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Background: Intrabiliary rupture (IBR) is one of the most common complications of hepatic hydatid cysts (HHC),and the surgical treatment of major intrabiliary rupture (MIBR) leads to serious morbidity and mortality. This study aimed to evaluate the efficacy of endoscopic definitive treatment of these patients., Methods: This study included 38 patients who underwent ERCP for MIBR between 2004 and 2022. Endoscopic sphincterotomy (ES) was performed, followed by evacuation of hydatid contents from the main bile ducts and cyst cavity. A nasobiliary drainage (NBD) catheter was placed inside the cyst cavity in 15 patients, while biliary stenting (BS) was performed in 23 patients. Demographic data, cyst characteristics, hospital stay, complications, treatment success, and recurrence were analyzed., Results: The mean age of patients was 46.5 years, with 12 female and 26 male. All patients had WHO-type-3B hydatid cysts with an average diameter of 7.9 cm. NBD was continued for definitive purposes in 13 of the 15 patients who underwent NBD, and full cure was achieved in 13 patients. Among the 23 patients who underwent biliary stenting (BS), 16 (69.5%) required a second ERCP due to jaundice or cholangitis, and 6 (26%) underwent percutaneous drainage due to hydatid cavity infection. The average length of hospital stay was 16.13 days in the BS group, while it was 8.3 days in the NBD group.The average follow-up period was 23 months. All patients achieved complete recovery., Conclusions: The endoscopic approach is an effective and safe method for the treatment of MIBR, and can be considered as a definitive treatment option in centers with adequate resources and experienced endoscopists., Competing Interests: Declarations. Disclosures: Drs. Alpen Yahya Gümüşoğlu, Hamit Ahmet Kabuli, Mahmut Said Değerli, Burak Atar, Osman Köneş, Ali Kocataş, Nilsu Damla Bingül and Kemal Dolay have no conflicts of interest or financial ties to disclose. Ethical approval: The study protocol was approved by the Ethics Committee of Bakirkoy Dr. Sadi Konuk Training and Research Hospital, (2024–04-24) and the written informed consent was obtained from each patient., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Several diseases originate from bile duct pathology. Despite studies on these diseases, certain etiologies of some of them still cannot be concluded. The most common disease of the bile duct in newborns is biliary atresia, whose prognosis varies according to the age of surgical correction. Other diseases such as Alagille syndrome, inspissated bile duct syndrome, and choledochal cysts are also time-sensitive because they can cause severe liver damage due to obstruction. The majority of these diseases present with cholestatic jaundice in the newborn or infant period, which is quite difficult to differentiate regarding clinical acumen and initial investigations. Intraoperative cholangiography is potentially necessary to make an accurate diagnosis, and further treatment will be performed synchronously or planned as findings suggest. This article provides a concise review of bile duct diseases, with interesting cases., Competing Interests: Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article., (©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.)

Background: To evaluate the efficacy of percutaneous and endoscopic therapeutic interventions for biliary strictures and leaks following LT in children., Methods: Retrospective analysis of 49 consecutive pediatric liver transplant recipients (27 girls, 22 boys, mean age at transplant 3.9 years) treated at our institution from 1989 to 2019 for biliary leak and/or biliary stricture was performed. Minimally invasive approach was considered clinically successful if it resulted in patency of the narrowed biliary segment and/or correction of the biliary leak., Results: Forty-two patients had a stricture at the biliary anastomosis; seven had a biliary leak. After an average 13.8 years of follow-up, long-term clinical success with minimally invasive treatment (no surgery or re-transplant) was achieved for 24 children (57%) with biliary stricture and 4 (57%) with biliary leaks. Eight patients required re-transplant; however, only one was due to failure of both percutaneous and surgical management. For biliary strictures, failure of non-surgical management was associated with younger age at stricture diagnosis (p < .02)., Conclusions: Percutaneous and endoscopic management of biliary strictures and leaks after LT in children is associated with a durable result in >50% of children., (© 2021 Wiley Periodicals LLC.)

Liver and biliary disease complicates pregnancy in varying degrees of severity to the mother and fetus, and anesthesiologists may be asked to assist in caring for these patients before, during, and after birth of the fetus. Therefore, it is important to be familiar with how different liver diseases impact the pregnancy state. In addition, knowing symptoms, signs, and laboratory markers in the context of a pregnant patient will lead to faster diagnosis and treatment of such patients. This review article discusses changes in physiology of parturients, patients with liver disease, and parturients with liver disease. Next, general treatment of parturients with acute and chronic liver dysfunction is presented. The article progresses to specific liver diseases with treatments as they relate to pregnancy. And finally, important aspects to consider when anesthetizing parturients with liver disease are discussed., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2021 International Anesthesia Research Society.)

Background: Bile duct injury (BDI) is a severe complication following cholecystectomy. Early recognition and treatment of BDI has been shown to reduce costs and improve patients' quality of life. The aim of this study was to assess the effect and cost-effectiveness of routine versus selective intraoperative cholangiography (IOC) in cholecystectomy., Methods: A systematic review and meta-analysis, combined with a health economic model analysis in the Swedish setting, was performed. Costs per quality-adjusted life-year (QALY) for routine versus selective IOC during cholecystectomy for different scenarios were calculated., Results: In this meta-analysis, eight studies with more than 2 million patients subjected to cholecystectomy and 9000 BDIs were included. The rate of BDI was estimated to 0.36 per cent when IOC was performed routinely, compared with to 0.53 per cent when used selectively, indicating an increased risk for BDI of 43 per cent when IOC was used selectively (odds ratio 1.43, 95 per cent c.i. 1.22 to 1.67). The model analysis estimated that seven injuries were avoided annually by routine IOC in Sweden, a population of 10 million. Over a 10-year period, 33 QALYs would be gained at an approximate net cost of €808 000 , at a cost per QALY of about €24 900., Conclusion: Routine IOC during cholecystectomy reduces the risk of BDI compared with the selective strategy and is a potentially cost-effective intervention., (© The Author(s) 2021. Published by Oxford University Press on behalf of BJS Society Ltd.)

