Bile Duct Involvement Research Articles

Experience in treating patients with autoimmune pancreatitis

Objective — to establish the main diagnostic signs of autoimmune pancreatitis and aspects of patient treatment. Materials and methods. The study analyzed the results of examination and treatment of 17 patients with autoimmune pancreatitis (AIP) from 2010 to 2022. Among the total number of patients with AIP, there were 11 men (65%) and 6 women (35%). The average age of the patients was 52.4 years. Among all patients with AIP, focal involvement of the pancreas was found in 3 (18%) patients, with a predominant involvement of the head of the pancreas. Segmental form of AIP was diagnosed in 6 (35%) patients, while diffuse form was found in 8 (47%) patients. Type 1 AIP was identified in 13 (76%) patients, and type 2 AIP in 4 (24%) patients. For all patients suspected of AIP, the HISORt criteria were assessed: instrumental visualization, serological and histological verification, determination of the volume of pancreatic involvement, and response to steroid therapy. Results. Recurrence of AIP was observed in 8 (47.0%) patients with type 1 AIP and 1 (5.8%) patient with type 2 AIP. AIP recurred in patients with proximal bile duct involvement, diffuse pancreatic involvement, persistently elevated IgG4 levels after steroid induction, delayed radiological remission, and damage to more than two organs. Increased serum levels of IgG, IgG4, apnd eosinophilia indicated a recurrence of IgG4‑RD. A repeat induction of steroids was performed in patients with recurrent AIP, which proved to be very effective, resulting in high remission rates, specifically in 7 (70%) patients with type 1 AIP and in 1 (100%) patient with type 2 AIP. Among all AIP patients that were operated on, 3 (40%) underwent Roux‑en‑Y hepaticojejunostomies, 1 (20%) pancreaticoduodenectomy, and 1 (20%) a Frey procedure. Conclusions. The low incidence of AIP necessitates the use of a clear diagnostic algorithm, and the peculiarities of the disease’s course require compliance with all the principles of conservative treatment and surgical interventions in case of surgical complications.

Gallbladder fossa lymphangioma encasing the common bile duct: a case report and review of the literature

Introduction: Lymphangiomas are rare low-flow lymphatic vessel malformations mostly diagnosed in childhood. Among the abdominal lymphangiomas, the gallbladder lymphangioma is a rare presentation, and only a few cases have been reported till date, of which majority were cystic lymphangiomas. Case presentation: The authors present a case of a 48-year-old female with abdominal pain and normal physical examination and laboratory findings. On the performed imaging, a multiloculated cystic lesion, located in the gallbladder fossa, was found. The patient underwent open cholecystectomy, and surprisingly, the cyst extended to the common bile duct, which was separated cautiously. The cyst was resected, and the histopathological findings confirmed cystic lymphangioma. Conclusion: The cystic lymphangioma of the gallbladder can be asymptomatic, although most of the literature has reported abdominal pain. The diagnosis of lymphangioma is complicated, especially when intra-cystic hemorrhage happens. Ultrasonography, computed tomography, and magnetic resonance imaging are usually performed. The surgical excision of the cyst and the gallbladder is the treatment of choice. Although bile duct involvement is extremely rare, it should be considered during the surgery.

Spontaneous perforation of the common bile duct: an uncommon complication of primary sclerosing cholangitis.

