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Objectives Adenocarcinomas of the biliary tract frequently present diagnostic challenges because of their histologic overlap with benign and preinvasive lesions. The molecular profiles of biliary adenocarcinomas vary by anatomical location. Variations in IDH1/2, common in intrahepatic cholangiocarcinoma, can lead to defective production of 5-hydroxymethylcytosine (5-hmC). Limited ancillary studies are available for biliary adenocarcinomas, and loss of 5-hmC staining could serve as a helpful ancillary diagnostic tool for biliary tract malignancies. Methods We evaluated 93 cases—20 benign biliary lesions, 15 preinvasive biliary neoplasms, 46 invasive biliary carcinomas, and 12 pancreatic adenocarcinomas—for 5-hmC staining. Preoperative biopsies from 16 cases of biliary carcinoma were also stained. Sixteen nonneoplastic/reactive bile duct biopsies served as controls. Results Loss of 5-hmC was seen in 41 of 46 (89.1%) biliary malignancies vs 0 of 20 benign tumors (P  < .001), for a sensitivity and specificity of 89.1% and 100%, respectively. Intrahepatic cholangiocarcinoma showed loss of 5-hmC in 11 of 13 (84.6%) cases, similar to the 30 of 33 (90.9%) cases in other biliary adenocarcinomas (P  = .61). Similarly, 5-hmC loss was more frequent in distal bile duct adenocarcinomas than in pancreatic ductal adenocarcinomas, at 15 of 17 (88.2%) vs 4 of 12 (33.3%), respectively (P  = .0045). There was no difference in the frequency of 5-hmC loss in patients that received neoadjuvant therapy vs those who did not (90.9% vs 88.6%, P   >.99). 5-hmC immunohistochemistry in preoperative biopsies was concordant with the resection specimen in 81.3% (13/16) of cases. Conclusions Loss of 5-hmC is not unique to intrahepatic cholangiocarcinoma among biliary carcinomas, but is a useful diagnostic marker differentiating malignancies of the biliary tree from benign mimics. [ABSTRACT FROM AUTHOR]

Cholangiopathies include a group of chronic progressive disorders, affecting the cholangiocytes, the epithelial cells that line the biliary tree, leading to liver parenchymal fibrosis and eventually end-stage liver disease necessitating transplantation. Experimental modeling of these multifactorial cholestatic diseases faces challenges due to the lack of adequate experimental in vitro and in vivo models. A novel approach employs three-dimensional organoid systems that offer several advantages for modeling disease and testing drug response in vitro. Organoids mimic intercellular communication, replicate the architecture of organs, and maintain the cell's original phenotype. Cholangiocyte organoids provide an in vitro model to study the pathogenesis and pharmacotherapeutic treatment of cholangiopathies and show great promise for regenerative therapies. In particular, patient-derived organoids allow personalized medicine approaches and the study of individual disease characteristics. This review highlights the significance of cholangiocyte organoid models in advancing our understanding of cholangiopathies and driving advancements in regenerative medicine strategies. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Hydatid cysts, primarily found in the liver (70%), are caused by parasitic infections and can lead to severe complications such as cyst rupture. This case report describes a unique instance of a hydatid liver cyst occupying the right lobe with a communicating part with the biliary tree that ruptured showing a concurrent superinfection. Hydatid cysts are a clinical pathology resulting from parasitic infections. They may occur in different organs of the body. However, these are mostly found in the liver (70%). This can cause significant complications including cyst rupture. Several case reports have described various hydatid cyst ruptures; however, only a few have reported an intra‐biliary hydatid cyst rupture. A 24‐year‐old male patient presented with right upper quadrant pain, jaundice, dark urine, and pale stool. Imaging studies, including Magnetic resonance cholangiopancreatography (MRCP) and computed tomography (CT), revealed a beavertail liver, cystobiliary communication and intrahepatic biliary tree‐ruptured hydatid cysts. The cyst was in the right liver lobe, which is the most common site for hydatid cysts. Surgical intervention involving laparoscopic de‐roofing and cyst removal resulted in a smooth recovery without complications. Several case reports have described various hydatid cyst ruptures; however, only a few have reported originally placed intra‐biliary hydatid cyst ruptures. This case report describes a unique instance of a hydatid liver cyst occupying the right lobe with a communicating part with the biliary tree that ruptured showing a concurrent superinfection. [ABSTRACT FROM AUTHOR]

Key Clinical Message Hydatid cysts, primarily found in the liver (70%), are caused by parasitic infections and can lead to severe complications such as cyst rupture. This case report describes a unique instance of a hydatid liver cyst occupying the right lobe with a communicating part with the biliary tree that ruptured showing a concurrent superinfection. Abstract Hydatid cysts are a clinical pathology resulting from parasitic infections. They may occur in different organs of the body. However, these are mostly found in the liver (70%). This can cause significant complications including cyst rupture. Several case reports have described various hydatid cyst ruptures; however, only a few have reported an intra‐biliary hydatid cyst rupture. A 24‐year‐old male patient presented with right upper quadrant pain, jaundice, dark urine, and pale stool. Imaging studies, including Magnetic resonance cholangiopancreatography (MRCP) and computed tomography (CT), revealed a beavertail liver, cystobiliary communication and intrahepatic biliary tree‐ruptured hydatid cysts. The cyst was in the right liver lobe, which is the most common site for hydatid cysts. Surgical intervention involving laparoscopic de‐roofing and cyst removal resulted in a smooth recovery without complications. Several case reports have described various hydatid cyst ruptures; however, only a few have reported originally placed intra‐biliary hydatid cyst ruptures. This case report describes a unique instance of a hydatid liver cyst occupying the right lobe with a communicating part with the biliary tree that ruptured showing a concurrent superinfection.

