Social Vulnerability and Premature Cardiovascular Mortality Among US Counties, 2014 to 2018.

BACKGROUND: Substantial differences exist between United States counties with regards to premature (<65 years of age) cardiovascular disease (CVD) mortality. Whether underlying social vulnerabilities of counties influence premature CVD mortality is uncertain. METHODS: In this cross-sectional study (2014-2018), we linked county-level CDC/ATSDR SVI (Centers for Disease Control and Prevention/Agency for Toxic Substances and Disease Registry Social Vulnerability Index) data with county-level CDC WONDER (Centers for Disease Control and Prevention Wide-Ranging Online Data for Epidemiological Research) mortality data. We calculated scores for overall SVI and its 4 subcomponents (ie, socioeconomic status; household composition and disability; minority status and language; and housing type and transportation) using 15 social attributes. Scores were presented as percentile rankings by county, further classified as quartiles on the basis of their distribution among all US counties (1st [least vulnerable] = 0 to 0.25; 4th [most vulnerable = 0.75 to 1.00]). We grouped age-adjusted mortality rates per 100 000 person-years for overall CVD and its subtypes (ischemic heart disease, stroke, hypertension, and heart failure) for nonelderly (<65 years of age) adults across SVI quartiles. RESULTS: Overall, the age-adjusted CVD mortality rate per 100 000 person-years was 47.0 (ischemic heart disease, 28.3; stroke, 7.9; hypertension, 8.4; and heart failure, 2.4). The largest concentration of counties with more social vulnerabilities and CVD mortality were clustered across the southwestern and southeastern parts of the United States. The age-adjusted CVD mortality rates increased in a stepwise manner from 1st to 4th SVI quartiles. Counties in the 4th SVI quartile had significantly higher mortality for CVD (rate ratio, 1.84 [95% CI, 1.43-2.36]), ischemic heart disease (1.52 [1.09-2.13]), stroke (2.03 [1.12-3.70]), hypertension (2.71 [1.54-4.75]), and heart failure (3.38 [1.32-8.61]) than those in the 1st SVI quartile. The relative risks varied considerably by demographic characteristics. For example, among all ethnicities/races, non-Hispanic Black adults in the 4th SVI quartile versus the 1st SVI quartile exclusively had significantly higher relative risks of stroke (1.65 [1.07-2.54]) and heart failure (2.42 [1.29-4.55]) mortality. Rural counties with more social vulnerabilities had 2- to 5-fold higher mortality attributable to CVD and subtypes. CONCLUSIONS: In this analysis, US counties with more social vulnerabilities had higher premature CVD mortality, varied by demographic characteristics and rurality. Focused public health interventions should address the socioeconomic disparities faced by underserved communities to curb the growing burden of premature CVD.

Race and Ethnicity and Cardiometabolic Risk Profile: Disparities Across Income and Health Insurance in a National Sample of US Adults.

CONTEXT: Income and health insurance are important social determinants of cardiovascular disease (CVD) and may explain much racial/ethnic variation in CVD burden. However, racial/ethnic disparities in cumulative cardiometabolic (CMB) risk profile by insurance type and income level have not been studied on a national scale. OBJECTIVES: To test the hypothesis that racial/ethnic minorities experience greater CMB burden at each income level and insurance type than non-Hispanic Whites (NHW). SETTING: This study used nationally representative data from the National Health Interview Survey (NHIS). DESIGN: Observational (cross-sectional). PARTICIPANTS: In total, 134661 (weighted N = 197780611) adults, 18 years or older, from the 2013-2017 NHIS. PRIMARY OUTCOME: CMB risk profile. INTERVENTION/ANALYSIS: Age-adjusted prevalence of optimal, average, and poor CMB risk profile-defined respectively as self-report of 0, 1-2, and 3 or more risk factors of diabetes, hypertension, obesity, or hypercholesterolemia-was examined for NHW, non-Hispanic Blacks (NHB), and Hispanics. Multivariable ordinal logistic regression models were used to test the association between race and ethnicity and CMB profile overall and separately by household income level and insurance type. RESULTS: Overall, 15% of NHB and 11% of Hispanics experienced poor CMB risk profile, compared with 9% for NHW. In fully adjusted models, NHB and Hispanics, respectively had nearly 25%-90% and 10%-30% increased odds of poor CMB profile across insurance types and 45%-60% and 15%-30% increased odds of poor CMB profile across income levels, relative to NHW. The observed disparities were widest for the Medicare group (NHB: OR = 1.90; Hispanics: OR = 1.31) and highest-income level (NHB: OR = 1.62). CONCLUSIONS: Racial/ethnic minorities experience poor CMB profile at each level of income and insurance. These findings point to the need for greater investigation of unmeasured determinants of minority cardiovascular (CV) health, including structural racism and implicit bias in CV care.