To describe the MR features and prognosis of patients with an uncommon complication of primary sclerosing cholangitis (PSC) characterized by a spontaneous perforation of the common bile duct (CBD) resulting in a peri-biliary collection and a pseudo-cystic appearance of the CBD. A single-center cohort of 263 patients with PSC who had at least two MRIs between 2003 and 2022 and a minimum follow-up of 1 year was retrospectively analyzed. MRI data (characteristics of CBD perforation and MR features of PSC) and clinical data were assessed. Analysis of survival without liver transplantation according to type of PSC (classical or CBD spontaneous perforation) was performed according to the Kaplan-Meier method and the curves were compared using the Log-Rank test. A total of nine (3.4%) PSC patients (5 males) had perforation of the CBD with a median age at diagnosis of 18 years compared to 33 years for the control group (p = 0.019). The peri-biliary collections were variable in appearance (fusiform or pedunculated), with a diameter ranging from 5 to 54 mm. All nine patients showed intra- and extra-hepatic bile duct involvement, dysmorphia, and high ANALI scores. The clinical course was characterized by numerous complications in most patients, and five patients (56%) underwent liver transplantation at a median time of 5 years from diagnosis, compared to 40 patients (16%) in the control group (p = 0.02). The spontaneous perforation of the common bile duct is an uncommon complication of primary sclerosing cholangitis that affects young patients and is associated with a poor prognosis. This uncommon complication of primary sclerosing cholangitis with perforation of the common bile duct resulting in a peri-biliary collection and a pseudo-cystic appearance of the common bile duct is characterized by a poor prognosis in younger patients. • Among 263 patients with primary sclerosing cholangitis (PSC), nine patients (3.6%) had an uncommon complication characterized on MRI by perforation of the common bile duct (CBD). • This perforation of the CBD was responsible in all nine cases for the formation of a peri-biliary collection, giving a pseudo-cystic appearance to the CBD. • The spontaneous perforation of the common bile duct is an uncommon complication of primary sclerosing cholangitis that affects young patients with a poor prognosis.

Biliary atresia: the development, pathological features, and classification of the bile duct.

Biliary atresia is an occlusive biliary disease involving intrahepatic and extrahepatic bile ducts. Its etiology and pathogenesis are unclear. There are many manifestations of bile duct involvement in biliary atresia, but little is known about its occurrence and development. In addition, different classification methods have been proposed in different periods of biliary atresia, each with its advantages and disadvantages. The combined application of biliary atresia classification will help to improve the survival rate of patients with native liver. Therefore, this article reviews the development, pathological features, and classification of intrahepatic and extrahepatic bile ducts in biliary atresia, to provide a reference for the study of the pathogenesis and the choice of treatment methods.

Cholangitis Induced by Immune Checkpoint Inhibitors: Analysis of Pharmacovigilance Data

Immune checkpoint inhibitors (ICIs) are remarkable anticancer therapies that have revolutionized the oncological prognosis of many cancers.1 The considerable efficacy of ICIs is associated with the onset of more- or less-serious, immune-related adverse effects (irAEs) affecting several organs, which can concern up to 70% of patients, owing to a loss of self-tolerance during the restoration of antitumor immunity.2 Checkpoint inhibitor-induced liver injury (CHILI), which may occur in up to 25% of patients, is treated with steroids as first-line treatment, and immunosuppressive drugs as second-line treatment.3 Recently, ICI-induced cholangitis was described as an emerging irAE. Hence, Pi etal4 reviewed all 53 published cases of ICI-induced cholangitis and compared the different types of bile duct involvement. We recently described CHILI according to the biological profile: cholestatic, hepatocellular, or mixed.5 Cholestatic profiles were associated with macroscopic and/or microscopic bile duct damage, and time to resolution was significantly longer. More recently, Onoyama etal6 and Parlati etal7 described a poorer response to steroids in cases of biliary histologic damage or ICI-induced sclerosing cholangitis. The latest European Society for Medical Oncology guidelines include the management of cholangitis, which is succinct and still poorly documented.3 The aim of this study therefore was to analyze the cases of ICI-induced cholangitis reported in the French pharmacovigilance system to describe their clinical characteristics, evolution, and outcome.

Effects of Cystic Duct Margin Involvement on the Survival Rates of Patients With Gallbladder Cancer: A Propensity Score-Matched Case-Control Study.