Organoid technology holds great promise for regenerative medicine but has not yet been applied to humans. We address this challenge using cholangiocyte organoids in the context of cholangiopathies, which represent a key reason for liver transplantation. Using single-cell RNA sequencing, we show that primary human cholangiocytes display transcriptional diversity that is lost in organoid culture. However, cholangiocyte organoids remain plastic and resume their in vivo signatures when transplanted back in the biliary tree. We then utilize a model of cell engraftment in human livers undergoing ex vivo normothermic perfusion to demonstrate that this property allows extrahepatic organoids to repair human intrahepatic ducts after transplantation. Our results provide proof of principle that cholangiocyte organoids can be used to repair human biliary epithelium., (Copyright © 2020 The Authors, some rights reserved; exclusive licensee American Association for the Advancement of Science. No claim to original U.S. Government Works.)

Cholangiopathies are chronic, progressive diseases of the biliary tree, and can be either acquired or genetic. The primary target is the cholangiocyte (CC), the cell type lining the bile duct that is responsible for bile modification and transport. Despite advances in our understanding and diagnosis of these diseases in recent years, there are no proven therapeutic treatments for the majority of the cholangiopathies, and liver transplantation is the only life-extending treatment option for patients with end-stage cholestatic liver disease. One potential therapeutic strategy is to facilitate endogenous repair of the biliary system, which may alleviate intrahepatic cholestasis caused by these diseases. During biliary injury, hepatocytes (HC) are known to alter their phenotype and acquire CC-like features, a process known as cellular reprogramming. This brief review discusses the potential ways in which reprogrammed HC may contribute to biliary repair, thereby restoring bile flow and reducing the severity of cholangiopathies. Some of these include modifying bile to reduce toxicity, serving as a source of de novo CC to repair the biliary epithelium, or creating new channels to facilitate bile flow.

Background and Aims: Temporary single, fully covered self-expanding metal stent (FCSEMS) placement for benign biliary strictures (BBSs) associated with chronic pancreatitis (CP) may require fewer interventions than endotherapy with multiple plastic stents and may carry less morbidity than biliary diversion surgery. This study aimed to assess long-term outcomes in CP-associated BBSs after FCSEMS placement and removal., Methods: In this open-label, multinational, prospective study, subjects with CP and a BBS treated with FCSEMS placement with scheduled removal at 10 to 12 months were followed for 5 years after FCSEMS indwell. Kaplan-Meier analyses assessed BBS resolution and cumulative probability of freedom from recurrent stent placement to 5 years after FCSEMS indwell., Results: One hundred eighteen patients were eligible for FCSEMS removal. At a median of 58 months (interquartile range, 44-64) post-FCSEMS indwell, the probability of remaining stent-free was 61.6% (95% confidence interval [CI], 52.5%-70.7%). In 94 patients whose BBSs resolved at the end of FCSEMS indwell, the probability of remaining stent-free 5 years later was 77.4% (95% CI, 68.4%-86.4%). Serious stent-related adverse events occurred in 27 of 118 patients (22.9%); all resolved with medical therapy or repeated endoscopy. Multivariate analysis identified severe CP (hazard ratio, 2.4; 95% CI, 1.0-5.6; P = .046) and longer stricture length (hazard ratio, 1.2; 95% CI, 1.0-1.4; P = .022) as predictors of stricture recurrence., Conclusion: In patients with symptomatic BBSs secondary to CP, 5 years after placement of a single FCSEMS intended for 10 to 12 months indwell, more than 60% remained asymptomatic and stent-free with an acceptable safety profile. Temporary placement of a single FCSEMS may be considered as first-line treatment for patients with CP and BBSs. (Clinical trial registration number: NCT01014390.)., (Copyright © 2020. Published by Elsevier Inc.)

Background: With the popularity of laparoscopic cholecystectomy, common bile duct injury has been reported more frequently. There is no perfect method for repairing porcine biliary segmental defects., Methods: After the decellularization of human arterial blood vessels, the cells were cultured with GFP + (carry green fluorescent protein) porcine bile duct epithelial cells. The growth and proliferation of porcine bile duct epithelial cells on the human acellular arterial matrix (HAAM) were observed by hematoxylin-eosin (HE) staining, electron microscopy, and immunofluorescence. Then, the recellularized human acellular arterial matrix (RHAAM) was used to repair biliary segmental defects in the pig. The feasibility of it was detected by magnetic resonance cholangiopancreatography, liver function and blood routine changes, HE staining, immunofluorescence, real-time quantitative PCR (RT-qPCR), and western blot., Results: After 4 weeks (w) of co-culture of HAAM and GFP + porcine bile duct epithelial cells, GFP + porcine bile duct epithelial cells grew stably, proliferated, and fused on HAAM. Bile was successfully drained into the duodenum without bile leakage or biliary obstruction. Immunofluorescence detection showed that GFP-positive bile duct cells could still be detected after GFP-containing bile duct cells were implanted into the acellular arterial matrix for 8 w. The implanted bile duct cells can successfully resist bile invasion and protect the acellular arterial matrix until the newborn bile duct is formed., Conclusion: The RHAAM can be used to repair biliary segmental defects in pigs, which provides a new idea for the clinical treatment of common bile duct injury., Competing Interests: Conflicts of interestThe authors have no financial conflicts of interest., (© The Korean Tissue Engineering and Regenerative Medicine Society 2019.)

Cholangioscopy provides an opportunity to directly visualize the bile duct for diagnosing biliary lesions and for therapeutic interventions. Although there are different cholangioscopy techniques available, single-operator cholangioscopy has gained widespread acceptance as the standard technique for interventions in the biliary system because of its ease of use and widespread availability. Single-operator cholangioscopy can be used for both diagnostic and therapeutic indications in the biliary tract. Diagnostic cholangioscopy is used for direct evaluation of indeterminate bile duct strictures with biopsies, diagnosing filling defects in the bile ducts observed during endoscopic retrograde cholangiography (ERC) imaging, preoperative mapping of the precise location and extension of tumors of the biliary tract, and diagnosis of intraductal neoplasms. Therapeutic cholangioscopy is used for visually guided treatment of biliary stones that have failed extraction with conventional ERC techniques, residual or impacted stones by using intraductal lithotripsy, ablation of biliary tumors and for facilitation of guidewire advancement into selective intrahepatic ducts for adequate biliary drainage. In this review, we will focus on advances in the single-operator cholangioscopy techniques in the diagnosis and management of biliary disorders., (© 2019 Japan Gastroenterological Endoscopy Society.)