Introduction: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. The most common sites are the head and neck, followed by the genitourinary tract and the extremities. Gallbladder RMS in pediatric patient has rarely been reported in literature. Case presentation: A 5-year-old boy presented to our clinic with right upper quadrant abdominal pain for about 2 weeks. Abdominal ultrasound (US) and contrast-enhanced computed tomography (CT) revealed a 2.7 × 1.8 cm mass located in the neck of gallbladder. Magnetic resonance cholangiopancreatography (MRCP) confirmed the heterogeneous mass within the gallbladder without invasion of the surrounding structures, without gallbladder wall thickening, and without dilation of the biliary tree. Liver function tests, alpha-fetoprotein (AFP), carcinoembryonic agent (CEA), and cancer antigen 19-9 (CA19-9) were within normal values. The patient underwent an uneventful laparoscopic cholecystectomy. The pathology analysis confirmed a botryoid rhabdomyosarcoma (RMS) of the gallbladder with clear margins. No metastases were detected by postoperative positron emission tomography (PET) scan. The patient received 11 postoperative cycles of chemotherapy with vincristine, cyclophosphamide, and actinomycin-D. At one year of follow up he is well and free of disease. Conclusion: Due to its rare occurrence, the preoperative diagnosis of gallbladder RMS is challenging. Gallbladder RMS should be considered in the differential diagnosis of pediatric patients who present a gallbladder mass.

Anatomy has remained an interest of physicians throughout the ages. The biliary tract spans from the liver to the hepatoduodenal mesentery, pancreas, and into the duodenum. Therefore, it is important for not only hepatobiliary surgeons but also general gastrointestinal surgeons, gastroenterologists, radiologists, and pathologists to be familiar with biliary anatomy and its variants. While surgery for hilar cholangiocarcinoma is one of the most challenging procedures, cholecystectomy is one of the most common procedures done from the beginning of surgical training. We hope that by answering the following questions, you will gain a comprehensive understanding of biliary anatomy and a greater appreciation for it. [ABSTRACT FROM AUTHOR]

Abstract Background Biliary tract cancers (BTCs), encompassing cholangiocarcinoma (CCA), gallbladder (GBC), and ampulla of Vater cancers (AVC), are common hepatobiliary cancer after hepatocellular carcinoma with a high mortality rate. As there is no effective chemopreventive agent to prevent BTCs, this study aimed to explore the role of statins on the risk of BTCs. Methods PubMed, Embase, and Cochrane Library from inception until 24 April 2020 were searched according to the Meta‐Analyses of Observational Studies in Epidemiology (MOOSE) guidelines. The adjusted risk ratios (aRRs) of BTCs and individual cancer were pooled using a random‐effects model. Results Eight observational studies (3 cohort and 5 case–control studies) were included with 10,485,231 patients. The median age was 68.0 years (IQR: 67.0–71.5) and 48.3% were male. Statins were associated with a lower risk of all BTCs (aRR: 0.67; 95% CI: 0.51–0.87). The pooled aRR for CCA was 0.60 (95% CI: 0.38–0.94) and GBC was 0.78 (95% CI: 0.68–0.90). There was only one study on AVC with aRR of 0.96 (95% CI: 0.66–1.41). The pooled aRR for lipophilic and hydrophilic statins was 0.78 (95% CI: 0.69–0.88) and 0.70 (95% CI: 0.61–0.80), respectively. The effects were attenuated in studies that adjusted for aspirin and/or non‐steroidal anti‐inflammatory drugs (aRR: 0.80, 95% CI: 0.72–0.89) and metformin (aRR: 0.80, 95% CI: 0.72–0.90). Conclusions Statins, both lipophilic and hydrophobic, were associated with a lower risk of BTCs, particularly CCA and GBC.

Normal hepatobiliary function depends on an adequate bile flow from the liver through the biliary tree to the gallbladder, where bile is stored and concentrated, and from the gallbladder to the duodenum when it is required for the digestive process. Interruption of this secretory function results in partial or complete cholestasis, which is accompanied by important repercussions due to the lack of bile acids in the intestine and their regurgitation from hepatocytes to blood together with potentially toxic compounds that are normally eliminated in bile. The presence of active and selective transporter proteins located at both poles of the plasma membrane of hepatocytes, cholangiocytes, and epithelial cells of the ileal mucosa, together with the ability of hepatocytes to synthesize bile acids from cholesterol, enables the so-called bile acid enterohepatic circulation, which is essential in liver and gastrointestinal tract physiology. The presence in the ducts of the biliary tree of agents reducing their luminal diameter by external compression or space-occupying obstacles, either in the duct wall or its lumen, can result in total or partial obstructive cholestasis. The clinical impact and management of cholestasis are different depending on the intrahepatic or extrahepatic location of the obstacle. Thus, surgical interventions can often be helpful in removing extrahepatic obstructions and restoring normal bile flow to the duodenum. In contrast, hepatocyte or cholangiocyte damage, either global, restricted to subcellular compartments, or more specifically affecting the elements of the canalicular secretory machinery, may result in hepatocellular cholestasis or cholangiopathies. In these cases, bile flow interruption is usually partial and, except for extremely severe cases when liver transplantation is required, these patients often treated with pharmacological agents, such as ursodeoxycholic acid (UDCA) and rifampicin. The present review gathers updated information on the etiopathogenesis and pathophysiological aspects of different types of cholestasis.

Abstract Background Jaundice within the first 1–2 weeks of a neonate’s life will generally self-resolve; however, if it lasts longer than this time frame it warrants further work up. Direct or conjugated hyperbilirubinemia can suggest neonatal cholestasis, which in turn reflects marked reduction in bile secretion and flow. The differential diagnosis for neonatal cholestasis is broad. Neonatal choledocholithiasis is a rare cause of neonatal cholestasis, but should be considered on the differential diagnosis for patients presenting with elevated conjugated bilirubin. Case presentation We describe an infant who presented with neonatal cholestasis. He subsequently underwent work up for biliary atresia, as this is one of the more time-sensitive diagnoses that must be made in neonates with conjugated hyperbilirubinemia. He was ultimately found to have choledocholithiasis on magnetic resonance cholangiopancreatography. He was managed conservatively with optimizing nutrition and ursodeoxycholic acid therapy. Conclusions We found that conservative management, specifically optimizing nutrition and treating with ursodeoxycholic acid, can be a sufficient approach to facilitating resolution of the choledocholithiasis and conjugated hyperbilirubinemia.

