Extrahepatic Bile Duct Dilatation Research Articles

Risk factors for post-endoscopic retrograde cholangiopancreatography (ERCP) abdominal pain in patients without post-ERCP pancreatitis

BackgroundAbdominal pain is often observed after endoscopic retrograde cholangiopancreatography (ERCP). Few studies have focused on the risk factors of post-ERCP abdominal pain without post-ERCP pancreatitis (PEP). This study aimed to identify risk factors of post-ERCP abdominal pain without PEP and investigate characteristics of the abdominal pain in non-PEP patients. MethodsData from patients who underwent ERCP from August 2019 to January 2020 were retrospectively collected. Characteristics of the abdominal pain after ERCP were recorded and compared between PEP and non-PEP patients. Multivariate analysis was conducted to identify risk factors of non-PEP abdominal pain. ResultsA total of 1295 ERCP procedures were investigated in this study, among which 100 (7.72%) patients presented post-ERCP abdominal pain without PEP and 63 (4.86%) patients with PEP. Multivariate analysis found 9 risk factors of non-PEP abdominal pain: age ≤ 65 years [odds ratio (OR): 1.971], primary ERCP (OR: 2.442), dilated extrahepatic bile duct (OR: 1.803), no papilla opening (OR: 2.095), pancreatic guidewire passages (OR: 2.258), white blood cells (WBC) ≤ 6.0 × 109/L (OR: 1.689), platelet (PLT) ≤ 250 × 109/L (OR: 2.505), serum γ-glutamyl transferase (γ − GT) ≤ 35 U/L (OR: 2.190), and albumin ≥ 40 g/L (OR: 1.762). The PEP group had later pain onset, higher pain frequency and longer hospital stay than those of the non-PEP pain group (P < 0.05). There were no significant differences in the pain duration, visual analogue scale score and mortality between the PEP group and non-PEP pain group (P > 0.05). ConclusionsThis study indicated that age ≤ 65 years, primary ERCP, dilated extrahepatic bile duct, no papilla opening, pancreatic guidewire passages, lower WBC, lower PLT, normal γ − GT and elevated albumin were independent risk factors for post-ERCP abdominal pain without PEP. The pain occurred earlier in non-PEP patients than in PEP patients.

Focal IgG4-related autoimmune pancreatitis with distal choledochal adenocarcinoma: a rare case report

BackgroundAutoimmune pancreatitis (AIP) is a rare disease that manifests as pancreatic involvement in systemic IgG4-related disease (IgG4-RD), a special type of chronic pancreatitis caused by autoimmune abnormalities. The main imaging manifestations of IgG4-related AIP consist of diffuse or localized pancreatic enlargement and irregular pancreatic duct narrowing. The diagnosis of AIP is challenging because it can present with focal lesions, similar to radiologically bile duct cancer or pancreatic cancer.Case presentationA 55-year-old male patient was admitted with painless jaundice and multiple radiographic findings of pancreatic head mass, as well as intrahepatic and extrahepatic bile duct dilatation. Various imaging methods indicated pancreatic cancer. However, the endoscopic ultrasonography guided fine needle aspiration (EUS-FNA) and a laparoscopic pancreatic biopsy suggested an IgG4-related AIP. After one month, magnetic resonance imaging showed that the lesion had slightly grown. Combined with CA19-9 and other indexes, the possibility of malignancy was high and there were still surgical indications. The pathological analysis following a pancreaticoduodenectomy revealed poorly differentiated adenocarcinoma in the distal common bile duct.ConclusionTo date, few reports have described pancreatic or extrapancreatic malignancies in AIP patients, and no association between AIP and bile duct adenocarcinoma has been previously confirmed. This case discuss the differentiation between AIP and malignancy, recent research progress, and the correlation between the two diseases, highlights the importance of carefully evaluating patients with AIP to rule out potential tumors, as well as the critical need for follow up treatment.

A205 PEMBROLIZUMAB ASSOCIATED SCLEROSING CHOLANGITIS RESPONDS TO COMBINATION OF STEROIDS AND MYCOPHENOLIC ACID: A CASE REPORT