Background In gallbladder cancer (GBC), extrahepatic bile duct (EHBD) resection is selectively performed if gross direct extension or microscopic involvement of the cystic duct margin (CDM) is detected. Although CDMis usually sent for frozen biopsy intraoperatively in most centers, there are no studies regarding the routine use of CDM frozen biopsy irrespective of the tumor location and paucity of literature regarding the impact ofCDM status on recurrence-free and overall survival inGBC. The presence of obstructive jaundice in GBC usually indicates the involvement of EHBD or cystic duct-bile duct junction. The present study aimed to analyze the necessity of routineCDM frozen biopsy inpatients with resectable GBC withoutjaundice, regardless of the tumor location. The impact of positive CDM on survival was also evaluated. Methods This retrospective observational case-control study was conducted from May 2009 to March 2021 and included 105 patients with resectable GBC without macroscopic EHBD infiltration andjaundice. Patients were divided into CDM-negative (n=91) and CDM-positive (n=14) groups. Propensity score matchingwas performed for variables such asperformance status, tumor size, tumor-node-metastasis (TNM) stage, and adjuvant chemotherapy. After propensity score matching, 27 patients (CDM-negative=13, CDM-positive=14) were included. The primary outcome was to analyze the role of routine CDM frozen biopsy regardless of tumor location, and secondary outcomes were to study the impact of positive CDM status on survival andevaluate predictive factors for CDM positivity. A subgroup analysis was conducted to assess clinicopathologic characteristics and outcomes of the anatomical location of the tumor. Results Of 105 patients, 91 had negative CDM, and 14 had positive CDM. Among 14 patients with positive CDM, only one patient had a tumor in the fundus/body, and the remaining had a tumor involving the neck. All CDM-positive patients underwent bile duct excision with hepaticojejunostomy. Common bile duct (CBD) involvement was present in 50% of patients with positive CDM in the final histopathological examination. In the matched population, patients with positive CDM had a significantly higher rate of neck tumors (p=0.001). Recurrence-free survival (24 vs. 12 months, p=0.30) and overall survival (24.5 vs. 20 months, p=0.417) were comparable between CDM-negative and CDM-positive groups, respectively. On multivariate analysis, preoperative and intraoperative tumor location were independent predictive factors for CDM positivity. On subgroup analysis, 30 patients had tumor involving the neck of the gallbladder, and the remaining 75 had at the fundus and body of the gallbladder. Neck tumors had inferior recurrence-free survival (17 vs. 30 months, p=0.012) and overall survival (24 vs. 36 months, p=0.048) compared to non-neck tumors. Conclusions Routine use of CDM frozen analysis in patients with resectable GBC without jaundice, regardless of tumor location, can be avoided. Itcan be selectively preferred in patients with GBC involving the neck since tumor location is found to be an independent predictive factorfor CDM positivity. Positive CDM has comparable survival outcomes to negative CDM, providing a similarR0 resection rate and tumor stage.However, neck tumors have aworse prognosis than non-neck tumors.

Differentiation of autoimmune pancreatitis from pancreatic adenocarcinoma using CT characteristics: a systematic review and meta-analysis.