Different topical products have been tested in liver resection to get a control of bleeding. This study compares the effectiveness and complications between two haemostatic agents Tachosil versus Hemopatch. A cohort study including patients who underwent liver resection since November 2014 to April 2016 was conducted. The study was performed in a single institution. Demographic variables, intraoperative characteristics and postoperative complications were analysed. A total of 92 patients (50 in Tachosil group and 42 in Hemopatch group) were included. No differences were found in patients who required intraoperative (Tachosil 6 (12%) vs Hemopatch 2 (4.8%); p = 0.28) and postoperative (Tachosil 4 (8%) vs Hemopatch 3 (7.1%); p = 0.87) blood transfusion. There were no differences in length of hospital stay (Tachosil 7.02 ± 4.1 days vs Hemopatch 7.63 ± 9.1; p = 0.67). Overall postoperative complications were similar between both patches (Tachosil 21 (42%) vs Hemopatch 14 (33%); p = 0.48). No differences were found in specific complications, however Hemopatch showed a higher incidence of intraabdominal abscess 5 (11.9%) and vs 0 (0%) p = 0.01.In this study no differences have been found between Hemopatch and Tachosil in the effectiveness and overall postoperative complication after liver resection, although Hemopatch shows a higher incidence of intraabdominal abscess. Further studies are necessary to confirm these findings.

Background: Balloon enteroscopy (BE) can be used for endoscopic retrograde cholangiography (ERC) to treat biliary strictures in patients with surgically altered anatomies. However, biliary strictures, including bilioenteric anastomotic strictures, are often very severe and dilation catheters cannot pass through them. The Soehendra stent retriever (SSR) is like a screw drill and can be useful for dilating severe strictures, but the utility of SSR during BE-assisted ERC (BE-ERC) is unclear. This study aimed to examine the efficacy and safety of a dilation technique using the SSR during BE-ERC., Methods: Between 2014 and 2018, 28 patients with surgically altered gastrointestinal anatomies and severe biliary strictures underwent BE-ERC, and the SSR was used for the dilation procedures. We evaluated the technical success, therapeutic success, and adverse event rates associated with SSR dilation., Results: The technical success rate was 93% (26/28). The procedures undertaken on two patients with non-anastomotic strictures failed technically because the SSR was not long enough to reach the strictures. The therapeutic success rate was 96% (25/26) for the patients whose procedures were technically successful. The adverse event rate was 7% (2/28), and the adverse events were mild and improved with conservative management. No bleeding or duct perforations occurred., Conclusions: Although the indications for using the SSR in patients with non-anastomotic strictures should be considered based on the distance between the tip of the scope and the stricture's location, SSR dilation may be a useful option during BE-ERC if a biliary stricture is very severe.

Background: Bilomas are defined collections of bile fluids mainly caused by iatrogenic injuries of the bile duct system. Owing to the infrequency of this disease, studies addressing bilomas are rare., Methods: By using an endoscopic database, this retrospective study identified 32 patients with bilomas treated between 2004 to 2015, in order to analyse aetiology, clinical presentation, spectrum of pathogens, and resolution rate of bilomas., Results: 65.6% of the study population (21/32) developed bilomas after surgery and 21.9% (7/32) after endoscopic retrograde cholangiography (ERC). Icterus, fever, and abdominal pain were the leading symptoms. 93.9% (46/49) of microbiological bile cultures revealed a positive microbiology. The predominant microorganisms were the group of Enterobacteriaceae (43.0%, 52/121), followed by Enterococcus spp. (32.2%, 39/121), and Candida spp. (9.1%, 11/121). Multiresistant bacteria like Enterobacteriaceae were isolated from one quarter of all patients. Single or multimodal treatment resulted in an overall complication rate of 4.8% (9/188). Clinical follow-up analysis showed a complete resolution rate of 78.3% for interventional therapy and 80% in the non-interventional group., Conclusions: Pathogen spectrum of bilomas mainly comprises the group of Enterobacteriacae and Enterococcus spp., with a high proportion of multiresistant bacteria. Different interventional approaches are available for biloma drainage, which seem to be safe and effective for most patients., Trial Registration: German Clinical Trials Register DRKS00015208 , retrospectively registered.

Competing Interests: None, (© Georg Thieme Verlag KG Stuttgart · New York.)

Paracoccidioidomycosis is a systemic granulomatous disease caused by the dimorphic fungus Paracoccidioides brasiliensis and is restricted to Latin America. It normally affects lungs, skin and lymph nodes. Abdominal organs are usually not involved. In rare cases paracoccidioidomycosis may simulate neoplasm. Herein we describe our experience with four cases of paracoccidioidomycosis mimicking cholangiocarcinoma. To the best of our knowledge, this is the largest case series on this subject produced in English. Paracoccidioidomycosis must be considered as a differential diagnosis of cholangiocarcinoma, especially in individuals who come from endemic areas., (Copyright © 2019 Fundación Clínica Médica Sur, A.C. Published by Elsevier España, S.L.U. All rights reserved.)

Inspissated bile syndrome (IBS) is a relatively rare condition. Many treatment options are available, including medication, surgery, and surgical interventions, such as insertion of cholecystostomy drain, endoscopic retrograde cholangiopancreatography, internal biliary drainage, and percutaneous transhepatic biliary drainage (PTBD). We herein report the first case of IBS that was successfully treated with PTBD in a two-month-old infant in Korea. PTBD was initiated on postnatal day 72. On postnatal day 105, we confirmed complete improvement and successfully removed the catheters. This report suggests that PTBD is a viable and safe treatment option for obstructive jaundice in very young infants., Competing Interests: The authors have no financial conflicts of interest., (© Copyright: Yonsei University College of Medicine 2018.)