Abstract Background Pembrolizumab is a check point inhibitor that targets programmed cell death receptor ligand 1 (PD-L1). However, check-point inhibitors are associated with its characteristic adverse event know as immune related adverse events that can affect a myriad of organ systems. In the hepatobiliary system, the most commonly described iRAE is hepatitis/hepatotoxicity and latest oncology guidelines offer recommendations on treatment and followup of immunotherapy related hepatitis (1). However, a more rare presentation of iRAE, that have only been described as case reports or case series, is secondary cholangitis. We report one of such a case. Aims Case report Methods Case report Results The patient is a 55 year-old Caucasian female know for lung adenocarcinoma presented to the emergency room on May 5th, 2020, having received her 8th cycle of pembrolizumab. Her blood works mildly elevated transaminitis; ALT 78 U/L, AST 11 U/L, alkaline phosphate of 130 U/L, and total bilirubin of 3.5 umol/L. MRI of the abdomen showed a normal appearing liver without focal parenchymal lesion, no biliary stones or obstructing masses, dilated common bile duct at 11 mm with small pericholecystic free fluid, and normal gall badder thickness of 2 mm. There was also notion of minimally prominent intrahepatic biliary radicles seen within the CBD. Etiology workup of cholestatic transaminitis was negative. A diagnosis of immunotherapy related sclerosing cholangitis was posed and the patient was treated with MMF and prednisone with good response. Conclusions Discussion This patient seems to have developed immunotherapy mediated cholangitis. Liver biopsy when performed in liver injury tend to show lobular hepatitis, with liver parenchyma infiltrated with lymphocytes and focal necrosis with acidophilic bodies. However, there seems to be another, less frequent pattern of injury described with pembrolizumab and associated with cholangiopathy, resembling primary biliary cholangitis. It is characterized with portal tracts enlarged with fibrosis and inflammatory cells infiltration; infiltrating cells are lymphocytes (2). Biliary epithelium with fibrosis with CD8+ T cells infiltration seem to be recurrently reported in case reports. (3, 4). Other cases have been describe in the literature. In many cases, steroid therapy was attempted, but response rates seem to be inconsistent. Nivolumab related cholangitis have been also been described and characterized by extra hepatic bile duct dilatation along with negative immunological markers such as ANA and IgG4 in patients with non-small cell lung cancer, and seems to respond only moderately to steroid therapy (6). Retrospective data of nivolumab related cholangiopathy seems to suggest that there is favourable response to the combination of MMF and steroid therapy (7). Funding Agencies None

Irreversible pancreatic electroporation, first institutional experience, case report and literature review

Presenter: Victor Manuel Correa Santillan MD | Instituto Jalisciense de Cancerología / Hospital Civil de Guadalajara Background: Pancreatic cancer is one of the pathologies that determine a poor prognosis, since it presents advanced stages at the time of diagnosis, with an overall 5-year survival < 5%. It is estimated that there was 495,773 new cases worldwide and many of these patients had ECOG 0-2 at the time of diagnosis.(1) Efforts have been made to develop new treatments to improve the care of patients with locally advanced pancreatic cancer, as a result of this, we have novel alternatives such as irreversible electroporation, which consists of inserting special needles into the tumor; Short electrical pulses of a high voltage current are then passed through the needles. The goal is to destroy cancer cells.(2) Methods: Describe the results of treatment of a patient with locally advanced pancreatic cancer using irreversible transurgical electroporation. In our institution for the first time. Results: A 58-year-old male presented as an outpatient with a 6-week history of progressive jaundice and weight loss of more than 8 kg. His laboratory test was CA 19-9 of 615U/ml, total bilirubin 19mg/dl, direct bilirubin 11.18 mg/dl, alkaline phosphatase 481U/L, gamma-glutamyl transferase of 849U/L. The cholangioresonance showed intra- and extrahepatic bile duct dilation with a 2cm mass in the head of the pancreas, with infiltration of the portal vein. The ERCP confirms the finding of a pancreatic head tumor, brush cytology is done, and drainage of the bile duct is achieved with a 16 Fr biliary stent. The cytology analysis shows inflammatory changes with atypia. Induction chemotherapy is started with six cycles of FOLFIRINOX; 8 weeks later, a Whipple procedure is attempted, during surgery infiltration of more than 180 degrees of the vena cava is seen; therefore, the surgery is aborted. Four weeks later, irreversible pancreatic electroporation is scheduled with three needles, two cycles, and two fields of trans-surgical depth, which was completed without complications. After the procedure, the chemotherapy continued with FOLFOX for four cycles and changed to XELOX / CAPEOX for six cycles due to gastrointestinal intolerance. Four months after chemotherapy completion, a PET-CT scan showed retroperitoneal lymph node activity without evidence of the pancreatic tumor; due to these findings, thirty sessions of Intensity-Modulated Radiotherapy was started. Conclusion: The evidence supporting the use of this technology reports a benefit in overall survival from 28 months to 85 months and overall progression-free periods up to 12 months, with a solid base of safety reporting a mortality rate of 2% to 3% to 90 days. And this is reflected in stable ECOGs for a longer time. (2,3) Although in Mexico there is the possibility of using this type of technology, they are limited by their high cost, even so, The Instituto Jalisciense de Cancerología is a pioneer in the comprehensive management of locally advanced pancreatic cancer, positioning itself as a reference center in the country. Bibliography 1. International Agency for Research on Cancer. GLOBOCAN 2020 2. National institute for Hearth and Care Excellence, Irreversible electroporation for treating pancreatic cancer, Interventional procedures guidance, 3. Balaban E and Col, 2016 Locally Advanced, Unresectable Pancreatic Cancer

Etiological classification and treatment strategies for secondary bile duct dilatation.

Secondary intra- and extrahepatic bile duct dilatation is a very common condition that can be caused by several diseases. However, it has been rarely discussed in the specialized literature. Moreover, no distinct etiology can be determined in some cases, which hampers the diagnosis and treatment. Here, we discuss the etiological classification and treatment strategies of secondary intra- and extrahepatic bile duct dilatation based on an extensive literature review, as well as our experimental research and clinical experience. The etiology of secondary intra- and extrahepatic bile duct dilatation can be classified in different ways. From a clinicopathological perspective, it can be classified into obstruction-, lesion-, and compression-induced dilatation. Treatment varies depending on the cause. For example, endoscopic dilation or stenting is used for biliary strictures, laparoscopic choledochectomy for stone removal, and resection for cholangiocarcinoma.

Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma (MEITL) Presenting As An Obstructive Pancreaticoduodenal Mass: A Case Report

Abstract Casestudy: A 74-year-old woman of Scottish descent presented with obstructive jaundice and weight loss. Imaging revealed a head of pancreas mass with extrahepatic bile duct dilation. Fine needle aspiration cytology of the mass was non-diagnostic, therefore the patient underwent a pancreaticoduodenectomy. Gross examination revealed a circumferential, necrotic lesion involving the periampullary duodenum with invasion into the pancreas and peri-pancreatic soft tissue. Microscopically, the lesion was composed of diffuse sheets of monomorphic medium-sized cells with fine chromatin, inconspicuous nucleoli, and scant pale cytoplasm. The duodenal mucosa overlying the lesion was ulcerated. Epitheliotropism was noted in the duodenal villi, crypt epithelium and pancreatic duct epithelium. The tumor showed extensive areas of necrosis and conspicuous perineural invasion. The duodenal mucosa uninvolved by the tumor showed no evidence of an underlying enteropathy, such as Celiac disease. By immunohistochemistry, the neoplastic cells were positive for CD45, CD3, CD8, CD56, TIA-1, granzyme B, TCR-BF1, and TCR-gamma, and were negative pan B-cell markers, CD4, CD5, CD25, CD30, EBER, HHV-8, and TCL-1. Molecular studies for T cell clonality revealed clonal TCR-alpha-beta and gamma-delta gene rearrangements. Given the morphologic and immunophenotypic findings, the diagnosis of monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) was rendered. This case is unique with features not typical of MEITL, namely mass-like pancreaticoduodenal presentation resulting in a Whipple resection, extensive necrosis, and perineural invasion.

S1505 Choledochoduodenal Fistula: An Old Complication of Choledocholithiasis With a New Presentation

INTRODUCTION: Enterobiliary fistulas were first described in 1654 with 68% of cases occurring between the gallbladder wall and duodenum. Choledochoduodenal fistulas (CDF) are less common (8.6%) and most are asymptomatic. This case highlights a rare presentation of pneumobilia, cholangitis, and pyogenic hepatic abscesses in a patient with CDF. CASE DESCRIPTION/METHODS: A 68 year old male with a past medical history of diabetes presented with rigors and fevers for one day. He denied abdominal pain, history of gallbladder disease, or recent travel history. Vitals signs were notable for BP 70/40, HR 110, T 101 F. Physical exam was remarkable for scleral icterus, but no abdominal tenderness or altered mentation. Lab results revealed WBC 31,000, creatinine 3.94, total bilirubin 9.6, direct bilirubin 7.9, AST 117, ALT 94, ALP 484, lactate 7.4, and positive blood cultures for Escherichia coli. CT abdomen without contrast revealed marked intra and extrahepatic bile duct dilatation with multiple focal densities identified in the liver and pneumobilia at the level of the distal common bile duct (CBD) (Figure 1). Antibiotics were started and he underwent ERCP which revealed a suprapapillary CDF with significant stone burden and pus in the CBD. Sphincterotomy was performed to the level of the CDF. Many stones were removed and two plastic stents were placed to ensure drainage. Follow up liver MRI revealed numerous hepatic abscesses. The patient’s vitals and laboratory markers improved with antibiotics, and he was scheduled for follow up outpatient ERCP to complete endotherapy. DISCUSSION: CDF can be caused by choledocholithiasis, duodenal ulcers, tumor invasion, or post-cholecystectomy complications. Most cases of CDF are asymptomatic and incidentally detected. However in the subset of symptomatic cases, cholangitis is the most common presentation. Furthermore, chronic cholangitis due to reflux of duodenal contents with bacterial flora may cause hepatic abscesses, though this has only been documented in case reports. Diagnosis can be achieved by ERCP with direct visualization of CDF and successful cholangiography through the papilla and fistula orifice. With few studies investigating this entity, proposed treatment options are conflicting. Some advocate for less invasive measures, such as endoscopic interventions for asymptomatic patients or distal CDF. Others regard CDF as a biliary abnormality that predisposes to recurrent infections and increases the risk for malignancy, and thus advocate for surgical management.Figure 1.: This sagittal view of a noncontrast CT abdomen/pelvis delineates hepatic abscesses, pneumobilia at the level of the choledochoduodenal fistula, and dilated intra and extrahepatic bile ducts.