Super-resolution for upper abdominal MRI: Acquisition and post-processing protocol optimization using brain MRI control data and expert reader validation.

PURPOSE: Magnetic resonance (MR) cholangiopancreatography (MRCP) is an established specialist method for imaging the upper abdomen and biliary/pancreatic ducts. Due to limitations of either MR image contrast or low through-plane resolution, patients may require further evaluation with contrast-enhanced computed tomography (CT) images. However, CT fails to offer the high tissue-ductal-vessel contrast-to-noise ratio available on T2-weighted MR imaging. METHODS: MR super-resolution reconstruction (SRR) frameworks have the potential to provide high-resolution visualizations from multiple low through-plane resolution single-shot T2-weighted (SST2W) images as currently used during MRCP studies. Here, we (i) optimize the source image acquisition protocols by establishing the ideal number and orientation of SST2W series for MRCP SRR generation, (ii) optimize post-processing protocols for two motion correction candidate frameworks for MRCP SRR, and (iii) perform an extensive validation of the overall potential of upper abdominal SRR, using four expert readers with subspeciality interest in hepato-pancreatico-biliary imaging. RESULTS: Obtained SRRs show demonstrable advantages over traditional SST2W MRCP data in terms of anatomical clarity and subjective radiologists' preference scores for a range of anatomical regions that are especially critical for the management of cancer patients. CONCLUSIONS: Our results underline the potential of using SRR alongside traditional MRCP data for improved clinical diagnosis.

Current management of cystic echinococcosis: a survey of specialist practice.

BACKGROUND: Cystic echinococcosis (CE) is a significant public health problem worldwide. However, there remains a dearth of evidence guiding treatment in various stages of CE. The 2010 World Health Organization (WHO) Informal Working Group on Echinococcosis (WHO IWGE) guidance is thus based on expert consensus rather than a good evidence base. This study aims to describe the way clinicians worldwide manage CE and to establish whether clinicians follow WHO IWGE guidance. METHODS: Using the online surveying tool SurveyMonkey, a questionnaire was produced detailing 5 clinical cases. Clinicians treating CE were identified and asked how to manage each case through tick-box and short-answer questions. RESULTS: The results showed great variation in practice worldwide. There are practices in common use that are known to be ineffectual, including puncture, aspiration, injection, reaspiration procedures on WHO type 2 cysts, or outdated, including interrupted, rather than continuous, courses of albendazole. A number of unsafe practices were identified such as using scolicidal agents in cysts communicating with the biliary tree and short-course medical therapy for disseminated disease. Most clinicians do not follow the WHO IWGE guidance, but the reasons for this are unclear. CONCLUSIONS: Management of CE varies greatly worldwide. There are key areas of CE for which there is no evidence on which to base guidelines, and randomized controlled trials are needed together with a well-designed international registry to collect data. Further work is required to establish why clinicians do not follow the IWGE guidance, together with better dissemination of future guidance.

Comprehensive Examination of Cholangiocarcinoma Patients Treated with Novel Targeted Therapies after Extended Molecular Profiling on Liquid Biopsies.

BACKGROUND: Cholangiocarcinoma (CCA) is associated with poor outcomes and limited treatment options, leading to increased use of targeted therapies for its management. Here, we performed one of the largest single-centre reviews evaluating outcomes following personalised targeted agents in CCA patients. METHODS: All consecutive CCA patients receiving systemic therapy between January 2010 and April 2023 at UCLH were included. The primary objective of this study was to evaluate treatment response, survival outcomes and predictors of clinical benefit in CCA patients treated with molecularly guided therapies. Patient demographic factors, disease characteristics and survival outcomes were evaluated using the Kaplan-Meier method and Cox proportional-hazards models. RESULTS: Of the 227 consecutive CCA patients, 162 (71%) had molecular profiling, of whom 56 (35%) were eligible and 55 received molecular-targeted treatment. CCA histological classifications comprised intrahepatic (N = 32), extrahepatic (N = 11), hilar (N = 4) and unknown (N = 9) subtypes. Most patients received targeted agents based on genomic profiling in a second treatment line setting (N = 34). Frequently observed genomic alterations occurred in the FGFR2 (N = 21), IDH1 (N = 7) and BRCA2 (N = 6) genes. Median progression-free survival (PFS) following first-, second- and third-line systemic therapy and overall survival (OS) were 8.44 (95% CI, 7.49-12.78), 5.65 (95% CI, 3.71-7.13), 5.55 (2.79-12.58) and 29.01 (24.21-42.91) months, respectively. CCA subtype and FGFR/BRCA molecular aberration status were not associated with PFS or OS. However, a prior CCA-related surgical history was predictive of OS (p = 0.02). Stratification by best overall response to second-line targeted agents demonstrated an association with PFS (p = 0.002) and OS (p = 0.02). Duration of treatment with second-line targeted therapy was associated with OS (p < 0.001). CONCLUSIONS: Patients receiving targeted therapeutics achieved promising outcomes, especially those attaining a favourable treatment response and those receiving targeted agents for longer periods. Liquid biopsies can reliably provide information on extended molecular profiling to aid patient selection for personalised therapies.