Background and Aims: ERCP is challenging in patients with Roux-en-Y gastric bypass (RYGB) anatomy. EUS-guided gastrogastrostomy (GG) creation is a promising novel technique to access the excluded stomach to facilitate conventional ERCP. We aimed to compare procedural outcomes and adverse events (AEs) between EUS-guided GG-assisted ERCP (EUS-GG-ERCP) and enteroscopy-assisted ERCP (e-ERCP) in patients with RYGB., Methods: Patients with RYGB anatomy who underwent EUS-GG-ERCP or e-ERCP between 2014 and 2016 at 5 tertiary centers were included. The primary outcome was technical success of ERCP, defined as successful cannulation of the selected duct with successful intervention as intended. Secondary outcomes included total procedural time (in the EUS-GG group, total procedural time included EUS-GG creation plus ERCP procedure time), length of hospital stay, and rate/severity of AEs graded according to the American Society for Gastrointestinal Endoscopy lexicon., Results: A total of 60 patients (mean age, 57.2 ± 13.2; 75% women) were included, of whom 30 (50%) underwent EUS-GG-ERCP and 30 (50%) underwent e-ERCP (double-balloon enteroscope ERCP, 19; single-balloon enteroscope ERCP, 11). The technical success rate was significantly higher in the EUS-GG-ERCP versus the e-ERCP group (100% vs 60.0%, P < .001). Total procedure time was significantly shorter in patients who underwent EUS-GG-ERCP (49.8 minutes vs 90.7 minutes, P < .001). Postprocedure median length of hospitalization was shorter in the EUS-GG group (1 vs 10.5 days, P = .02). Rate of AEs was similar in both groups (10% vs 6.7%, P = 1)., Conclusions: EUS-GG-ERCP may be superior to e-ERCP in patients with RYGB anatomy in terms of a higher technical success and shorter procedural times and offers a similar safety profile., (Copyright © 2018 American Society for Gastrointestinal Endoscopy. Published by Elsevier Inc. All rights reserved.)

Liver diseases constitute an important medical problem, and a number of these diseases, termed cholangiopathies, affect the biliary system of the liver. In this review, we describe the current understanding of the causes of cholangiopathies, which can be genetic, viral or environmental, and the few treatment options that are currently available beyond liver transplantation. We then discuss recent rapid progress in a number of areas relevant for decoding the disease mechanisms for cholangiopathies. This includes novel data from analysis of transgenic mouse models and organoid systems, and we outline how this information can be used for disease modeling and potential development of novel therapy concepts. We also describe recent advances in genomic and transcriptomic analyses and the importance of such studies for improving diagnosis and determining whether certain cholangiopathies should be viewed as distinct or overlapping disease entities., (Copyright © 2018 The Authors. Published by Elsevier B.V. All rights reserved.)

We studied the etiological spectrum, clinicolaboratory and histological profile, and outcome of infants and children under 18 years of age presenting between December 2010 and May 2016 with histological evidence of paucity of intralobular bile ducts (PILBD, bile ducts to portal tract ratio < 0.6) Post-transplant PILBD was excluded. Of 632 pediatric liver biopsies screened, 70 had PILBD-44 were infants. PILBD was classified histologically into destructive (n = 50) and non-destructive PILBD (n = 20). Presentations were jaundice (98%), organomegaly (94%), pale stools (50%), and pruritus (43%). Infants had more cholestasis but less fibrosis on histology. Overall, 29 required liver transplantation (LT) for portal hypertension (n = 26), decompensation (n = 25), growth failure (n = 20), intractable pruritus (n = 5), and recurrent cholangitis (n = 2). Destructive PILBD has an odds for poor outcome (decompensation or need for LT within 1 year) of 1.53 (95% CI = 1.15-2.04). On binary logistic regression analysis, poor outcome was related to advanced fibrosis on liver biopsy [Exp (B) = 5.46, 95% CI = 1.56-19.04]., Conclusion: PILBD was present in 11% of pediatric liver biopsies and has a varied etiological spectrum. Destructive PILBD has poor outcome. Need for LT is guided by the presence of advanced fibrosis. What is Known: • Natural history of syndromic ductal paucity (Alagille syndrome) is complex. • Duct loss is commonly seen with late presentation of biliary atresia. What is New: • The study classifies the etiological spectrum of ductal paucity histologically into destructive and non-destructive. • Destructive duct loss carries poor prognosis regardless of the etiology of liver disease with subsequent need for liver transplantation.

Background: The increased incidence of bile duct injuries (BDIs) after the adoption of laparoscopic cholecystectomy has been well documented. However, the longitudinal impact of bile leaks and BDIs on survival and healthcare use have not been studied adequately. The aims of this analysis were to determine the incidence, long-term outcomes, and costs of bile leaks and ductal injuries in a large population., Study Design: The California Office of Statewide Health Planning and Development database was queried from 2005 to 2014. Bile leaks, BDIs, and their management strategy were defined. Survival was calculated by Kaplan-Meier failure estimates with multivariable regression and propensity analyses. Cost analyses used inflation adjustments and institution-specific cost-to-charge ratios., Results: Of 711,454 cholecystecomies, bile leaks occurred in 3,551 patients (0.50%) and were managed almost exclusively by endoscopists. Bile duct injuries occurred in 1,584 patients (0.22%) with 84% managed surgically. Patients with a bile leak were more likely to die at 1 year (2.4% vs 1.4%; odds ratio 1.85; p < 0.001). Similarly, BDI patients had an increased 1-year mortality (7.2% vs 1.3%; odds ratio 2.04; p < 0.0001). Survival of BDI patients was better with an operative approach (odds ratio 0.19; p < 0.001) when compared with endoscopic management. Operatively managed BDIs were also associated with fewer emergency department visits and readmissions, as well as lower cumulative costs at 1 year ($60,539 vs $118,245; p < 0.001)., Conclusions: The 0.22% incidence of BDIs observed in California is lower than reported in the first decade after the introduction of laparoscopic cholecystectomy. Bile leaks are 2.3 times more common than BDIs. Patients with a bile leak or BDI have diminished survival. Surgical repair of a BDI leads to enhanced survival and reduced cumulative cost compared with endoscopic management., (Copyright © 2018 American College of Surgeons. Published by Elsevier Inc. All rights reserved.)