S1544 An Interesting Presentation of Cholangiocarcinoma: Esophageal Varices

INTRODUCTION: Esophageal varices are an uncommon presentation for cholangiocarcinoma. CASE DESCRIPTION/METHODS: A 75 year old female with a past medical history of anemia presented to her primary care physician with a complaint of fatigue for two months. The patient took NSAIDs regularly, but denied any melena, hematochezia or hematemesis. Lab work confirmed iron deficiency anemia, she was referred for upper endoscopy and colonoscopy. Findings were notable for grade I varices in the lower third of the esophagus and grade I internal hemorrhoids. Given the findings of varices, she was referred for a RUQ ultrasound with findings of cirrhosis without evidence of portal hypertension. The patient had no history of alcohol abuse or viral hepatitis, so these findings were concerning for a diagnosis of NASH. The patient was hospitalized after developing jaundice, abdominal distension and a 15-pound weight loss with associated clay colored stools and dark urine. At the time of admission, her total bilirubin was 22. MRI of the abdomen revealed intrahepatic and extrahepatic bile duct dilation with abrupt narrowing suspicious for stricture. Other findings included cirrhosis, esophageal varices, mild ascites, and mild splenomegaly. The patient underwent ERCP with findings of moderate dilation of the upper and middle third of the main bile duct, left and right hepatic ducts, all intrahepatic branches and the common hepatic duct. A biliary sphincterotomy was performed and 1 stent placed. Brushings of the common bile duct were notable for cholangiocarcinoma. A subsequent PET scan demonstrated mild avidity in the area of the CBD but no discrete lesion and no metastatic disease. DISCUSSION: Cancer arising from the perihilar region accounts for 60-70% of extrahepatic cholangiocarcinomas and are further classified according to the pattern of involvement of the hepatic ducts. Tumors involving the hepatic duct bifurcation, hilar cholangiocarcinomas, are collectively called Klatskin tumors. Patients with extrahepatic cholangiocarcinomas become symptomatic when there is obstruction of the biliary drainage system. Typical presenting symptoms include dark urine, jaundice, clay colored stools, weight loss, and abdominal pain. Small extrahepatic bile duct cancers may not be visualized on imaging, but an obstructing malignant lesion is suggested by ductal dilation in the absence of stones. Proximal lesions cause dilation of just the intrahepatic ducts, while distal lesions cause dilations of both the intrahepatic and extrahepatic ducts.Figure 1.: Grade I Esophageal Varices.Figure 2.: Esophageal varices in the lower third of the esophagus.Figure 3.: Common Bile Duct Dilation on MRI.

S1407 A Rare Case of Pancreaticobiliary IgG4-Related Disease and Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome)

INTRODUCTION: IgG4-Related Disease (IgG4-RD) is a rare but increasingly recognized condition. It can manifest as pancreatitis and sclerosing cholangitis. Eosinophilic granulomatosis with polyangiitis (EGPA) may increase IgG4 levels but is not associated with IgG4-RD. CASE DESCRIPTION/METHODS: A 42 year old white male with longstanding EGPA on chronic prednisone had progressive malaise, sinopulmonary symptoms, and brief bouts of abdominal pain. He was admitted for an EGPA flare and abnormal liver enzymes. His exam was benign except for RUQ tenderness. Work-up revealed AST 280 U/L, ALT 330 U/L, Alkaline Phosphatase 727 U/L, bilirubin 1.6 mg/dL, ESR 82 mm/hr, and 1220 eosinophils/microL. Autoimmune, viral hepatitis, and infectious workup was negative. Chest x-ray was normal. Ultrasound showed cholelithiasis without cholecystitis or biliary dilation. MRCP showed non-visualization of a 2 cm segment in the common bile duct (CBD), and a 2.1 cm pancreatic tail cyst, prompting EUS/ERCP. Initial EUS was notable for diffuse thickening of the cystic and common bile duct, and intra- and extrahepatic bile duct dilatation and stricturing with a dominant 2 cm CBD stricture without associated mass. Following sphincterotomy, the CBD was brushed and ampulla biopsied (both benign), and CBD and pancreatic duct stented. IgG4 returned 1658 mg/dL (17x normal), and liver enzymes only mildly improved. Follow-up EUS/ERCP showed the 2.2 cm pancreatic tail cyst adjacent to a 21 × 19 mm pancreatic tail mass. Cyst aspirate was consistent with a pseudocyst. Fine needle biopsies of the pancreatic mass showed lymphocytic invasion, fibrosis and sclerosis, consistent with IgG4-RD. Cholangioscopy with Spybite sampling of the CBD stricture showed non-specific inflammatory changes. A percutaneous liver biopsy noted only hepatic steatosis. His sinopulmonary symptoms remained uncontrolled and liver enzymes remained elevated. Rituximab infusions were initiated for both EGPA and IgG4-RD. He had symptom resolution and his liver associated enzymes returned to normal. DISCUSSION: This case demonstrates IgG4-RD with pancreaticobiliary disease in a patient on chronic steroids for EGPA. Bile duct, pancreatic, and liver biopsies all lacked features of EGPA. Pancreatitis and cholangitis are not associated with EGPA. Thus, it is likely the patient had two distinct and rare processes. His chronic steroid treatment for EGPA may have masked longstanding IgG4-RD. Rituximab was shown to be an effective second-line treatment modality for both conditions.Figure 1.: EUS - Significant common bile duct wall thickening at the level of the common bile duct stricture.Figure 2.: ERCP - Areas of dilatation and stricturing/narrowing involving the intra- and extrahepatic bile ducts consistent with cholangitis. A 2cm dominant stricture is noted in the common bile duct.Figure 3.: EUS - A 21 mm × 19 mm distal body/tail mass which underwent fine needle biopsy. Biopsies returned with features of lymphocytic invasion, fibrosis, and sclerosis consistent with autoimmune pancreatitis (IgG4-RD).