The gallbladder is not part of the standard survey of a fetus during obstetrical ultrasound. Yet, some anomalies can be detected. Most are clinically benign, but some are features of or associated with more serious complications. Biliary atresia constitutes the diagnosis with the worst prognosis. Its in utero diagnosis is difficult. Still, markers do exist and should be searched for. Knowledge of the normal appearances, variants and abnormalities of the gallbladder contributes to proper management. Any suspicion should lead to meticulous postnatal workup. Pre- or postnatal magnetic resonance imaging can provide additional information in select cases. [ABSTRACT FROM AUTHOR]

Duodeno-pancreatic and extrahepatic biliary tree injuries are rare in both adult and pediatric trauma patients, and due to their anatomical location, associated injuries are very common. Mortality is primarily related to associated injuries, but morbidity remains high even in isolated injuries. Optimal management of duodeno-bilio-pancreatic injuries is dictated primarily by hemodynamic stability, clinical presentation, and grade of injury. Endoscopic and percutaneous interventions have increased the ability to non-operatively manage these injuries. Late diagnosis and treatment are both associated to increased morbidity and mortality. Sequelae of late presentations of pancreatic injury and complications of severe pancreatic trauma are also increasingly addressed endoscopically and with interventional radiology procedures. However, for moderate and severe extrahepatic biliary and severe duodeno-pancreatic injuries, immediate operative intervention is preferred as associated injuries are frequent and commonly present with hemodynamic instability or peritonitis. The aim of this paper is to present the World Society of Emergency Surgery (WSES) and American Association for the Surgery of Trauma (AAST) duodenal, pancreatic, and extrahepatic biliary tree trauma management guidelines.

Abstract Background Liver transplantation is considered to be the treatment of choice in cases of end-stage liver disease; however, as a major procedure, the operation is fraught with complications. The etiology, symptoms, and diagnostic methods for arterial, portal, and biliary issues are thoroughly discussed. Interventional procedures such as balloon angioplasty and stent placement in the arterial and portal systems, as well as biliary interventional procedures, are described. Results In our study, we reviewed 25 cases of post-living donor transplanted liver, with anastomotic complications including biliary stenosis 40%, hepatic vein stenosis 20%, portal vein stenosis 16%, biliary leakage 16%, and hepatic artery stenosis or pseudo-aneurysm 16%. We had 10 cases of biliary stenosis, 7 of which were successfully treated with the insertion of an internal/external drain, and one case failed. Two patients died. We had four cases of hepatic venous obstruction with successfully implanted stents and a perfect outcome, efficacy, and patency rate of 100%. We also had two cases of hepatic artery stenosis that were perfectly managed by stent placement, with a patency rate of 100%. We came across two cases of hepatic artery pseudo-aneurysm. One case failed due to large sac size, while the other was successful. Finally, in our study, we had a 100% success rate in 5 cases of portal vein stenosis in the early postoperative period. Conclusions Percutaneous IR was effective treatment for hepatic vein occlusion, portal vein stenosis, hepatic artery stenosis, and anastomotic biliary stricture after living donor liver transplantation. The interventional radiology team is now an integral part of the multi-disciplinary care of transplant patients. As new interventional instruments are developed and experience is gained, the outcomes of interventional treatments will continue to improve.

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

Background: Biliary tract cancers (BTCs), encompassing cholangiocarcinoma (CCA), gallbladder (GBC), and ampulla of Vater cancers (AVC), are common hepatobiliary cancer after hepatocellular carcinoma with a high mortality rate. As there is no effective chemopreventive agent to prevent BTCs, this study aimed to explore the role of statins on the risk of BTCs. Methods: PubMed, Embase, and Cochrane Library from inception until 24 April 2020 were searched according to the Meta‐Analyses of Observational Studies in Epidemiology (MOOSE) guidelines. The adjusted risk ratios (aRRs) of BTCs and individual cancer were pooled using a random‐effects model. Results: Eight observational studies (3 cohort and 5 case–control studies) were included with 10,485,231 patients. The median age was 68.0 years (IQR: 67.0–71.5) and 48.3% were male. Statins were associated with a lower risk of all BTCs (aRR: 0.67; 95% CI: 0.51–0.87). The pooled aRR for CCA was 0.60 (95% CI: 0.38–0.94) and GBC was 0.78 (95% CI: 0.68–0.90). There was only one study on AVC with aRR of 0.96 (95% CI: 0.66–1.41). The pooled aRR for lipophilic and hydrophilic statins was 0.78 (95% CI: 0.69–0.88) and 0.70 (95% CI: 0.61–0.80), respectively. The effects were attenuated in studies that adjusted for aspirin and/or non‐steroidal anti‐inflammatory drugs (aRR: 0.80, 95% CI: 0.72–0.89) and metformin (aRR: 0.80, 95% CI: 0.72–0.90). Conclusions: Statins, both lipophilic and hydrophobic, were associated with a lower risk of BTCs, particularly CCA and GBC. [ABSTRACT FROM AUTHOR]