Cholangiocarcinoma or IgG4-associated cholangitis: how feasible it is to avoid unnecessary surgical interventions?

OBJECTIVE: To evaluate the experience of a tertiary hepatopancreaticobiliary (HPB) center in the diagnostic approach and management of patients with suspicion of cholangiocarcinoma (CCa), focusing on excluding patients with IgG4-associated cholangitis (IAC) from unnecessary major surgical interventions. METHODS: Between January 2008 and September 2010, a total number of 152 patients with suspicion of CCa underwent evaluation through a HPB multidisciplinary team meeting. Patients without tissue diagnosis were managed surgically or medically on the basis of probable presence of IAC as underlying pathology. Serology, immunostaining, and imaging were reviewed and analyzed according to the HISORt (Histology, Imaging, Serology, Other organ involvement, Response to therapy) criteria for IAC. RESULTS: Tissue diagnosis during the diagnostic workup was achieved in 104 patients (68%), whereas the remaining 48 were classified as "highly suspicious for CCa" (n = 35) or as "probable IAC" (n = 13). Among 16 "highly suspicious for CCa" patients who underwent surgery, pathology revealed 2 patients harboring IAC (n = 1) and a benign chronic inflammatory biliary stricture (n = 1), respectively. Among the 13 patients with primarily medical management as "probable IAC," final diagnosis was CCa (n = 3) and IAC (n = 9), while 1 patient had no proven diagnosis. The accuracy of serum IgG4 for diagnosis of IAC reached 60%. Sensitivity and specificity of immunostaining for IAC in biopsy specimens were 56% and 89%, respectively. Imaging features suggesting IAC yielded sensitivity, specificity, and accuracy of 75%, 89%, and 83%, respectively. Initial imaging was revised at the referral institute in 75% of IAC patients (P = 0.009), while an isolated stricture (P = 0.038), a biliary mass (P = 0.006), and normal pancreas on computed tomography (P = 0.01) were statistically significant parameters for distinguishing between CCa and IAC. The mean time for establishing a diagnosis of IAC was 12.4 months (range: 2.5-32 months). CONCLUSIONS: Differential diagnosis between CCa and IAC mandates high index of suspicion and low threshold for referral in high volume institutes. The delayed establishment of diagnosis particularly for CCa needs to be balanced versus avoiding unnecessary surgery for IAC. Imaging features may be most helpful for optimal management.

Endoscopic retrograde pancreatography criteria to diagnose autoimmune pancreatitis: an international multicentre study.

BACKGROUND: Characteristic pancreatic duct changes on endoscopic retrograde pancreatography (ERP) have been described in autoimmune pancreatitis (AIP). The performance characteristics of ERP to diagnose AIP were determined. METHODS: The study was done in two phases. In phase I, 21 physicians from four centres in Asia, Europe and the USA, unaware of the clinical data or diagnoses, reviewed 40 preselected ERPs of patients with AIP (n=20), chronic pancreatitis (n=10) and pancreatic cancer (n=10). Physicians noted the presence or absence of key pancreatographic features and ranked the diagnostic possibilities. For phase II, a teaching module was created based on features found most useful in the diagnosis of AIP by the four best performing physicians in phase I. After a washout period of 3 months, all physicians reviewed the teaching module and reanalysed the same set of ERPs, unaware of their performance in phase I. RESULTS: In phase I the sensitivity, specificity and interobserver agreement of ERP alone to diagnose AIP were 44, 92 and 0.23, respectively. The four key features of AIP identified in phase I were (i) long (>1/3 the length of the pancreatic duct) stricture; (ii) lack of upstream dilatation from the stricture (<5 mm); (iii) multiple strictures; and (iv) side branches arising from a strictured segment. In phase II the sensitivity (71%) of ERP significantly improved (p<0.05) without a significant decline in specificity (83%) (p>0.05); the interobserver agreement was fair (0.40). CONCLUSIONS: The ability to diagnose AIP based on ERP features alone is limited but can be improved with knowledge of some key features.