To determine informational CT findings for distinguishing autoimmune pancreatitis (AIP) from pancreatic ductal adenocarcinoma (PDAC) and to review their diagnostic accuracy. A systematic and detailed literature review was performed through PubMed, EMBASE, and the Cochrane library. Similar descriptors to embody the identical image finding were labeled as a single CT characteristic. We calculated the pooled diagnostic odds ratios (DORs) of each CT characteristic using a bivariate random-effects model. A total of 145 various descriptors from 15 studies (including 562 AIP and 869 PDAC patients) were categorized into 16 CT characteristics. According to the pooled DOR, 16 CT characteristics were classified into three groups (suggesting AIP, suggesting PDAC, and not informational). Seven characteristics suggesting AIP were diffuse pancreatic enlargement (DOR, 48), delayed homogeneous enhancement (DOR, 46), capsule-like rim (DOR, 34), multiple pancreatic masses (DOR, 16), renal involvement (DOR, 15), retroperitoneal fibrosis (DOR, 13), and bile duct involvement (DOR, 8). Delayed homogeneous enhancement showed a pooled sensitivity of 83% and specificity of 85%. The other six characteristics showed relatively low sensitivity (12-63%) but high specificity (93-99%). Four characteristics suggesting PDAC were discrete pancreatic mass (DOR, 23), pancreatic duct cutoff (DOR, 16), upstream main pancreatic duct dilatation (DOR, 8), and upstream parenchymal atrophy (DOR, 7). Eleven CT characteristics were informational to distinguish AIP from PDAC. Diffuse pancreatic enlargement, delayed homogeneous enhancement, and capsule-like rim suggested AIP with the highest DORs, whereas discrete pancreatic mass suggested PDAC. However, pooled sensitivities of informational CT characteristics were moderate. This meta-analysis underscores eleven distinctive CT characteristics that aid in differentiating autoimmune pancreatitis from pancreatic adenocarcinoma, potentially preventing misdiagnoses in patients presenting with focal/diffuse pancreatic enlargement. • Diffuse pancreatic enlargement (pooled diagnostic odds ratio [DOR], 48), delayed homogeneous enhancement (46), and capsule-like rim (34) were CT characteristics suggesting autoimmune pancreatitis. • The CT characteristics suggesting autoimmune pancreatitis, except delayed homogeneous enhancement, had a general tendency to show relatively low sensitivity (12-63%) but high specificity (93-99%). • Discrete pancreatic mass (pooled diagnostic odds ratio, 23) was the CT characteristic suggesting pancreatic ductal adenocarcinoma with the highest pooled DORs.

Pushing the Limits: Associating Liver Partition and Portal Vein Ligation for Staged Hepatectomy for Advanced Hepatocellular Carcinoma with Macrovascular Invasion

INTRODUCTION: Patients with advanced hepatocellular carcinoma and macrovascular invasion (MVI) have dismal prognosis and are referred to systemic treatment or palliation. The aim of this study is the investigation the outcomes of patients with HCC and MVI undergoing operation in the form of the associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) procedure. METHODS: Demographics and operative data as well as follow-up were retrospectively reviewed. All types of hepatectomy and all types of ALPPS modification were included. MVI was categorized according to the Japanese Liver Cancer Study Group classification. RESULTS: A total of 28 patients were included in the study. Viral etiology was the most common cause of chronic liver disease (89.3%); 85.7% of patients were cirrhotic, with a median Model for End-Stage Liver Disease score of 9 (7 to 10). MVI of the hepatic veins or inferior vena cava was diagnosed in 46.4% of patients, and portal vein involvement was present in 64.2% of cases. Four patients (14.2%) were diagnosed with bile duct involvement. No patients died after step 1, although complication occurred in 21.4% of cases. After step 2, 3 patients (11.5%) died and 20 (69.2%) developed complication. Grade B and C after hepatectomy liver failure occurred in 57.6% and 11.5% of patients, respectively. After a median follow-up of 18 months (7 to 35), median survival was 22 months (3 to 40). Eleven patients (39.3%) experienced recurrence. Median disease-free survival was 15 months (5 to 26). CONCLUSION: The ALPPS procedure is an extreme rescue approach in otherwise inoperable advanced HCC with MVI. The procedure is associated with high morbidity and mortality and patient selection is pivotal. Oncologic outcomes are safe and should be further investigated.