The biliary system is a highly branched tubular network consisting of intrahepatic bile ducts (IHBDs) and extrahepatic bile ducts (EHBDs). IHBDs are derived from hepatic progenitor cells, while EHBDs originate directly from the endoderm through a separate branching morphogenetic process. Traits that are important for cancer are often found to overlap in developmental and other processes. Therefore, it has been suggested that intrahepatic cholangiocarcinomas (iCCAs) and extrahepatic cholangiocarcinomas (eCCAs) have different developmental mechanisms. While much evidence is being gathered on the mechanism of iCCAs, the evidence for eCCA is still very limited. The main reason for this is that there are very few appropriate animal models for eCCA. We can gain important insights from these animal models, particularly genetically engineered mouse models (GEMMs). GEMMs are immunocompetent and mimic human CCA subtypes with a specific mutational pattern, allowing the development of precancerous lesions, that is, biliary intraepithelial neoplasia (BilIN) and intraductal papillary neoplasm of the bile duct (IPNB). This review provides a summary of the pathogenesis and mechanisms of eCCA that can be revealed by GEMMs. Furthermore, we discuss several clinical questions, such as whether BilIN and IPNB really become malignant, whether the peribiliary gland is the origin of eCCAs, and others. [ABSTRACT FROM AUTHOR]

Background: Liver transplantation is considered to be the treatment of choice in cases of end-stage liver disease; however, as a major procedure, the operation is fraught with complications. The etiology, symptoms, and diagnostic methods for arterial, portal, and biliary issues are thoroughly discussed. Interventional procedures such as balloon angioplasty and stent placement in the arterial and portal systems, as well as biliary interventional procedures, are described. Results: In our study, we reviewed 25 cases of post-living donor transplanted liver, with anastomotic complications including biliary stenosis 40%, hepatic vein stenosis 20%, portal vein stenosis 16%, biliary leakage 16%, and hepatic artery stenosis or pseudo-aneurysm 16%. We had 10 cases of biliary stenosis, 7 of which were successfully treated with the insertion of an internal/external drain, and one case failed. Two patients died. We had four cases of hepatic venous obstruction with successfully implanted stents and a perfect outcome, efficacy, and patency rate of 100%. We also had two cases of hepatic artery stenosis that were perfectly managed by stent placement, with a patency rate of 100%. We came across two cases of hepatic artery pseudo-aneurysm. One case failed due to large sac size, while the other was successful. Finally, in our study, we had a 100% success rate in 5 cases of portal vein stenosis in the early postoperative period. Conclusions: Percutaneous IR was effective treatment for hepatic vein occlusion, portal vein stenosis, hepatic artery stenosis, and anastomotic biliary stricture after living donor liver transplantation. The interventional radiology team is now an integral part of the multi-disciplinary care of transplant patients. As new interventional instruments are developed and experience is gained, the outcomes of interventional treatments will continue to improve. [ABSTRACT FROM AUTHOR]

Background: Jaundice within the first 1-2 weeks of a neonate's life will generally self-resolve; however, if it lasts longer than this time frame it warrants further work up. Direct or conjugated hyperbilirubinemia can suggest neonatal cholestasis, which in turn reflects marked reduction in bile secretion and flow. The differential diagnosis for neonatal cholestasis is broad. Neonatal choledocholithiasis is a rare cause of neonatal cholestasis, but should be considered on the differential diagnosis for patients presenting with elevated conjugated bilirubin.Case Presentation: We describe an infant who presented with neonatal cholestasis. He subsequently underwent work up for biliary atresia, as this is one of the more time-sensitive diagnoses that must be made in neonates with conjugated hyperbilirubinemia. He was ultimately found to have choledocholithiasis on magnetic resonance cholangiopancreatography. He was managed conservatively with optimizing nutrition and ursodeoxycholic acid therapy.Conclusions: We found that conservative management, specifically optimizing nutrition and treating with ursodeoxycholic acid, can be a sufficient approach to facilitating resolution of the choledocholithiasis and conjugated hyperbilirubinemia. [ABSTRACT FROM AUTHOR]

Abstract Background The aim of this study was to present a percutaneous transhepatic biliary puncture simulator that can be used without radiation exposure and that reflects the conventional anatomy of the biliary ducts and its vicinity structures. Methods An anatomically based model of the biliary tree was developed using a cord network fixed to a wooden frame. The skin, ribs, intercostal muscles, and right lower lobe pleura were simulated using foam sponge, plastic tubes, a polystyrene foam panel, and an air pad, respectively. For the puncture, we used a 20-G Chiba needle and a wire with distal double arches; these were used to troll a cord, simulating the successful puncture of a bile duct. A camera was also placed above the model to allow the trainees to train eye-hand coordination while viewing the image on a monitor in real time. The simulator was tested with 60 radiology residents to evaluate the confidence and skills transferability of the training model. Results After receiving an introduction of the system and 5 min of training under tutor surveillance, all participants were able to troll a cord of the biliary simulator by themselves in less than 4 min. Only one participant punctured the simulated pleura. The participants’ evaluations showed positive results, with increased user confidence and skills transferability after the training session. Conclusions This proposed simulator can be an effective tool to improve a trainee’s confidence and competence while achieving procedural and non-procedural interventional radiology skills related to the liver. Trial registration Retrospectively registered

Although machine perfusion is a hot topic today, we are just at the beginning of understanding the underlying mechanisms of protection. Recently, the first randomized controlled trial reported a significant reduction of ischemic cholangiopathies after transplantation of livers donated after circulatory death, provided the grafts were treated with an endischemic hypothermic oxygenated perfusion (HOPE). This approach has been known for more than fifty years, and was initially mainly used to preserve kidneys before implantation. Today there is an increasing interest in this and other dynamic preservation technologies and various centers have tested different approaches in clinical trials and cohort studies. Based on this, there is a need for uniform perfusion settings (perfusion route and duration), and the development of general guidelines regarding the duration of cold storage in context of the overall donor risk is also required to better compare various trial results. This article will highlight how cold perfusion protects organs mechanistically, and target such technical challenges with the perfusion setting. Finally, the options for viability testing during hypothermic perfusion will be discussed.