S1601 Malignant Melanoma of the Gallbladder Masquerading as Cholangitis and Cholecystitis

INTRODUCTION: Metastatic Malignant melanoma is reported in the stomach, intestine and colon. The involvement of the gallbladder and the bile duct are extremely rare. CASE DESCRIPTION/METHODS: A 61-year-old male presented with abdominal pain, nausea, vomiting, fever, chills, and 10lb weight loss. Physical examination was notable for right upper quadrant abdominal tenderness with a positive Murphy’s sign and minimal jaundice. Lab data showed no leukocytosis and normal hemoglobin. Elevated liver tests with Alk Phos 435U/L, ALT 546U/L, AST 474U/L, and Tbili 3.1mg/dl prompted an abdominal ultrasound which showed intrahepatic and extrahepatic bile duct dilatation (common bile duct (CBD) 11mm). A diffusely abnormal thick-walled gall bladder filled with debris was seen. This was concerning for cholecystitis and cholangitis. He was started on piperacillin/tazobactam. An endoscopic ultrasound (EUS) revealed hyperechoic material suggestive of thick sludge versus tissue within gallbladder lumen extending into the CBD (Figure 1a). Endoscopic retrograde cholangio-pancreatography (ERCP) revealed irregular filling defects within CBD close to cystic duct takeoff (Figure 1b). After sphincterotomy, balloon sweeps evacuated pigmented tissue debris (Figure 1c) which was retrieved and sent for pathology. Unusual EUS/ ERCP findings prompted an MRI which showed high T1 signal within periportal nodes and gallbladder suggesting hemorrhage. Surgical consultation was obtained and patient underwent laparoscopic cholecystectomy, but frozen section indicated malignancy, therefore, an open liver resection and lymphadenectomy was completed. The gross pathology showed a 2.5 × 1.6 × 1.5cm mass arising from the gallbladder (Figure 2a) with invasion into the peri muscular connective tissue on the hepatic side but with no direct extension. An immunostain on the tumor and the prior biliary tissue debris showed positive staining for Vimentin, and S100 and a diagnosis of melanoma was confirmed (Figures 2b and 2c). Surgical staging was pT2bN1. Skin and ocular examination did not reveal any lesions. DISCUSSION: Melanoma involving the gastrointestinal tract is usually metastatic from known cutaneous melanoma; however primary melanoma of the GI tract have been reported. Within the GI tract, primary gallbladder melanoma is extremely rare with less than 40 cases reported in the literature. Clinical course is typically aggressive; our patient developed metastatic disease to brain soon after gallbladder resection.Figure 1.: EUS and ERCP image.Figure 2.: Pathology image.

S1459 Immune Checkpoint Inhibitor-Induced Pancreatic Injury: An Atypical Presentation

INTRODUCTION: Immune checkpoint inhibitors (ICI) are effective in the treatment of several advanced cancer types. ICI-pancreatic injury (ICI-PI) is uncommon and often asymptomatic with incidentally elevated pancreatic enzymes. We present a case of ICI-PI mimicking autoimmune pancreatitis (AIP) with obstructive jaundice. CASE DESCRIPTION/METHODS: A 60-year-old male with acral melanoma metastatic to the liver was treated with four cycles of nivolumab and ipilimumab. After three cycles of maintenance nivolumab, surveillance positron emission tomography (PET) revealed diffuse fluorodeoxyglucose (FDG)-avidity of the pancreas with peripancreatic fat stranding (Image 1). He endorsed mild abdominal discomfort with an elevated lipase 10x the upper limit of normal (Table 1). He had no gallstones on imaging and socially drank alcohol. ANA was negative with normal IgG4. He was given a diagnosis of ICI-PI and treated with intravenous fluids and discharged on a 14-day steroid taper and lab monitoring. Three weeks after completing the steroid taper, he presented with worsening abdominal pain and gray colored stool. Labs showed elevated liver tests consistent with a cholestatic obstructive pattern. Computed tomography (CT) revealed diffuse mild enlargement of the pancreas. MRCP showed dilated extrahepatic bile duct up to 11mm with an intrapancreatic stricture. Endoscopic ultrasound (EUS) showed diffusely lobular pancreas with hypoechoic areas and intervening hyperechoic standing. The pancreatic duct was not dilated. The bile duct was dilated and abruptly narrowed as it entered the pancreatic head. Fine needle aspiration (FNA) of the pancreatic head with 25G needle revealed pancreatic acini and focal findings suggestive of chronic pancreatitis without metastatic melanoma (Image 2). A diagnosis of ICI-PI mimicking AIP was made. He received high dose oral steroids with a long taper. His liver enzymes normalized. Repeat PET showed interval resolution of pancreatic FDG-avidity and peripancreatic fat stranding with continued remission of melanoma. DISCUSSION: This is a unique case of ICI-PI evolving into obstructive jaundice and resolving with steroids. Although he had negative serologies, his imaging and clinical course mimicked AIP. Both ICI related adverse events and AIP are T-cell driven processes suggesting a possible similar pathogenesis. The incidence of ICI-PI will likely rise with the increasing use of ICIs; thus, awareness of ICI-PI and its variable presentation are clinically salient.Table 1.: Liver enzymes, total bilirubin and lipase from the last cycle of nivolumab therapy through both hospitalizations. Normal aspartate aminotransferase (AST) is 0–40 IU/L. Normal alanine aminotransferase (ALT) is 0-32 IU/L. Normal alkaline phosphatase (ALP) is 39-117 IU/L. Normal total bilirubin is 0–1.2 mg/dL. Upper limit of normal lipase is 51 U/LFigure 1.: Image 1. Positron emission tomography (PET) imaging. A. Prior to nivolumab therapy. B. After three cycles of maintenance nivolumab. C. After high dose oral steroids and normalization of liver enzymes.Figure 2.: Image 2. Biopsy obtained during endoscopic ultrasound (EUS). A. Benign pancreatic acini and focal findings suggestive of chronic pancreatitis on hematoxylin and eosin (H&E) stain. B. SOX10 stain with control. SOX10 is a melanoma marker and thus no metastatic melanoma identified.