Purpose: The purpose of this study was to retrospectively assess the potential of percutaneous transhepatic biliary drainage (PTBD) in patients with nondilated bile ducts (NDBD) using a transgallbladder opacification of the bile ducts., Patients and Methods: Eight patients with NDBD (7 men, 1 women; median age, 65 years; Q1-Q3, 35-69 years; range, 22-77 years) who underwent PTBD after opacification of the bile ducts through the gallbladder were evaluated. Opacification of NDBD was performed using a retrograde injection of contrast material through the gallbladder. The opacified peripheral NDBD was punctured percutaneously and a drainage catheter was introduced under fluoroscopy guidance. The success and safety of the procedure were assessed., Results: PTBD could be achieved in 6/8 patients (75%) and no significant complications were observed. The biliary tree opacification was attempted but could not be achieved due to biliary sludge that obstructed the cystic duct in 2/8 patients (25%). Two minor complications in two different patients were observed consisting of transient hemobilia and chills., Conclusion: Opacification of the bile ducts using a transgallbladder approach appears to be a safe and successful procedure for PTBD in patients with NDBD., (Copyright © 2017 Editions françaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.)

In the last 25years, a number of animal models, mainly rodents, have been generated with the goal to mimic cholestatic liver injuries and, thus, to provide in vivo tools to investigate the mechanisms of biliary repair and, eventually, to test the efficacy of innovative treatments. Despite fundamental limitations applying to these models, such as the distinct immune system and the different metabolism regulating liver homeostasis in rodents when compared to humans, multiple approaches, such as surgery (bile duct ligation), chemical-induced (3,5-diethoxycarbonyl-1,4-dihydrocollidine, DDC, α-naphthylisothiocyanate, ANIT), viral infections (Rhesus rotavirustype A, RRV-A), and genetic manipulation (Mdr2, Cftr, Pkd1, Pkd2, Prkcsh, Sec63, Pkhd1) have been developed. Overall, they have led to a range of liver phenotypes recapitulating the main features of biliary injury and altered bile acid metabolisms, such as ductular reaction, peribiliary inflammation and fibrosis, obstructive cholestasis and biliary dysgenesis. Although with a limited translability to the human setting, these mouse models have provided us with the ability to probe over time the fundamental mechanisms promoting cholestatic disease progression. Moreover, recent studies from genetically engineered mice have unveiled 'core' pathways that make the cholangiocyte a pivotal player in liver repair. In this review, we will highlight the main phenotypic features, the more interesting peculiarities and the different drawbacks of these mouse models. This article is part of a Special Issue entitled: Cholangiocytes in Health and Disease edited by Jesus Banales, Marco Marzioni, Nicholas LaRusso and Peter Jansen., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Objectives: Endoscopic retrograde cholangiopancreatography is used to manage biliary pathology in pediatric patients. Plastic biliary stents have been used in this population for obstructive lesions and bile leaks; however, they are sometimes not effective due to migration, occlusion, or ineffective sealing. Fully covered self-expanding metal stents (FCSEMS) have larger diameters making them more suitable for some situations. Their use in pediatrics has, however, not been defined. The aim of the present study is to describe our experience with FCSEMS at our institution., Methods: We present a series of all patients who underwent FCSEMS placement at Children's Hospital Colorado including 3 adolescents and 1 young adult with complex medical needs., Results: Patient age range was 12 to 24 years and the weight ranged between 36 and 75 kg. All patients underwent previous endoscopic retrograde cholangiopancreatography and 1 or more rounds of plastic stenting without adequate clinical response before consideration of FCSEMS placement. Indications included recalcitrant biliary anastomotic stricture after liver transplant, persistent bile leak after needle perforation, recurrent obstructive choledocholithiasis after cholecystectomy, and malignant biliary stricture. Sizes of FCSEMS depended on patient bile duct size and biliary pathology. Dwell time was 6 to 8 weeks. Three patients had resolution of biliary pathology after FCSEMS therapy. One patient had distal migration of FCSEMS necessitating repeat stenting. There were no adverse events from FCSEMS placement or removal., Conclusions: FCSEMS therapy should be considered in appropriate pediatric patients when plastic biliary stents are not effective. Further studies are needed to evaluate the safety and efficacy of FCSEMS in the pediatric age group.

Diseases of the liver and biliary tree have been described with significant frequency among patients with human immunodeficiency virus (HIV), and its advanced state, acquired immunodeficiency syndrome (AIDS). Through a variety of mechanisms, HIV/AIDS has been shown to affect the hepatic parenchyma and biliary tree, leading to liver inflammation and biliary strictures. One of the potential hepatobiliary complications of this viral infection is AIDS cholangiopathy, a syndrome of biliary obstruction and liver damage due to infection-related strictures of the biliary tract. AIDS cholangiopathy is highly associated with opportunistic infections and advanced immunosuppression in AIDS patients, and due to the increased availability of highly active antiretroviral therapy, is now primarily seen in instances of poor access to anti-retroviral therapy and medication non-compliance. While current published literature describes well the clinical, biochemical, and endoscopic management of AIDS-related cholangiopathy, information on its epidemiology, natural history, and pathology are not as well defined. The objective of this review is to summarize the available literature on AIDS cholangiopathy, emphasizing its epidemiology, course of disease, and determinants, while also revealing an updated approach for its evaluation and management., Competing Interests: Conflict-of-interest statement: No potential conflicts of interest.