Safra yoluna rüptüre karaciğer kist hidatik tedavisi:Endoskobik drenaj

Abstract:
 Hydatid cyst disease is a zoonotic disease caused by the transmission of Echinococcus granulosus, which is in the cestode class, to humans during the larval period. This disease causes serious problems by affecting public health and national economy as an important parasitic disease, especially in many developing countries. Liver cyst cysts can be complicated according to their diameter and location. Complication rate reaches 60%. The leading complications are bile duct involvement and related complications. Bile duct problems should be solved first in cysts associated with biliary tract. ERCP and endoscopic sphinterotomy can be performed. Here we discussed the treatment and management of a 35-year-old patient with hydatid cyst that opened into the biliary tract with ERCP, who presented to the emergency room with fever and signs of infection.
 Keywords:endoscopic cyst evacuation, cholangitis, endoscopic retrograde cholangiopancreatography

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.

Longitudinal Assessment of Bile Duct Loss in Primary Biliary Cholangitis.

Bile duct involvement is a key finding of primary biliary cholangitis (PBC). The aim of this study was to evaluate baseline ductopenia and disease progression. Retrospective longitudinal histological follow-up of treatment-naive patients with PBC. Eighty-three patients were included, with ductopenia correlated to fibrosis stage at baseline. The cumulative incidence of severe ductopenia remained stable after 5 years, whereas fibrosis continually increased over time. Baseline AST-to-Platelet Ratio Index and elevated alkaline phosphatase >2 times the normal with abnormal bilirubin were associated with ductopenia progression. Bile duct injury does not seem to follow the same course as fibrosis in PBC.

Important CT Findings for Prediction of Incomplete (R1 or R2) Resection and Poor Survival of Perihilar Cholangiocarcinoma after Curative-Intent Surgery

Purpose: To evaluate CT findings to predict incomplete (R1 or R2) resection and poor survival in patients with perihilar cholangiocarcinoma using pre-operative CT.Materials and Methods: From 2006 to 2012, a total of 139 patients with perihilar cholangiocarcinoma who underwent pre-operative multiphase CT and subsequent curative-intent surgery were included. After two radiologists independently reviewed CT findings including the likelihood of bile duct (BD) involvement from intrapancreatic common bile duct (CBD) to bilateral second-order branches and peritumoral fat stranding using a 5-point scale, vessel involvement (no, abutment, encasement), and LN involvement, imaging findings were finalized by a consensus of two radiologists. When the likelihood scale of 4 or more on preoperative CT was regarded as BD involvement, the diagnostic ability of CT was analyzed by the receiver operating curve using histopathologic results as a reference standard. Residual tumor categorized into no residual tumor (R0) and residual tumor (R+; R1 or R2). Predictive factors of R+ resection on pre-operative CT were analyzed by logistic regression. Cox proportional hazard model was used to determine the prognostic factor for overall survival by using pre-operative CT findings and laboratory results.Results: Seventy-one patients were R0 and sixty-eight patients were R+ resection. For resectability evaluation, mid-CBD involvement (score ≥ 4) in pre-operative CT was significant factor for R+ resection in multivariable analysis (<i>P</i> < 0.01) with substantial interobserver agreement. In multivariable Cox regression, intrapancreatic CBD involvement (score ≥ 4, hazard ratio [HR] = 1.81, <i>P</i> < 0.01) as well as elevated total bilirubin (HR = 1.53, <i>P</i> = 0.04) and CA 19-9 level (HR = 1.75, <i>P</i> < 0.01) were significant predictors for poor survival. Diagnostic ability to predict mid-CBD and intrapancreatic CBD involvement on pre-operative CT were 0.71 and 0.72 (AUC values).Conclusions: Distal longitudinal extent of perihilar cancer on pre-operative CT is a significant factor for margin positive resection and poor survival on curative-intent surgery.