Novel Percutaneous Image-guided Treatment of Choledocholithiasis in a 30-Day-old Infant.

Novel Percutaneous Image-guided Treatment of Choledocholithiasis in a 30-Day-old Infant.

Imaging of Pancreaticobiliary Maljunction.

Pancreaticobiliary maljunction (PBM) is a congenital malformation in which the pancreatic and bile ducts join outside the duodenal wall, usually forming a long common channel. A major issue in patients with PBM is the risk of biliary cancer. Because the sphincter of Oddi does not regulate the pancreaticobiliary junction in PBM, pancreatic juice frequently refluxes into the biliary tract and can cause various complications, including biliary cancer. Most cancers arise in the gallbladder or dilated common bile duct, suggesting that bile stasis is related to carcinogenesis. Early diagnosis and prophylactic surgery to reduce the risk of cancer are beneficial. The diagnosis of PBM is made mainly on the basis of imaging findings. The development of diagnostic imaging modalities such as multidetector CT and MR cholangiopancreatography has provided radiologists with an important role in diagnosis of PBM and its complications. Radiologists should be aware of PBM despite the fact that it is rare in non-Asian populations. In this review, the authors present an overview of PBM with emphasis on diagnosis and management of PBM and its complications. For early diagnosis, the presence of extrahepatic bile duct dilatation or gallbladder wall thickening may provide a clue to PBM with or without biliary dilatation, respectively. The pancreaticobiliary anatomy should be closely examined if imaging reveals these findings. Radiologists should also carefully evaluate follow-up images in PBM patients even years after prophylactic surgery because residual bile ducts remain at risk for cancer.©RSNA, 2020.

Peri-ampullary duodenal diverticulum: effect on extrahepatic bile duct dilatation after cholecystectomy

To investigate the effect of peri-ampullary duodenal diverticula (PAD) on extrahepatic bile duct (EHBD) dilatation before and after cholecystectomy. During a 5-year period, a total of 860 consecutive patients with prior cholecystectomy were examined using abdominal computed tomography (CT). After exclusion of those with other obstructive EHBD lesions, 61 patients with PAD were recruited for evaluation of EHBD dilatation before and after cholecystectomy and were compared with a randomly sampled control group (n=113) without PAD. EHBD diameter was measured on coronal reconstruction CT using electronic callipers on the picture archiving and communication system monitors by two reviewers in consensus. There was no significant difference in EHBD diameter between PAD and non-PAD groups (8.2±2.8 versus 7.8±2.3 mm; p=0.276) before cholecystectomy. Compared with preoperative diameter, EHBD was significantly dilated after cholecystectomy (7.9±2.5 versus 9.8±3.4 mm, p<0.001), regardless of the presence of PAD; the degree of change was more prominent in the PAD group than in the non-PAD group (3.3±2.4 versus 1.1±1.6 mm; p<0.001) after surgery. The size of PAD did not affect the degree of EHBD dilatation after cholecystectomy (p=0.522). In the non-PAD group, the degree of EHBD dilatation was positively correlated with the follow-up interval after cholecystectomy (r=0.298; p=0.002), while the PAD group showed no significant correlation (r=-0.036; p=0.797). In patients with ≥2 mm postoperative EHBD dilatation, PAD incidence was higher than that in other patients (odds ratio, 8.739; p<0.001). Regardless of their size or postoperative follow-up duration, PAD induce marked post-cholecystectomy biliary dilatation.

Congenital Absence of the Gall Bladder Associated with Forme-Fruste Choledochal Cyst: A Rare Case Report

An 11-year old girl presented with episodes of upper abdominal pain, fever, and non-bilious vomiting. Abdominal ultrasound (US) revealed a dilated common bile duct with non-visualization of the gallbladder. A magnetic resonance cholangiopancreatogram (MRCP) confirmed absence of the gallbladder and cystic duct, a ‘forme fruste’ choledocal cyst (FFCC) and no evidence of pancreatobiliary malunion (PBMU). Surgical excision of the dilated extrahepatic bile duct (EHBD) and a Roux-en-Y hepaticodocho-jejunostomy was successful. The discussion centers on the extremely rare association of congenital absence of the gallbladder (CAGB) with FFCC.