Purpose: The goal of this study was to analyze the outcomes of percutaneous transhepatic management of benign biliary disorders in pediatric patients., Materials and Methods: This study included 11 pediatric patients who underwent percutaneous transhepatic biliary interventional procedures between September 2007 and December 2016. There were 3 males and 8 females with a mean age of 9.6±5.4 (SD)years (range: 2-17years). Technical details, complications and outcome of the procedures were evaluated., Results: The underlying pathologies were bile duct stones (n=2), bile leakage (n=4), choledochal cyst (n=3) and benign biliary stricture (n=2). The therapeutic interventional procedures were as follows; percutaneous stone removal in patients with bile duct stones, external biliary drainage in patients with choledochal cyst, bile diversion by internal-external percutaneous biliary drainage (IE-PBD) in patients with bile leakage, plastic stent placement, IE-PBD with balloon dilatation in patients with benign biliary stricture. The procedures were successful in all patients technically and clinically. One patient experienced intermittent fever., Conclusion: Percutaneous transhepatic biliary interventional procedure is an effective and safe approach for the treatment of pediatric patients with bile duct stones, bile leakage, symptomatic choledochal cyst and benign biliary stricture when endoscopic procedure is unavailable or fails., (Copyright © 2017 Éditions françaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.)

Background: Hepatic artery thrombosis after liver transplantation is a devastating complication associated with ischemic cholangiopathy that can occur even after successful revascularization. This study explores long-term outcomes after hepatic artery thrombosis in adult liver transplantation recipients, focusing on the probability, risk factors, and resolution of ischemic cholangiopathy., Methods: A retrospective chart review of 1,783 consecutive adult liver transplantations performed between 1995 and 2014 identified 44 cases of hepatic artery thrombosis (2.6%); 10 patients underwent immediate retransplantation, and 34 patients received nontransplant treatments, involving revascularization (n = 19) or expectant nonrevascularization management (n = 15)., Results: The 1-year graft survival after nontransplant treatment was favorable (82%); however, 16 of the 34 patients who received a nontransplant treatment developed ischemic cholangiopathy and required long-term biliary intervention. A Cox regression model showed that increased serum transaminase and bilirubin levels at the time of hepatic artery thrombosis diagnosis, but not nonrevascularization treatment versus revascularization, were risk factors for the development of ischemic cholangiopathy. Ischemic cholangiopathy in revascularized grafts was less extensive with a greater likelihood of resolution within 5-years than that in nonrevascularized grafts (100% vs 17%). Most liver abscesses without signs of liver failure also were reversible. Salvage retransplantation after a nontransplant treatment was performed in 8 patients with a 1-year survival rate equivalent to immediate retransplantation (88% vs 80%)., Conclusion: Selective nontransplant treatments for hepatic artery thrombosis resulted in favorable graft survival. Biliary intervention can resolve liver abscess and ischemic cholangiopathy that developed in revascularized grafts in the long-term; salvage retransplantation should be considered for ischemic cholangiopathy in nonrevascularized grafts because of a poor chance of resolution., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: The total cost of bile duct injuries (BDIs) in an unselected national cohort of patients undergoing cholecystectomy are unknown. The aim was to evaluate costs associated with treatment of cholecystectomy-related BDIs and to calculate cost effectiveness of routine vs. on-demand intraoperative cholangiography (IOC)., Methods: Data from Swedish patients suffering a BDI during a 5 year period were analysed. Questionnaires to investigate loss-of-production and health status (EQ-5D) were distributed to patients who suffered a BDI during cholecystectomy and who underwent uneventful cholecystectomy (matched control group). Costs per quality-adjusted-life-year (QALY) gained by intraoperative diagnosis were estimated for two strategies: routine versus on-demand IOC during cholecystectomy., Results: Intraoperative diagnosis, immediate intraoperative repair, and minor BDI were all associated with reduced direct treatment costs compared to postoperative diagnosis, delayed repair, and major BDI (all p < 0.001). No difference was noted in loss-of-production for minor versus major BDIs or between different treatment strategies. The cost per QALY gained with routine intraoperative cholangiography (ICER-incremental cost-effectiveness ratio) to achieve intraoperative diagnosis was €50,000., Conclusions: Intraoperative detection and immediate intraoperative repair is the superior strategy with less than half the cost and superior functional patient outcomes than postoperative diagnosis and delayed repair. The cost per QALY gained (ICER) using routine IOC was considered reasonable., (Copyright © 2017 International Hepato-Pancreato-Biliary Association Inc. Published by Elsevier Ltd. All rights reserved.)

Common bile duct (CBD) injury is the most serious complication of laparoscopic cholecystectomy. Recently, laparoscopic techniques have been used in the management of postoperative bile leak and CBD injury; this literature has not been reviewed. We reviewed the literature on CBD injury, the approach to its diagnosis and management, and reports of laparoscopic management techniques. We combined this review with our experience in laparoscopic methods to highlight diagnostic and therapeutic options. Laparoscopic techniques can be used to prevent, diagnose and treat CBD injuries. Intraoperatively, CBD injury can be prevented in the case of short cystic duct with the use of a loop ligature or transfixing suture, and it can be diagnosed using intraoperative cholangiography or other visualization techniques. When CBD injury is suspected postoperatively, repeat laparoscopy can be used to control sepsis with abdominal washout; as a diagnostic tool to guide management; and, in some settings, as a therapeutic tool for suturing small duct leaks, drain insertion and postoperative endoscopic retrograde cholangiopancreatography with sphincterotomy. Definitive laparoscopic repair is possible when certain criteria are met. Open surgery should be considered when the CBD is small, the injury occurred more than 72 hours previously, injury or anatomy are complex, port positioning is awkward for repair, or local experience is limited with laparoscopic management. There is an emerging role for laparoscopy in the management of CBD injuries. More case reports and series are needed to show the safety and efficacy of this technique, encourage its wider adoption, and allow outcomes assessment on a larger scale.