Obstructive cholestasis can lead to significant alterations of the biliary tree depending on the extent and duration of the biliary occlusion. Current experimental studies reported about advanced techniques for corrosion cast and 3D reconstruction (3D-reco) visualizing delicate microvascular structures in animals. We compared these two different techniques for visualization and quantitative assessment of the obstructed murine biliary tree with classical 2D histology. Male mice (n = 36) were allocated to 3 different experiments. In experiments 1 and 2, we injected two different media (Microfil© for 3D-reco, MV; Batson's No.17 for corrosion cast, CC) into the extrahepatic bile duct. In experiment 3 we sampled liver tissue for 2D histology (HE, BrdU). Time points of interest were days 1, 3, 5, 7, 14, and 28 after biliary occlusion. We used different types of software for quantification of the different samples: IMALYTICS Preclinical for 3D scans (MV); NDP.view2 for the digital photography of CC; HistoKat software for 2D histology. We achieved samples in 75% of the animals suitable for evaluation (MV and CC, each with 9/12). Contrasting of terminal bile ducts (4th order of branches) was achieved with either technique. MV permitted a fast 3D-reco of the hierarchy of the biliary tree, including the 3rd and 4th order of branches in almost all samples (8/9 and 6/9). CC enabled focused evaluation of the hierarchy of the biliary tree, including the 4th to 5th order of branches in almost all samples (9/9 and 8/9). In addition, we detected dense meshes of the smallest bile ducts in almost all CC samples (8/9). MV and CC allowed a quantitative assessment of anatomical details of the 3rd and 4th order branches of almost every sample. The 2D histology identified different kinetics and areas of proliferation of hepatocytes and cholangiocytes. Complementary usage of 3D-reco, corrosion casting and 2D histology matched dense meshes of small bile ducts with areas of intensive proliferative activity of cholangiocytes as periportal proliferative areas of 4th and 5th order branches (∼terminal bile ducts and bile ductules) matched with its morphological information the matching assessment of areas with increased proliferative activity (BrdU) and a partial quantification of the characteristics of the 4th order branches of the biliary tree. The 3D-reco and corrosion casting of the murine biliary tree are feasible and provide a straightforward, robust, and reliable (and more economical) procedure for the visualization and quantitative assessment of architectural alterations, in comparative usage with the 2D histology. [ABSTRACT FROM AUTHOR]

Noninvasive visualization of the biliary tree is of paramount importance in any patient who may require a biliary surgical intervention. Most hospitals and imaging centers offer ultrasound, computed tomography (CT), cholescintigraphy (commonly known as hepatobiliary iminodiacetic acid scan), and MRI whenever acute biliary pathology is suspected. PET combined with CT is sometimes utilized to evaluate biliary malignancy and metastatic disease. Unfortunately, these imaging modalities are often ordered incorrectly and have numerous limitations. This article discusses the strengths and weaknesses or each imaging modality and focuses on appropriateness of their utilization in clinical practice., Competing Interests: Disclosure The author has nothing to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Purpose: To compare quantitative biliary measurements obtained with three different magnetic resonance cholangiopancreatography (MRCP) acquisition methods. Methods: This retrospective study was IRB-approved. Patients with combinations of clinically indicated 3D FSE MRCP with sensitivity encoding (SENSE), 3D FSE SENSE MRCP with compressed sensing (CS-FSE; acceleration factor 8), and 3D gradient and spin-echo (GRASE) MRCP, acquired between October 2018 and March 2020, were included. The MRCP + Tuning Threshold algorithm (Perspectum Ltd., Oxford, UK) was used to segment 3D biliary models from MRCP data, with multiple metrics quantified from the models. Single measure, two-way, mixed-effects intra-class correlations, Bland–Altman analyses, and Wilcoxon signed-rank tests were used to compare quantitative measurements. Results: From 160 MRCP datasets (25 3D FSE, 67 3D CS-FSE, 68 3D GRASE) in 69 patients, 48 datasets (7 [28%] 3D FSE, 14 [21%] 3D CS-FSE, 27 [40%] 3D GRASE) failed post-processing due to motion artifacts. The remaining 112 MRCP datasets (18 3D FSE, 53 3D CS-FSE, 41 3D GRASE) from 60 patients were included in the analysis. There was good to excellent agreement between 3D FSE and 3D CS-FSE MRCP for diameter of the left and right hepatic ducts, biliary volume, number and length of ducts, and total length of dilations (ICC: 0.83–0.93). The only metrics that exhibited good agreement between 3D FSE and 3D GRASE MRCP were biliary volume (ICC: 0.75) and total number of dilations (ICC: 0.77). Conclusion: 3D CS-FSE MRCP produces comparable biliary diameter metrics and global duct quantification to 3D FSE MRCP at a significantly reduced acquisition time. [ABSTRACT FROM AUTHOR]

Background Many factors have been incriminated in occurrence of bile duct injuries during laparoscopic cholecystectomy (LC). Misidentification of the anatomy and lack of surgeon's experience seems to be preliminary; however, many techniques were used to avoid such injuries. Aim of the work To asses feasibility of delineation of biliary tree by intracholecystic injection of methylene blue (MB) during LC. Patients and methods A prospective study had been conducted in Ain Shams University Hospitals between January 2019 and January 2020. The study included 60 patients who presented to outpatient department (OPD) with abdominal ultrasound showing chronic calcular cholecystitis and all underwent intracholecystic injection of MB during LC. Results As regards MB delineation of different parts of gall bladder and biliary tract component, gall bladder delineation occurred in all patients (100%), whereas cystic duct delineated in 80% of patients (48/60) and common bile duct (CBD) delineated in 41.7% of patients (25/60) as general and separate assessment of each element alone with no detected cases of bile duct injury. There was significant increase in CBD delineation in patients with single stone and thin-walled gall bladder. Extravasation of dye from the gall bladder into the abdominal cavity occurred and this led to anatomical misidentification and significant prolongation of operative time (extra time was needed for irrigation and suction). Conclusion The technique by which the MB is injected into the lumen of gall bladder to delineate the cystic duct and CBD is feasible, cheap, and performed without any radiation exposure and without using special equipment. By this technique, no cases of biliary injury were detected; however, critical view of safety remains the gold standard surgical technique to prevent bile duct injury. [ABSTRACT FROM AUTHOR]