Race, Racism, and Cardiovascular Health: Applying a Social Determinants of Health Framework to Racial/Ethnic Disparities in Cardiovascular Disease.

Health care in the United States has seen many great innovations and successes in the past decades. However, to this day, the color of a person's skin determines-to a considerable degree-his/her prospects of wellness; risk of disease, and death; and the quality of care received. Disparities in cardiovascular disease (CVD)-the leading cause of morbidity and mortality globally-are one of the starkest reminders of social injustices, and racial inequities, which continue to plague our society. People of color-including Black, Hispanic, American Indian, Asian, and others-experience varying degrees of social disadvantage that puts these groups at increased risk of CVD and poor disease outcomes, including mortality. Racial/ethnic disparities in CVD, while documented extensively, have not been examined from a broad, upstream, social determinants of health lens. In this review, we apply a comprehensive social determinants of health framework to better understand how structural racism increases individual and cumulative social determinants of health burden for historically underserved racial and ethnic groups, and increases their risk of CVD. We analyze the link between race, racism, and CVD, including major pathways and structural barriers to cardiovascular health, using 5 distinct social determinants of health domains: economic stability; neighborhood and physical environment; education; community and social context; and healthcare system. We conclude with a set of research and policy recommendations to inform future work in the field, and move a step closer to health equity.

Endoscopic retrograde cholangiography does not reliably distinguish IgG4-associated cholangitis from primary sclerosing cholangitis or cholangiocarcinoma.

BACKGROUND & AIMS: Distinction of immunoglobulin G4-associated cholangitis (IAC) from primary sclerosing cholangitis (PSC) or cholangiocarcinoma is challenging. We aimed to assess the performance characteristics of endoscopic retrograde cholangiography (ERC) for the diagnosis of IAC. METHODS: Seventeen physicians from centers in the United States, Japan, and the United Kingdom, unaware of clinical data, reviewed 40 preselected ERCs of patients with IAC (n = 20), PSC (n = 10), and cholangiocarcinoma (n = 10). The performance characteristics of ERC for IAC diagnosis as well as the κ statistic for intraobserver and interobserver agreement were calculated. RESULTS: The overall specificity, sensitivity, and interobserver agreement for the diagnosis of IAC were 88%, 45%, and 0.18, respectively. Reviewer origin, specialty, or years of experience had no statistically significant effect on reporting success. The overall intraobserver agreement was fair (0.74). The operating characteristics of different ERC features for the diagnosis of IAC were poor. CONCLUSIONS: Despite high specificity of ERC for diagnosing IAC, sensitivity is poor, suggesting that many patients with IAC may be misdiagnosed with PSC or cholangiocarcinoma. Additional diagnostic strategies are likely to be vital in distinguishing these diseases.

Duodenal web in an adult presenting with acute pancreatitis and acquired megaduodenum: report of a case.

Duodenal webs are a cause of intestinal atresia in infants and surgical repair is the established treatment of choice. However, the late-onset postoperative complications have not been adequately studied, especially in adults who have undergone surgical interventions as infants. This report describes the case of a 65-year-old female patient who presented with consecutive episodes of acute pancreatitis and a history of duodenal atresia repaired by a gastrojejunostomy in early infancy. Imaging studies revealed the presence of megaduodenum and suggested the possibility of impacted stones at the ampulla of Vater. An intact duodenal web at the level of papilla of Vater was revealed during surgery. Excision of the web, tapering of the duodenum and duodenojejunostomy was performed to relieve the obstructive cause of pancreatitis and to restore the intestinal continuity. At the 1-year follow-up, the patient is free of any symptoms, has no diet restrictions, and has increased her body weight as well.

Community and Social Context: An Important Social Determinant of Cardiovascular Disease.