Background: We aimed to analyze the benefits of adding ultrasound (US) guidance to the standard fluoroscopically assisted percutaneous transhepatic biliary drainage (F-PTBD). We also performed a systematic literature review of success and complication rates of US-PTBD in a wide field of indications., Methods: We evaluated a total of 81 US-PTBDs carried out in our institution, 74% of which were part of the management of malignancy. In addition, we compared our results with those of a total of 5,272 procedures (3,779 F-PTBD and 1,493 US-PTBD) reported in the literature., Results: US-PTBD was technically successful in 94% of attempts with a mean of 2.2 needle passes. Procedural success was achieved in 86% of cases. There were no procedure-related deaths or severe complications. Minor complications were catheter dislodgement (15%) as well as one case each of a porto-biliary fistula, hematoma, and biloma. A systematic review of the literature also showed that US-PTBD has a similar technical success rate to F-PTBD but lower median rates of severe early complications (0% versus 8%) and procedural death (0% versus 1%)., Conclusions: Given our results and our review of the literature, US-PTBD is as effective as F-PTBD and has significantly lower complication rates. US-PTBD should be preferred to F-PTBD. © 2017 Wiley Periodicals, Inc. J Clin Ultrasound 45:400-407, 2017., (© 2017 Wiley Periodicals, Inc.)

Introduction: The effect of endoscopic sphincterotomy prior to endoscopic biliary stenting to prevent post-endoscopic retrograde cholangiopancreatography pancreatitis remains to be fully elucidated. The aim of this study is to prospectively evaluate the non-inferiority of non-endoscopic sphincterotomy prior to stenting for naïve major duodenal papilla compared with endoscopic sphincterotomy prior to stenting in patients with biliary stricture., Methods and Analysis: We designed a multicentre randomised controlled trial, for which we will recruit 370 patients with biliary stricture requiring endoscopic biliary stenting from 26 high-volume institutions in Japan. Patients will be randomly allocated to the endoscopic sphincterotomy group or the non-endoscopic sphincterotomy group. The main outcome measure is the incidence of pancreatitis within 2 days of initial transpapillary biliary drainage. Data will be analysed on completion of the study. We will calculate the 95% confidence intervals (CIs) of the incidence of pancreatitis in each group and analyse weather the difference in both groups with 95% CIs is within the non-inferiority margin (6%) using the Wald method., Ethics and Dissemination: This study has been approved by the institutional review board of Hokkaido University Hospital (IRB: 016-0181). Results will be submitted for presentation at an international medical conference and published in a peer-reviewed journal., Trial Registration Number: The University Hospital Medical Information Network ID: UMIN000025727 Pre-results., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Aim: To evaluate cholangioscopy in addition to endoscopic retrograde cholangiopancreatography (ERCP) for management of biliary complications after liver transplantation (LT)., Methods: Twenty-six LT recipients with duct-to-duct biliary reconstruction who underwent ERCP for suspected biliary complications between April and December 2016 at the university hospital of Muenster were consecutively enrolled in this observational study. After evaluating bile ducts using fluoroscopy, cholangioscopy using a modern digital single-operator cholangioscopy system (SpyGlass DS™) was performed during the same procedure with patients under conscious sedation. All patients received peri-interventional antibiotic prophylaxis and bile was collected during the intervention for microbial analysis and for antibiotic susceptibility testing., Results: Thirty-three biliary complications were found in a total of 22 patients, whereas four patients showed normal bile ducts. Anastomotic strictures were evident in 14 (53.8%) patients, non-anastomotic strictures in seven (26.9%), biliary cast in three (11.5%), and stones in six (23.1%). A benefit of cholangioscopy was seen in 12 (46.2%) patients. In four of them, cholangioscopy was crucial for selective guidewire placement prior to planned intervention. In six patients, biliary cast and/or stones failed to be diagnosed by ERCP and were only detectable through cholangioscopy. In one case, a bile duct ulcer due to fungal infection was diagnosed by cholangioscopy. In another case, signs of bile duct inflammation caused by acute cholangitis were evident. One patient developed post-interventional cholangitis. No further procedure-related complications occurred. Thirty-seven isolates were found in bile. Sixteen of these were gram-positive (43.2%), 12 (32.4%) were gram-negative bacteria, and Candida species accounted for 24.3% of all isolated microorganisms. Interestingly, only 48.6% of specimens were sensitive to prophylactic antibiotics., Conclusion: Single-operator cholangioscopy can provide important diagnostic information, helping endoscopists to plan and perform interventional procedures in LT-related biliary complications., Competing Interests: Conflict-of-interest statement: Hüsing-Kabar A received funding from the Dean’s Office of the Medical Faculty of the Westfälische-Wilhelms-University of Muenster. None of the other authors have conflicts of interest related to the subject of this manuscript.

Introduction: Although patient and graft survival rate has increased in recent years, biliary complications after liver transplantation are associated with significant morbidity and mortality., Methods and Materials: We reviewed the database of 1930 patients who underwent deceased donor liver transplantation between 2000 and 2013. The patients had abnormal results in liver tests, as well as fever and jaundice. Abdominal sonography was performed, and if complication was identified, the patient underwent an interventional procedure by endoscopic retrograde cholangiopancreatography or percutaneous transhepatic cholangiography. If the complication was not resolved by the mentioned procedures, exploration of common bile duct and Roux-en-Y choledochojejunostomy or revision of Roux-en-Y choledochojejunostomy was done., Results: Our study group comprised 105 patients including 66 (63%) men and 39 (37.1%) women with a mean age of 36.7 ± 12.5 years (range: 15-66 years). Among 1930 patients, 105 (5.4%) cases presented with biliary complication after liver transplantation, of which 97 (5%) and 8 (0.4%) cases presented with biliary stricture and bile leak/biloma, respectively., Conclusion: In our study, most patients with biliary complications after liver transplantation responded to interventional procedures, with 37.1% requiring surgical exploration.