Injuries to the biliary tree during surgical, endoscopic or invasive radiological diagnostic or therapeutic procedures involving the pancreas, liver or organs of the upper gastrointestinal tract give rise to the need to develop a method for clear discrimination of biliary anatomy from surrounding tissue. Hyperspectral imaging (HSI) is an emerging optical technique in disease diagnosis and image-guided surgery with inherent advantages of being a non-contact, non-invasive, and non-ionizing technique. HSI can produce quantitative diagnostic information about tissue pathology, morphology, and chemical composition. HSI was applied in human liver transplantation and compared to porcine model operations to assess the capability of discriminating biliary anatomy from surrounding biological tissue. Absorbance spectra measured from bile ducts, gall bladder, and liver show a dependence on tissue composition and bile concentration, with agreement between human and porcine datasets. The bile pigment biliverdin and structural proteins collagen and elastin were identified as contributors to the bile duct and gall bladder absorbance spectra.

Abstract Background An aberrant biliary duct of segment 5 (B5) is a rare anomaly of the biliary tract. All anatomical anomalies of the biliary tract are risk factors for bile duct injury during surgery. We report a case of cholelithiasis with an aberrant B5 that was detected during a detailed preoperative imaging examination and treated with laparoscopic cholecystectomy. Case presentation A 69-year-old woman was admitted to the emergency room of our hospital with abdominal pain. She was diagnosed with cholelithiasis, and an aberrant B5 branching off the hepatic duct was suggested during preoperative imaging. Laparoscopic cholecystectomy was performed at our surgical department. There were no intra- or postoperative complications, and the patient was discharged on the fourth day after surgery. Conclusions Laparoscopic cholecystectomy can be safely performed without intra- or postoperative complications in patients with cholelithiasis and an aberrant B5 if it is accurately diagnosed preoperatively.

Background/Aim: Biliary tree and pancreatic duct can appear in different variations whose proper understanding is obligatory for surgeons. Magnetic resonance cholangiopancreatography (MRCP) is considered a safe and accurate tool for evaluating biliary tree and pancreatic duct. Typical anatomy for right hepatic duct (RHD) and left hepatic duct (LHD) is reported as 57% and 63%, respectively. The most common (4-10%) pancreatic anomaly is divisum. In the present study, we evaluated and determined the prevalence of biliary tree and pancreatic duct variations among patients at a university hospital. Materials and Methods: The MRCP records of 370 patients from 2015 to 2017 were obtained for cross-sectional study. Images were retrospectively reviewed for variations by two independent senior radiologists. Demographic data were obtained for all the patients. Huang et al. classification was used for RHD and LHD variations. The cystic duct was reported based on its course and insertion pattern. The pancreatic duct was observed for the presence of divisum, its course, and configuration. Results: Three hundred and twenty-five patients were included in the final study. Most commonly observed variant for RHD were A1 (34.2%) and A2 (32.2%). For LHD, B1 (71.4%) was the most common variant. Cystic duct insertion was commonly seen as right lateral insertion (27.7%). Pancreatic divisum was observed in 0.6% of cases. Nationality, origin, and gender-specific variations were obtained. Conclusion: Variations in biliary anatomy and pancreatic duct are very diverse and extend from the intrahepatic biliary system down to the pancreas. Performing a similar study on a larger population is mandatory to illustrate the range of variations present within the community.

Abstract Duodeno-pancreatic and extrahepatic biliary tree injuries are rare in both adult and pediatric trauma patients, and due to their anatomical location, associated injuries are very common. Mortality is primarily related to associated injuries, but morbidity remains high even in isolated injuries. Optimal management of duodeno-bilio-pancreatic injuries is dictated primarily by hemodynamic stability, clinical presentation, and grade of injury. Endoscopic and percutaneous interventions have increased the ability to non-operatively manage these injuries. Late diagnosis and treatment are both associated to increased morbidity and mortality. Sequelae of late presentations of pancreatic injury and complications of severe pancreatic trauma are also increasingly addressed endoscopically and with interventional radiology procedures. However, for moderate and severe extrahepatic biliary and severe duodeno-pancreatic injuries, immediate operative intervention is preferred as associated injuries are frequent and commonly present with hemodynamic instability or peritonitis. The aim of this paper is to present the World Society of Emergency Surgery (WSES) and American Association for the Surgery of Trauma (AAST) duodenal, pancreatic, and extrahepatic biliary tree trauma management guidelines.

The biliary tree is a network of tubes that connects the liver to the gallbladder, an organ right beneath it. The bile duct is the major tube in the biliary tree. The dilatation of a bile duct is a key indicator for more major problems in the human body, such as stones and tumors, which are frequently caused by the pancreas or the papilla of vater. The detection of bile duct dilatation can be challenging for beginner or untrained medical personnel in many circumstances. Even professionals are unable to detect bile duct dilatation with the naked eye. This research presents a unique vision-based model for biliary tree initial diagnosis. To segment the biliary tree from the Magnetic Resonance Image, the framework used different image processing approaches (MRI). After the image's region of interest was segmented, numerous calculations were performed on it to extract 10 features, including major and minor axes, bile duct area, biliary tree area, compactness, and some textural features (contrast, mean, variance and correlation). This study used a database of images from King Hussein Medical Center in Amman, Jordan, which included 200 MRI images, 100 normal cases, and 100 patients with dilated bile ducts. After the characteristics are extracted, various classifiers are used to determine the patients' condition in terms of their health (normal or dilated). The findings demonstrate that the extracted features perform well with all classifiers in terms of accuracy and area under the curve. This study is unique in that it uses an automated approach to segment the biliary tree from MRI images, as well as scientifically correlating retrieved features with biliary tree status that has never been done before in the literature. [ABSTRACT FROM AUTHOR]