Disease prevention frameworks and clinical practice guidelines in the United States (US) have traditionally ignored upstream social determinants of health (SDOH), which are critical for reducing disparities in cardiovascular disease (CVD)-the leading cause of death in the US. Existing evidence demonstrates a protective effect of social support, social cohesion, and community engagement on overall health and wellbeing. Increasing community and social support is a major objective of the Healthy People 2030 initiative, with special provisions for vulnerable populations. However, to date, existing evidence of the association between community and social context (CSC)-an integral SDOH domain-and CVD has not been reviewed extensively. In particular, the individual and cumulative impact of CSC on CVD risk and the pathways linking CSC to cardiovascular outcomes are not well understood. In this review, we critically appraise current knowledge of the association between CSC and CVD, describe potential pathways linking CSC to CVD, and identify opportunities for evidence-based policy and practice interventions to improve CVD outcomes.

Presentation and management of post-treatment relapse in autoimmune pancreatitis/immunoglobulin G4-associated cholangitis.

BACKGROUND & AIMS: Autoimmune pancreatitis (AIP) is a multisystem disorder that often has extrapancreatic manifestations such as immunoglobulin G4-associated cholangitis (IAC). Patients respond rapidly to steroids but can relapse after therapy. We assessed the clinical management of relapse in a group of patients with AIP/IAC. METHODS: We performed a prospective study of patients diagnosed with AIP from 2004-2007 who received steroids. Treatment outcome was defined clinically, radiologically, and biochemically as response to steroids, remission after steroids, failure to wean steroids, and relapse. Steroids +/- azathioprine (AZA) were used to treat patients who failed, relapsed, or could not be weaned from steroids. RESULTS: Twenty-eight patients with AIP were studied; 23 (82%) had IAC. All patients responded within 6 weeks to prednisolone therapy. Twenty-three patients achieved remission after a median of 5 months of treatment (range, 1.5-17 months), whereas 5 patients (18%) could not be weaned because of a disease flare. Of the patients who achieved remission, 8 of 23 (35%) subsequently relapsed. Overall, 13 of 23 patients (57%) with AIP/IAC relapsed, compared with 0 of the 5 with isolated AIP (P = .04, Fisher exact test). Steroids were increased/restarted in all patients who relapsed; 10 also received AZA. Remission was achieved and maintained in 7 patients; they remain on AZA monotherapy at a median of 14 months (range, 1-27 months). CONCLUSIONS: Relapse or failure to wean steroids occurred in 46% of patients with AIP. Patients with IAC are at particularly high risk of relapse. AZA appears to be effective in patients with post-treatment relapse or who cannot be weaned from steroids. To view this article's video abstract, go to the AGA's YouTube Channel.

Quantitative pancreatic MRI: a pathology-based review.

MRI plays an important role in the clinical management of pancreatic disorders and interpretation is reliant on qualitative assessment of anatomy. Conventional sequences capturing pancreatic structure can however be adapted to yield quantitative measures which provide more diagnostic information, with a view to increasing diagnostic accuracy, improving patient stratification, providing robust non-invasive outcome measures for therapeutic trials and ultimately personalizing patient care. In this review, we evaluate the use of established techniques such as secretin-enhanced MR cholangiopancreatography, diffusion-weighted imaging, T 1, T 2* and fat fraction mapping, but also more experimental methods such as MR elastography and arterial spin labelling, and their application to the assessment of diffuse pancreatic disease (including chronic, acute and autoimmune pancreatitis/IgG4 disease, metabolic disease and iron deposition disorders) and cystic/solid focal pancreatic masses. Finally, we explore some of the broader challenges to their implementation and future directions in this promising area.

Pancreatoscopy-Directed Electrohydraulic Lithotripsy for Pancreatic Ductal Stones in Painful Chronic Pancreatitis Using SpyGlass.

OBJECTIVE: Painful chronic pancreatitis is often associated with main duct obstruction due to stones. Approaches to management are challenging, including surgery, extracorporeal shock wave lithotripsy, or endoscopic approaches. Here, we report our experience of pancreatoscopy + electrohydraulic lithotripsy (EHL) for pancreatic duct (PD) stones using SpyGlass. METHODS: We retrospectively audited the use of SpyGlass (Legacy and DS) + EHL. Indication, procedural details, and clinical outcomes were assessed. RESULTS: A total of 118 SpyGlass + EHL procedures for stones were performed, of which 8 (7%) for pancreatic stones, in 6 patients (3 female; mean [standard deviation] age, 45 [7] years). All patients had painful chronic pancreatitis, with radiological evidence of a dilated PD, and main duct stone disease. Surgical options had been considered in all cases. Stone fragmentation and PD decompression were achieved in 83% (n = 5) without complications. Two patients required 2 EHL procedures to achieve clearance. In 1 patient with failed clearance, pancreatoscopy revealed a stone in the adjacent parenchyma and not in PD. All patients with successful EHL had pain relief/marked improvement at clinical review (mean [standard deviation] follow-up, 2.7 [1.1] years). CONCLUSIONS: Pancreatoscopy + EHL may have a valuable role in treating obstructing PD stones, possibly avoiding the need for surgery in some patients.