Morphomolecular Classification Update on Hepatocellular Adenoma, Hepatocellular Carcinoma, and Intrahepatic Cholangiocarcinoma

Hepatocellular adenomas (HCAs), hepatocellular carcinomas (HCCs), and intrahepatic cholangiocarcinomas (iCCAs) are a highly heterogeneous group of liver tumors with diverse pathomolecular features and prognoses. High-throughput gene sequencing techniques have allowed discovery of distinct genetic and molecular underpinnings of these tumors and identified distinct subtypes that demonstrate varied clinicobiologic behaviors, imaging findings, and complications. The combination of histopathologic findings and molecular profiling form the basis for the morphomolecular classification of liver tumors. Distinct HCA subtypes with characteristic imaging findings and complications include HNF1A-inactivated, inflammatory, β-catenin-activated, β-catenin-activated inflammatory, and sonic hedgehog HCAs. HCCs can be grouped into proliferative and nonproliferative subtypes. Proliferative HCCs include macrotrabecular-massive, TP53-mutated, scirrhous, clear cell, fibrolamellar, and sarcomatoid HCCs and combined HCC-cholangiocarcinoma. Steatohepatitic and β-catenin-mutated HCCs constitute the nonproliferative subtypes. iCCAs are classified as small-duct and large-duct types on the basis of the level of bile duct involvement, with significant differences in pathogenesis, molecular signatures, imaging findings, and biologic behaviors. Cross-sectional imaging modalities, including multiphase CT and multiparametric MRI, play an essential role in diagnosis, staging, treatment response assessment, and surveillance. Select imaging phenotypes can be correlated with genetic abnormalities, and identification of surrogate imaging markers may help avoid genetic testing. Improved understanding of morphomolecular features of liver tumors has opened new areas of research in the targeted therapeutics and management guidelines. The purpose of this article is to review imaging findings of select morphomolecular subtypes of HCAs, HCCs, and iCCAs and discuss therapeutic and prognostic implications. Online supplemental material is available for this article. ©RSNA, 2022.

Molecular Identification of Cystoisospora Belli in Patients Infected with the Human Immunodeficiency Virus

Infection with Cystoisospora belli causes severe diarrhea, bile duct involvement, and Extra-intestinal spread in patients with acquired immunodeficiency syndrome (AIDS). This study included eight adult patients with AIDS and chronic diarrhea diagnosed stool cystosisporiasis (oocysts) and / or duodenal biopsies (asexual and sexual stages in epithelium). Identification was carried out at the molecular level using a nested-polymerase chain reaction (PCR) technique that amplifies a fragment of the ribosomal ribonucleic acid (RNA) small subunit gene, using DNA extracted from feces and biopsies. This work allowed us to make the diagnosis of cystosisporiasis through analysis coproparasitological, optimize the DNA extraction protocol from samples fecal and implement the nested -PCR technique for the diagnosis of C. belli in biological samples from infected patients.

NON-INVASIVE IMAGING OF CHOLANGIO-PANCREATIC DISEASES USING MAGNETIC RESONANCE CHOLANGIOPANCREATOGRAPHY AND CORRELATION WITH ENDOSCOPIC RETROGRADE CHOLANGIOPANCREATOGRAPHY

NON-INVASIVE IMAGING OF CHOLANGIO-PANCREATIC DISEASES USING MAGNETIC RESONANCE CHOLANGIOPANCREATOGRAPHY AND CORRELATION WITH ENDOSCOPIC RETROGRADE CHOLANGIOPANCREATOGRAPHY

Liver Transplantation for Hepatocellular Carcinoma With Bile Duct Tumor-Associated Thrombi: A Systematic Review and Pooled Analysis.

The significance of bile duct tumor-associated thrombi in patients undergoing transplantation for hepatocellular carcinoma (HCC) is controversial. Therefore, we performed a systematic review of the literature with pooled analysis to investigate the impact of biliary invasion on HCC recurrence and patient survival. Of 1,584 references screened, eight were included for analysis. Demographics, patient and tumor factors, recurrence, and survival data were analyzed. Time to recurrence and death were extracted from each paper by cross-referencing survival curves. A total of 35 patients across eight studies were pooled for analysis when follow-up data were available. At 1 year, 92.9% of patients undergoing transplantation for HCC with bile duct thrombi were alive. Overall survival at 3 and 5 years was 65.5 and 49.6%, respectively. At 1 year, 21.6% of patients had recurrence of their disease, while at 3 years, 50.4% of patients had recurrence. Of those patients with recurrence in the first year, 71.4% recurred within the first 3 months after transplantation. Overall patient survival decreased within the first 5 years, but then stabilized. The 5-year survival rate of 49.6% in this pooled analysis is lower than that reported for patients undergoing transplantation for HCC within the Milan criteria (50-78%) or recent reports in patients with portal vein involvement (63.6%), though data is limited by a lack of long-term follow-up in this understudied population. Transplantation for patients with HCC with bile duct involvement may be a viable treatment option, warranting further investigation.

Associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) for advanced hepatocellular carcinoma with macrovascular invasion.

Patients with advanced hepatocellular carcinoma (HCC) and macrovascular invasion (MVI) have dismal prognosis and are referred to systemic treatment or palliation. To investigate the outcomes of patients with HCC and MVI undergoing the associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) procedure. Demographics and operative data were retrospectively reviewed. All types of hepatectomies and all types of ALPPS modifications were included. MVI was categorized according to the Japanese Liver Cancer Study Group classification. 28 patients were included. Viral aetiology was the most common cause of chronic liver disease (89.3%). 85.7% of patients were cirrhotic, with a median MELD score of 9 (7-10). MVI of the hepatic veins or inferior vena cava was diagnosed in 46.4% of patients while portal vein involvement was present in 64.2% of cases. Four patients (14.2%) were diagnosed with bile duct involvement. No patients died after Step 1 while complications occurred in 21.4% of cases. Following step 2, 3 patients (11.5%) died and 20 (69.2%) developed complications. Grade B and C post-hepatectomy liver failure occurred in 57.6% and 11.5% of patients, respectively. After a median follow-up of 18months (7-35), median survival was 22months (3-40). Eleven patients (39.3%) recurred. Median disease-free survival was 15months (5-26). The ALPPS procedure is an extreme rescue approach in otherwise inoperable advanced HCC with MVI. The procedure is associated with high morbidity and mortality and patients' selection is pivotal. Oncological outcomes are safe and should be further investigated.

Inflammatory Bowel Diseases Affect the Phenotype and Disease Course of Coexisting Immune-Mediated Inflammatory Diseases: A Systematic Review With Meta-Analysis.

It is unclear whether inflammatory bowel diseases (IBDs) affect the phenotype and severity of co-occurring immune-mediated inflammatory diseases (IMIDs). We aimed to investigate the characteristics of IMIDs in relation to co-occurring IBD. We conducted a systematic review of Medline and EMBASE databases from inception to September 2020. We identified studies reporting the phenotype, severity, or disease course of IMIDs among patients with or without co-occurring IBD. A meta-analysis was conducted using random effects models. The electronic search yielded 13220 studies that we narrowed down to 73 eligible studies for full-text review, including 42 on primary sclerosing cholangitis, 12 on axial spondyloarthropathies, and 8 studies on psoriasis. In primary sclerosing cholangitis, IBD was associated with less frequent involvement of extrahepatic bile ducts (risk ratio [RR], 0.50; 95% confidence interval [CI], 0.33-0.75), longer liver transplantation-free survival (hazard ratio, 0.70; 95% CI, 0.60-0.82), and no increased risk of cholangiocarcinoma (RR, 0.88; 95% CI, 0.59-1.31). Patients with axial spondyloarthropathies and co-occurring IBD were characterized by an increased risk of dactylitis (RR, 2.06; 95% CI, 1.24-3.42), a lower Bath Ankylosing Spondylitis Radiology Index (mean difference [MD] = -2.28; 95% CI, -3.26 to -1.30), and better Schober's test results (MD = 1.07; 95% CI, 0.64-1.49). Psoriasis and co-occurring IBD was associated with reduced disease severity (RR, 1.41; 95% CI, 1.02-1.96) and less frequent presentation in nails (RR, 0.14; 95% CI, 0.05-0.42), with no apparent impact on psoriatic arthritis (RR, 0.94; 95% CI, 0.27-3.31). This systematic review with meta-analysis found IBD is associated with a distinct disease phenotype among the IMIDs investigated. Our findings emphasize the importance of multidisciplinary approaches to patients with co-occurring IMIDs and IBD.