The study of the fractal architecture of various organs and structures expanded the possibilities for determining the ranges of their functioning and structural arrangement, which, as a result, was reflected in the development of new approaches to diagnostics and therapeutic impacts. The architecture of the excretory duct systems, similar to the hemo‐ and lymph‐ circulatory beds and the bronchial tree, is considered fractal. At the same time, information about hitherto unknown structures of the biliary tree continues to appear in the literature. We aimed to study the features of the spatial geometry of the biliary tree and assess the significance of both its fractal and Euclidean characteristics for the development of approaches that facilitate comprehensive description of intrahepatic biliary tract architecture. We investigated the architecture of the biliary trees of six men, seven male canines, and seven male Wistar rats using the corrosion casting method. Corrosion casts were prepared by injecting solidifying latexes into the bile ducts. The preparations were studied using a light stereomicroscope and a scanning electron microscope. Biliary tree branching is of various types. In addition, the correlation between variations in the caliber and length of the bile ducts and their order is not significant. Therefore, the biliary tree should not be considered as a classical fractal and it consists of the main modules, represented by the network of the bile canaliculi (first nonfractal module) and a biliary tree with a fractal branching (second module) that drains the bile canaliculi mesh and the additional modules represented by the mucosal biliary glands (in mammals with the gallbladder) or the periportal biliary plexus (in mammals without a gallbladder) and the aberrant biliary ducts. Such a configuration of the biliary bed should optimally ensure the smooth implementation of the physiological function of the liver, as well as its adaptation to different pathologies accompanied by biliary hypertension. It also might be considered in the diagnosis and assessment of ductular reaction, biliary regeneration, and/or carcinogenesis. [ABSTRACT FROM AUTHOR]

Abstract Background Biliary rhabdomyosarcoma (RMS) is the most common biliary tumor in children. The management of affected patients contains unique challenges because of the rarity of this tumor entity and its critical location at the porta hepatis, which can make achievement of a radical resection very difficult. Methods In a retrospective chart analysis we analysed children suffering from biliary RMS who were registered in three different CWS trials (CWS-96, CWS-2002P, and SoTiSaR registry). Results Seventeen patients (12 female, 5 male) with a median age of 4.3 years were assessed. The median follow-up was 42.2 months (10.7–202.5). The 5-year overall (OS) and event free survival (EFS) rates were 58% (45–71) and 47% (34–50), respectively. Patients > 10 years of age and those with alveolar histology had the worst prognosis (OS 0%). Patients with botryoid histology had an excellent survival (OS 100%) compared to those with non-botryoid histology (OS 38%, 22–54, p = 0.047). Microscopic complete tumor resection was achieved in almost all patients who received initial tumor biopsy followed by chemotherapy and delayed surgery. Conclusion Positive predictive factors for survival of children with biliary RMS are age ≤ 10 years and botryoid tumor histology. Primary surgery with intention of tumor resection should be avoided.

Background: Infestation with Ascaris lumbricoides is worldwide. About one fourth of the world's population is known to be infected by ascariasis. It is endemic in various parts of Bangladesh. Objectives: To study the various types of clinical presentations, socio-demographic background and sonographic patterns of biliary ascariasis. Results: Mean age (mean ± SD) of presentation was 35.3 ± 15.9 years and female were 80%. Ninety percent of patients were from rural areas and 80% were bearing a lower class socioeconomic background. The most common presentation was upper abdominal pain in 100% of patients. History of worm emesis was present in 34% and previous biliary tree pathology in 24% of patients. Ultrasonography demonstrated the commonest site of worm entrapment was common bile duct in 76% of patients and the commonest pattern was stripe sign pattern in 56% of patients. Conclusion: In endemic countries, ascariasis should to be suspected in patients with biliary disease, especially if there is history of emesis of worm or previous history of biliary tree pathology. Ultrasonography is a reliable, easily available and relatively inexpensive modality of investigation for suspected biliary ascariasis. [ABSTRACT FROM AUTHOR]

In normal human livers, EpCAMpos cells are mostly restricted in two distinct niches, which are (i) the bile ductules and (ii) the mucous glands present inside the wall of large intrahepatic bile ducts (the so-called peribiliary glands). These EpCAMpos cell niches have been proven to harbor stem/progenitor cells with great importance in liver and biliary tree regeneration and in the pathophysiology of human diseases. The EpCAMpos progenitor cells within bile ductules are engaged in driving regenerative processes in chronic diseases affecting hepatocytes or interlobular bile ducts. The EpCAMpos population within peribiliary glands is activated when regenerative needs are finalized to repair large intra- or extra-hepatic bile ducts affected by chronic pathologies, including primary sclerosing cholangitis and ischemia-induced cholangiopathies after orthotopic liver transplantation. Finally, the presence of distinct EpCAMpos cell populations may explain the histological and molecular heterogeneity characterizing cholangiocarcinoma, based on the concept of multiple candidate cells of origin. This review aimed to describe the precise anatomical distribution of EpCAMpos populations within the liver and the biliary tree and to discuss their contribution in the pathophysiology of human liver diseases, as well as their potential role in regenerative medicine of the liver.

Biliary fistula and bile leakage are complications that can occur during hepato-biliary surgery (both open and laparoscopic) and percutaneous biliary intervention. In some cases, spontaneous resolution is documented but more often re-intervention (surgical or percutaneous) is necessary. We present the case of a male patient who underwent right hepatectomy with bilio-digestive anastomosis for a cholangiocarcinoma which developed a bilo-cutaneous fistula through the path of a previously inserted percutaneous transhepatic drainage. Sealing of bilo-cutaneous fistula was obtained using N-butil-Cyanoacrylate. This technique has already been reported in some papers as a useful tool for biliary tree obliteration; however, to our knowledge, no cases describing the use of glue to seal a sub-cutaneous route are available in literature.

