year-old Hispanic male presented to the hospital for evaluation due to a 2 month history of poor oral intake and worsening abdominal fullness, distension, discomfort, and intermittent pain. The patient reported one prior hospitalization for pancreatitis of unclear etiology, 3 years ago. A CT abdomen & pelvis was obtained; findings reported include multiple hepatic subcapsular cystic liver lesions, abdominal omental caking, ascites, and extrahepatic biliary and pancreatic ductal dilatation without definitive evidence of a mass. CEA and CA 19-9 were found to be elevated at 49.4 and 416 respectively. CT chest revealed a left-sided pleural effusion. Upper endoscopy and colonoscopy were negative. Paracentesis yielded gelatinous ascites. Clinical suspicion for appendiceal malignancy was raised which required surgical exploration and biopsy to confirm. An MRI of the abdomen was obtained to assess for pancreatic mass given presence of double duct sign. MRI revealed 2.2 x 2.7 x 5 cm lesion in the body of the pancreas, pseudomyxoma peritonei with scalloping of the liver and extension into the right posterior mediastinum via the esophageal hiatus, metastatic deposits in the porta hepatis and peritoneal carcinomatosis. No apparent abnormality in the appendix was observed. EUS FNA was performed; splenic vessel invasion by the mass was noted. Cytopathology supported a diagnosis of pancreatic adenocarcinoma. The patient was initiated on palliative chemotherapy with gemcitabine and nab-paclitaxel which led to a significant reduction in ascites and pleural effusion and improvement in symptoms. Discussion: This case highlights the potential of malignancies such as pancreatic adenocarcinoma to cause pseudomyxoma peritonei despite it more commonly being associated with appendiceal origin. Knowledge of this can help guide diagnostic and therapeutic plans.