Diagnostic Value of Endoscopic Ultrasonography in Evaluation of Undetermined Etiology of Bile Ducts Dilation in Imaging

Aim: The aim is to determine the role of endoscopic ultrasonography in assessment of etiological diagnosis of bile ducts dilation when conventional imaging is inconclusive. Background Endoscopic ultrasonography (EUS) is an effective and primordial procedure in exploration of abnormalities of biliopancreatic junction. However, its results depend of the operator expertise, and its availability is currently insufficient in our regions. Methods: This is a retrospective descriptive study conducted from January 2008to May2019, including 42 patients with intra-and/or extra-hepatic bile duct dilatation without visible obstacle on imaging. TODANI classification was considered for common bile duct (CBD) cystic dilations. Statistical analysis was performed by SPSS software version24.0. Results: We enrolled 42patients responding to inclusion criteria in the study, which represented 10.18% of all indications of EUS. The mean age of our patients was 59.05±13.11 years, with a female predominance. Endoscopic findings showed a normal papilla in 92, 9%. EUS showed a dilated CBD in 66.8% of cases, she has allowed to make the diagnosis of a cystic dilation of CBD in 52,4%, predominated by type Ia and Ib in 45,2% and 4.8% respectively, of ampulloma in 4.8% (n = 2), papillomatosis of the bile ducts in 2.4% (n = 1), one case of pancreatic head tumor (2,4%), and choledocholithiasis in 4.8% (n = 2). By against EUS showed no expansion in 33.3% (n = 14). Conclusion: Our study confirms the prominent place of EUS in etiological profile of bile ducts dilatations when imaging is inconclusive.

Gallbladder Adenosquamous Carcinoma Associated with Biliopancreatic Maljunction: aggressive resection and survival

Background: Pancreaticobiliary maljunction (PBM) is a congenital malformation in which the pancreatic and bile ducts join outside the duodenal wall. It has been reported to be associated with an increased risk of gallbladder carcinoma. Gallbladder adenosquamous carcinoma is rare, with a worse prognosis compared to patients with adenocarcinoma. We report a case of adenosquamous carcinoma of gallbladder associated with PBM. Methods: A 56-year-old woman was admitted to the hospital complaining of pain in right hypochondrium. Ultrasonography and computed tomography revealed an elevated lesion inside the gallbladder and dilated extrahepatic bile duct. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography showed PBM with biliary dilatation of 15mm in the largest diameter. Results: Cholecystectomy, bile duct resection with a Roux-en-Y hepaticojejunostomy and hepatic resection (S4b-S5) with regional lymphadenectomy was performed. Histology demonstrated an adenosquamous cell carcinoma of the gallbladder with invasion to the muscle layer and two metastases of the resected lymph nodes (T2N1M0). Fourteen months after the operation, the patient is doing well with no signs of recurrence. Conclusion: PBM is a high risk factor for the development of gallbladder carcinoma because of a continuous reflux of pancreatic juice into the biliary tract. Aggressive surgical resection without residual tumor (R0) provides a chance for long-term survival in patients with gallbladder adenosquamous carcinoma.

Difficulties and countermeasures of diagnosis and treatment of biliary dilatation

The pathogenesis of biliary dilatation is unclear and the preoperative evaluation of pancreaticobiliary junction type is still difficult. The indication of minimally invasive surgery is limited. Diagnosis and treatment of gestational biliary dilatation and reoperation of biliary dilatation are two tricky problems. There are two dilemmas in the treatment of biliary dilatation, including the balance between radical resection of pathological bile ducts and organ reservation, as well as balance between radical resection of pathological bile ducts and surgical safety. The most difficult regions of diagnosis and treatment lie in the hilar dilated bile duct and dilated bile duct in the pancreatic segment. Dong′s classification simplifies the classification of extrahepatic bile duct dilatation and subdivides the classification of intrahepatic bile duct dilatation on the basis of Todani classification. It highlights the difficulties of treatment and provides more accurate basis for choosing appropriate method according to the classification. The therapeutic goals of biliary dilatation are the radical excision of pathological bile duct, removal of the secondary lesions and reconstruction of excellent cholangiojejunostomy. The principle of surgical procedure choice is choosing appropriate method according to the classification. Perihilar surgery technique and pancreatic door plate descending technique can be used to deal with the dilated bile ducts at hilar and pancreatic segment on the basis of accurate evaluation in order to improve the thoroughness and security of pathological bile duct resection. Not only short-term complications such as postoperative bile leakage and pancreatic leakage require management, but radical resection of lesion and long-term life quality of patients should be paid more attention. Key words: Biliary dilatation; Perihilar surgery technique; Dong′s classification; Pancreatic door plate descending technique

Pancreatic-Type Mixed Acinar Neuroendocrine Carcinoma Arising in the Common Bile Duct: A Case Report