• In the hepatobiliary system anatomical variations can happen. • Though type II choledochal cyst has great outcome after surgery, it has differentials to think about before exploration. • The anatomic variation in choledochal cyst will cause diagnostic and management challenges. Choledochal cyst (CC) is an uncommon congenital disease of the biliary tract. There are five main types of CC with several recognized sub-types. However, occasional variants with a difficulty in diagnosis and management do occur. We report a case of a nine years old female child diagnosed with CC who presented with right quadrant abdominal pain with unremarkable physical findings. Investigation using abdominal CT scan suggested type II choledochal cyst. The intraoperative finding revealed an unusual site of the cyst that is at the confluence of common hepatic duct (CHD) posteriorly. The cyst was successfully excised and the child is doing well on her follow ups. In the management of choledochal cyst the anatomy should be clearly defined with detailed investigations like Abdominal CT Scan or cholangiography before surgical excision as abnormal variants which usually do not fit into the known classification types and subtypes. This might confuse with other differentials like gall bladder duplication. Surgical excision is the gold standard management option. This case report will alert surgeons that there are different anatomic variant of choledochal cysts out of the known classifications and with meticulous dissection will help proper excision and avoid unnecessary complications. [ABSTRACT FROM AUTHOR]

• Hydatid disease is a parasitic infestation by a tapeworm of the genus Echinococcosis of which 50%–75% of hydatid cysts occur in the liver. • In approximately one-fourth of the cases, hepatic hydatid cyst ruptures into the biliary tree producing obstructive jaundice. • Moreover, primary hydatid cysts of the biliary tract have been reported in English literature. • This is an interesting and intriguing case of obstructive jaundice caused by hydatid cyst in extrahepatic ducts 13 years after liver hydatid endocystectomy. • ERCP represent an important management strategy for patients with hydatid cysts in extra-hepatic ducts even primary or complications of liver hydatid cyst. Hydatid disease affects most commonly the liver, and complications with the rupture into the biliary tree develop in approximately one-fourth of the cases. Moreover, primary hydatid cysts of the biliary tract have been reported. We report an extremely rare case of obstructive jaundice caused by hydatid cyst in extrahepatic ducts 13 years after liver hydatid endocystectomy treated by Endoscopic retrograde cholangiopancreatography (ERCP). A 28-year-old male patient who had undergone surgical treatment – removal of liver hydatid cyst 13 years earlier, presented with signs of obstructed jaundice, confirmed with blood tests results and magnetic resonance cholangiopancreatography (MRCP). Actually, there were no pathological changes detected in the hepatic parenchyma, but the intrahepatic and extrahepatic bile ducts were dilated. ERCP was performed and the entire hydatid material was evacuated and washed out into the gastrointestinal tract. In addition, after laparoscopic cholecystectomy, hydatid cysts were also confirmed in the gallbladder. Generally, the obstructive jaundice caused by hydatid cyst in the extrahepatic ducts can also be caused by the rupture of the liver hydatid cyst in the biliary tract, or by primary hydatid cyst in the biliary tract. The ERCP plays a key role in the diagnosis and the treatment of this pathology. The ERCP, has now become an important diagnostic and therapeutic procedure in management of primary extrahepatic hydatid cysts and of complicated liver hydatid cysts. [ABSTRACT FROM AUTHOR]

Background/Aim: Biliary tree and pancreatic duct can appear in different variations whose proper understanding is obligatory for surgeons. Magnetic resonance cholangiopancreatography (MRCP) is considered a safe and accurate tool for evaluating biliary tree and pancreatic duct. Typical anatomy for right hepatic duct (RHD) and left hepatic duct (LHD) is reported as 57% and 63%, respectively. The most common (4-10%) pancreatic anomaly is divisum. In the present study, we evaluated and determined the prevalence of biliary tree and pancreatic duct variations among patients at a university hospital. Materials and Methods: The MRCP records of 370 patients from 2015 to 2017 were obtained for cross-sectional study. Images were retrospectively reviewed for variations by two independent senior radiologists. Demographic data were obtained for all the patients. Huang et al. classification was used for RHD and LHD variations. The cystic duct was reported based on its course and insertion pattern. The pancreatic duct was observed for the presence of divisum, its course, and configuration. Results: Three hundred and twenty-five patients were included in the final study. Most commonly observed variant for RHD were A1 (34.2%) and A2 (32.2%). For LHD, B1 (71.4%) was the most common variant. Cystic duct insertion was commonly seen as right lateral insertion (27.7%). Pancreatic divisum was observed in 0.6% of cases. Nationality, origin, and gender-specific variations were obtained. Conclusion: Variations in biliary anatomy and pancreatic duct are very diverse and extend from the intrahepatic biliary system down to the pancreas. Performing a similar study on a larger population is mandatory to illustrate the range of variations present within the community. [ABSTRACT FROM AUTHOR]

Contrast enhanced MRI of the liver provides valuable information in the evaluation of both chronic liver disease and focal liver lesions. Currently, two classes of MRI contrast agents are available for clinical use, namely the extracellular contrast agent (ECA) and the hepatobiliary agent (HBA). The use of appropriate contrast agents for liver MRI requires knowledge of the clinical situation and question to be answered. ECAs have been used for decades since their introduction into clinical practice and provide excellent dynamic phase information that is useful in characterizing focal liver lesions. In the last decade, HBAs, particularly Gadoxetate, have been found useful for characterizing lesions with functioning hepatocytes and more importantly in evaluating the biliary tree. Gadoxetate, however, provides less satisfactory dynamic phase images compared to ECAs, particularly during the arterial phase. In this perspective article, we will discuss the various intravenous contrast agents used for liver MRI and their ideal utilization. [ABSTRACT FROM AUTHOR]