Analysis of Clinical, Serological, and Imaging Features of Autoimmune Pancreatitis and a Case-Control Study on Prognostic Factors in Response to Hormone Therapy.

Objective The paper aimed to analyze the clinical, serological, and imaging features of autoimmune pancreatitis (AIP) and the prognostic factors affecting hormone therapy. Methods A total of 106 patients with AIP enrolled in our hospital from March 2016 to August 2018 were treated with the hormone. The curative effect and recurrence were followed up. The patients were divided into relapse group (n = 42) and nonrelapse group (n = 64) according to the recurrence within 3 years after initial hormone therapy. The symptoms and signs, laboratory examination, and treatment were compared, and binary logistic regression was employed to explore the risk factors of AIP recurrence. Results Among the 106 patients included in this study, there were 78 males and 28 females, with a male-to-female ratio of 3:1. The average age of onset was 56.25 ± 8.87 years; the minimum age was 39 years; and the maximum age was 7 years. The main clinical symptoms were jaundice (67.92%), abdominal pain (48.11%), and abdominal distension (33.96%). In addition, there were symptoms of weight loss, nausea, vomiting, itching, and gray stool. Previous complications included 27.35% diabetes (29/106), 22.64% hypertension (24/106), 35.84% smoking (38/106), and 28.30% alcohol consumption (30/106). The serological characteristics were mainly the increase in serum IgG4 level; 92.45% (98/106) level was higher compared to the upper limit of normal value; the median level was 11.65 g/L; and the highest level was 35.79 g/L. A total of 88.67% (94/106) had an abnormal liver function. The results of imaging examination indicated that 58.49% (62/106) of extrapancreatic organs were involved, of which 46.22% (49/106) were the most common bile duct involvement. All the patients in the group reached a state of remission after hormone treatment. After the disease was relieved, the patients were followed up for 3 years. The recurrence rate was 39.62% (42/106), and the median time of recurrence (month) was 9 (range 2–36). The recurrence rates within 1, 2, and 3 years were 20.75%, 31.13%, and 39.62%, respectively. Among the recurrent patients, 52.38% (22/42) relapsed within 1 year, 78.57% (33/42) within 2 years, and 100.00% (42/42) within 3 years. Multivariate analysis showed that the short duration of glucocorticoid therapy and involvement of extrapancreatic organs were risk factors for relapse after glucocorticoid therapy in patients with type I AIP. Conclusion Type 1 AIP is more common in middle-aged and elderly men. The clinical symptoms of jaundice, abdominal pain, and abdominal distension are common, often accompanied by involvement of extrapancreatic organs, of which bile duct involvement is the most common. Type 1 AIP glucocorticoid treatment acceptance and disease remission are better, but the recurrence rate is higher after glucocorticoid treatment. Patients with a short time of glucocorticoid treatment and involvement of extrapancreatic organs may have a higher risk of recurrence.

Liver Transplantation with Concurrent Pancreatoduodenectomy for Hilar Cholangiocarcinoma with Common Bile Duct Involvement

Introduction: Neoadjuvant chemoradiotherapy with orthotopic liver transplantation (OLT) is effective treatment for perihilar cholangiocarcinoma (pCCA). Although visible common bile duct (CBD) involvement is an exclusion, patients with primary sclerosing cholangitis (PSC) and microscopic CBD involvement detected by frozen section or endoscopic brushings prior to neoadjuvant therapy have undergone pancreatoduodenectomy (PD) or planned en-bloc hepatopancreatoduodenectomy (HPD) during OLT.