The role of multidisciplinary meetings for benign pancreatobiliary diseases: a tertiary centre experience.

Multidisciplinary meetings are central to the management of chronic and complex diseases and they have become widely established across the modern healthcare. Patients with pancreatobiliary diseases can often present with complex clinical dilemmas, which fall out with the scope of current guidelines. Therefore, these patients require a personalised management approach discussed in a multidisciplinary meeting.

Acute exacerbation of dysphagia due to foreign body impaction in an oesophageal stricture due to eosinophilic esophagitis.

A 43-year woman with a 4-year history of swallowing difficulty for solids presented with absolute dysphagia, which was only slightly relieved by intravenous relaxant given in the emergency department. Barium swallow showed a smooth polypoid filling defect in the mid-oesophagus, with a hold-up at this level. Gastroscopy showed a narrowed ringed oesophagus with an impacted foreign body. This was extracted with a basket, with relief of the dysphagia. Oesophageal biopsies confirmed the diagnosis of eosinophilic esophagitis.

Adrenal cancer in neurofibromatosis type 1: case report and DNA analysis.

UNLABELLED: Adrenal cortical carcinoma (ACC) has previously only been reported in eight patients with type 1 neurofibromatosis (NF1). There has not been any clear evidence of a causal association between NF1 gene mutations and adrenocortical malignancy development. We report the case of a 49-year-old female, with no family history of endocrinopathy, who was diagnosed with ACC on the background of NF1, due to a novel germline frame shift mutation (c.5452_5453delAT) in exon 37 of the NF1 gene. A left adrenal mass was detected by ultrasound and characterised by contrast computerised tomography (CT) scan. Biochemical tests showed mild hypercortisolism and androgen excess. A 24-h urinary steroid profile and (18)flouro deoxy glucose PET suggested ACC. An open adrenalectomy was performed and histology confirmed ACC. This is the first reported case with DNA analysis, which demonstrated the loss of heterozygosity (LOH) at the NF1 locus in the adrenal cancer, supporting the hypothesis of an involvement of the NF1 gene in the pathogenesis of ACC. LOH analysis of the tumour suggests that the loss of neurofibromin in the adrenal cells may lead to tumour formation. LEARNING POINTS: ACC is rare but should be considered in a patient with NF1 and adrenal mass when plasma metanephrines are normal.Urinary steroid metabolites and PET/CT are helpful in supporting evidence for ACC.The LOH at the NF1 region of the adrenal tumour supports the role of loss of neurofibromin in the development of ACC.

Radical surgery in the presence of biliary metallic stents: revising the palliative scenario.

BACKGROUND: The application of endobiliary self-expandable metallic stents (SEMS) is considered the palliative treatment of choice in patients with biliary obstruction in the setting of inoperable malignancies. In the presence of SEMS, however, radical surgery is the only curative option when the resectability status is revised in case of malignancies or for overcoming complications arising from their application in benign conditions that masquerade as inoperable tumours. The aim of our study was to report our surgical experience with patients who underwent an operation due to revision of the initial palliative approach, whilst they had already been treated with biliary SEMS exceeding the hilar bifurcation. METHODS: Three patients with hilar cholangiocarcinoma that was considered inoperable and one patient with IgG4 autoimmune cholangio-pancreatopathy mimicking pancreatic cancer underwent radical resections in the presence of biliary SEMS. RESULTS: After a detailed preoperative workup, two right trisectionectomies, one left extended hepatectomy and a radical extrahepatic biliary resection were performed. All cases demanded resection and reconstruction of the portal vein. R0 resection was achieved in all the malignant cases. Two patients required multiple biliodigestive anastomoses entailing three and seven bile ducts respectively. There was one perioperative death due to postoperative portal vein and hepatic artery thrombosis, whilst two patients developed grade III complications. At follow-up, one patient died at 13 months due to disease recurrence, whilst the remaining two are free of disease or symptoms at 21 and 12 months, respectively. CONCLUSIONS: Revising the initial palliative approach and operating in the setting of biliary metallic stents is extremely demanding and carries significant mortality and morbidity. Radical resection is the only option for offering cure in such complex cases, and this should only be attempted in advanced hepatopancreaticobiliary centres with active involvement in liver transplantation.