Aim: To present clinical characteristics, diagnosis and treatment strategies in elderly patients with biliary diseases., Methods: A total of 289 elderly patients with biliary diseases were enrolled in this study. The clinical data relating to these patients were collected in our hospital from June 2013 to May 2016. Patient age, disease type, coexisting diseases, laboratory examinations, surgical methods, postoperative complications and therapeutic outcomes were analyzed., Results: The average age of the 289 patients with biliary diseases was 73.9 ± 8.5 years (range, 60-102 years). One hundred and thirty-one patients (45.3%) had one of 10 different biliary diseases, such as gallbladder stones, common bile duct stones, and cholangiocarcinoma. The remaining patients (54.7%) had two types of biliary diseases. One hundred and seventy-nine patients underwent 9 different surgical treatments, including pancreaticoduodenectomy, radical resection of hilar cholangiocarcinoma and laparoscopic cholecystectomy. Ten postoperative complications occurred with an incidence of 39.3% (68/173), and hypopotassemia showed the highest incidence (33.8%, 23/68). One hundred and sixteen patients underwent non-surgical treatments, including anti-infection, symptomatic and supportive treatments. The cure rate was 97.1% (168/173) in the surgical group and 87.1% (101/116) in the non-surgical group. The difference between these two groups was statistically significant ( χ 2 = 17.227, P < 0.05)., Conclusion: Active treatment of coexisting diseases, management of indications and surgical opportunities, appropriate selection of surgical procedures, improvements in perioperative therapy, and timely management of postoperative complications are key factors in enhancing therapeutic efficacy in elderly patients with biliary diseases., Competing Interests: Conflict-of-interest statement: The authors declare that there are no conflicts of interest related to this study.

Background: Pancreaticobiliary maljunction (PBM) refers to the union of the pancreatic and biliary ducts outside of the duodenal wall. Patients are at increased risk of bile duct and gallbladder cancer, likely secondary to pancreatic juice refluxing into the biliary tree, and it is recommended that they undergo biliary diversion., Methods: This is a case series of all patients in our institution with PBM and bilioenteric anastomosis who presented with symptomatic pancreatic duct stones in a disconnected bile duct. IRB approval was obtained prior to the initiation of the study., Results: We describe eight cases of this finding. All patients underwent ERCP, with stones successfully removed from the disconnected bile duct in seven patients and from the pancreatic duct in one patient., Conclusion: This novel finding has not been described in the medical literature, and may become more prevalent as more patients with PBM undergo bilioenteric anastomosis., (Copyright © 2016 IAP and EPC. Published by Elsevier B.V. All rights reserved.)

Portal biliopathy (PB) is defined as the presence of biliary abnormalities in patients with non-cirrhotic/non-neoplastic extrahepatic portal vein obstruction (EHPVO) and portal cavernoma (PC). The pathogenesis of PB is due to ab extrinseco compression of bile ducts by PC and/or to ischemic damage secondary to an altered biliary vascularization in EHPVO and PC. Although asymptomatic biliary abnormalities can be frequently seen by magnetic resonance cholangiopancreatography in patients with PC (77%-100%), only a part of these (5%-38%) are symptomatic. Clinical presentation includes jaundice, cholangitis, cholecystitis, abdominal pain, and cholelithiasis. In this subset of patients is required a specific treatment. Different therapeutic approaches aimed to diminish portal hypertension and treat biliary strictures are available. In order to decompress PC, surgical porto-systemic shunt or transjugular intrahepatic porto-systemic shunt can be performed, and treatment on the biliary stenosis includes endoscopic (Endoscopic retrograde cholangiopancreatography with endoscopic sphincterotomy, balloon dilation, stone extraction, stent placement) and surgical (bilioenteric anastomosis, cholecystectomy) approaches. Definitive treatment of PB often requires multiple and combined interventions both on vascular and biliary system. Liver transplantation can be considered in patients with secondary biliary cirrhosis, recurrent cholangitis or unsuccessful control of portal hypertension., Competing Interests: Conflict-of-interest statement: Authors declare no conflict of interests for this article.

The Food and Drug Administration (FDA) is classifying the metallic biliary stent system for benign strictures into class II (special controls). The special controls that will apply to the device are identified in this order and will be part of the codified language for the metallic biliary stent system for benign strictures' classification. The Agency is classifying the device into class II (special controls) in order to provide a reasonable assurance of safety and effectiveness of the device.

Thrombosis of the splenoportal axis not associated with liver cirrhosis or neoplasms is a rare disease whose prevalence ranges from 0.7 to 3.7 per 100,000 inhabitants. However, this entity is the second most common cause of portal hypertension. Prothrombotic factors are present as an underlying cause in up to 70% of patients and local factors in 10-50%. The coexistence of several etiological factors is frequent. Clinical presentation may be acute or chronic (portal cavernomatosis). The acute phase can present as abdominal pain, nausea, vomiting, fever, rectorrhagia, intestinal congestion, and ischemia. In this phase, early initiation of anticoagulation is essential to achieve portal vein recanalization and thus improve patient prognosis. In the chronic phase, symptoms are due to portal hypertension syndrome. In this phase, the aim of treatment is to treat or prevent the complications of portal hypertension. Anticoagulation is reserved to patients with a proven underlying thrombophilic factor., (Copyright © 2016 Elsevier España, S.L.U. y AEEH y AEG. All rights reserved.)

Endoscopic retrograde cholangiopancreatography (ERCP) requires deep biliary cannulation. When deep biliary cannulation is failed, the endoscopic ultrasonography rendezvous technique (EUS-RV) is a useful salvage method. From the previous 15 articles that included 382 EUS-RV cases, the overall success rate of EUS-RV is 81 % with a complication rate of 10 %. In EUS-RV, the bile duct is punctured under EUS guidance and a guidewire is advanced into the duodenum via the papilla. The EUS scope is then switched to a duodenoscope and inserted into the bile duct over the guidewire exiting the papilla, or the guidewire is grasped with forceps and passed through the working channel; the catheter can then be inserted through the papilla over the wire. There are three puncture routes for EUS-RV: transgastric puncture of the intrahepatic bile duct (IHBD), transduodenal puncture of the extrahepatic bile duct (EHBD) via the proximal duodenum (D1), and transduodenal puncture of the EHBD via the second portion of the duodenum (D2). The puncture route for each patient should be selected based on the patient condition. GW selection for EUS-RV is critical, a hydrophilic GW is useful for this procedure. Although EUS-RV is now performed relatively routinely in a few high-volume centers, procedure standardization and the development of exclusive devices for EUS-RV are still underway. The development of exclusive devices for EUS-RV and prospective comparative studies with other salvage methods are needed to truly evaluate the procedure's usefulness and safety.