Abstract Mixed acinar neuroendocrine carcinoma (MAcNEC) of pancreatic type arising in an extrapancreatic location is extremely rare. We present a case of a 70-year-old woman with constipation, abdominal discomfort, and jaundice. Imaging studies revealed a 1.8-cm solid mass in the common bile duct causing dilatation of intrahepatic and extrahepatic bile ducts, which was resected. Microscopically, the tumor was limited to the wall of the bile duct, without involvement of the pancreas, and was composed of solid sheets and nests of relatively monomorphic cells with minimal to moderate amounts of cytoplasm, granular chromatin, focally prominent nucleoli, and up to 160 mitoses per 10 high-power fields. Immunohistochemistry showed equal to or greater than 30% positivity for synaptophysin, chromogranin, chymotrypsin, and trypsin. A diagnosis of MAcNEC was rendered. Immunohistochemistry plays a pivotal role in the identification of these tumors and discrimination from other related entities, neuroendocrine neoplasms in particular. Because pancreatic heterotopia has been described in the common bile duct, we postulate that this case of MAcNEC in the common bile duct, the first to be reported in the literature, may have arisen via malignant transformation of pancreatic heterotopia.

Application value of perihilar surgery technique in the reoperation of biliary dilatation of central large intra- and extra-hepatic bile ducts above the hilar convergence

Objective To explore the application value of perihilar surgery technique in the reoperation of biliary dilatation of central large intra- and extra-hepatic bile ducts above the hilar convergence. Methods The retrospective cross-sectional study was conducted. The clinical data of 3 patients with biliary dilatation of central large intra- and extra-hepatic bile ducts above the hilar convergence who underwent the reoperation in the Renji Hospital Affiliated to Shanghai Jiaotong University School of Medicine from August 2017 to January 2018 were collected. All three patients had been collected a detailed previous surgical history. After preoperative lab and imaging examinations, evaluation of liver function, residual liver volume and general condition were done, then 3 patients underwent left hemihepatectomy + dilated right hepatic duct and extrahepatic bile duct resection, right anterior and posterior hepatic duct reconstructive surgery and Roux-en-Y anastomosis of the jejunum. The surgical procedures followed as: intra-abdominal adhesions separation, extrahepatic antergrade dissection of porta hepatis, transverse cutting the dilated extrahepatic bile duct, split the cantlie line, exposure of the hilar plate, left hemihepatectomy, dilated right hepatic bile duct resection, right anterior and posterior hepatic duct remodeling and biliary-enteric anastomosis. Observation indicators included: (1) surgical and postoperative recovery; (2) follow-up. Follow-up using outpatient examination and telephone interview was performed to detect general condition, complications, liver function and residual choledochal cysts up to May 2018. Results (1) Surgical and postoperative recovery: All the 3 patients underwent choledochal cysts resection + left hemihepatectomy + dilated right hepatic duct + right anterior and posterior hepatic duct reconstructive surgery and Roux-en-Y anastomosis of the jejunum using the perihilar surgery technique and extrahepatic anterograde combined by intrahepatic retrograde dissection method exposing portal hepatis. There was no perioperative death. The operation time and volume of intraoperative blood loss in 3 patients were 435 minutes, 490 minutes, 395 minutes and 250 mL, 300 mL, 200 mL, respectively. There was no intraoperative blood transfusion. Three patients had no bleeding and abdominal liver function. One patient with bile leakage and delayed gastric emptying at 1 week postoperatively received puncture drainage, gastrointestinal decompression, gastric lavage with hypertonic saline, acupuncture and total parenteral nutrition, then bile leakage was cured after 3-week therapy, gastric motility was improved after 5-week therapy, and then gastric tube was removed. The abdominal drainage tube was removed at 3 weeks postoperatively in 1 patient and at 1 week postoperatively in 2 patients. The postoperative gross specimen examinations showed intra- and extra-hepatic bile duct dilatation in 3 patients, including 2 combined with choledocholithiasis and 1 with left intrahepatic bile duct cancer. The postoperative pathological findings showed that 3 patients had intrahepatic bile duct cystic dilatation with chronic inflammation, peripheral small bile duct hyperplasia with inflammatory cell infiltration, 1 of which had intrahepatic intraductal papilloma with high grade intraepithelial neoplasia. One and 2 patients were discharged from hospital at 7 weeks postoperatively and 2 weeks postoperatively, respectively. (2) Follow-up: All 3 patients were followed up for 4-8 months. During the follow-up, patients had good general condition and no symptoms of cholecystitis such as abdominal pain, chills and fever, liver function was normal, and no residual bile duct cyst was found by enhanced scan of CT. Conclusion The perihilar surgery technique and extrahepatic anterograde combined by intrahepatic retrograde dissection method exposing portal hepatis for the treatment of biliary dilatation of central large intra- and extrahepatic bile ducts above the hilar convergence can increase the radical resection rate and surgical efficacy. Key words: Biliary dilatation; Perihilar technique; Central large hepatic bile duct above the hilar convergence; Extrahepatic anterograde combined by intrahepatic retrograde dissection method exposing portal hepatis; Surgical procedures, operative