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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.

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.

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.

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.

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.

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.

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.

Rhabdomyosarcoma (RMS) is the most common tumor of biliary tree in childhood. Biliary tree is an uncommon site for RMS to occur, accounting for about 1% of all pediatric RMS. A preoperative diagnosis is not difficult with background knowledge of its imaging features. The diagnosis of this malignancy is especially important as it many times mimics a more common and benign condition of choledochal cyst. The key to diagnosis is to distinguish solid component of the tumor from organized sludge seen in choledochal cyst. Imaging plays a vital role for preoperative staging as well.

Cholangiocarcinoma (CCA) is a highly-aggressive malignancy arising from the biliary tree, characterized by a steady increase in incidence globally and a high mortality rate. Most CCAs are diagnosed in the advanced and metastatic phases of the disease, due to the paucity of signs and symptoms in the early stages. This fact, along with the poor results of the local and systemic therapies currently employed, is responsible for the poor outcome of CCA patients and strongly supports the need for novel therapeutic agents and strategies. In recent years, the introduction of next-generation sequencing technologies has opened new horizons for a better understanding of the genetic pathophysiology of CCA and, consequently, for the identification and evaluation of new treatments tailored to the molecular features or alterations progressively elucidated. In this review article, we describe the potential targets under investigation and the current molecular therapies employed in biliary tract cancers. In addition, we summarize the main drugs against CCA under evaluation in ongoing trials and describe the preliminary data coming from these pioneering studies.

Calcium (Ca2+) is a versatile second messenger that regulates a number of cellular processes in virtually every type of cell. The inositol 1,4,5-trisphosphate receptor (ITPR) is the only intracellular Ca2+ release channel in cholangiocytes, and is therefore responsible for Ca2+-mediated processes in these cells. This review will discuss the machinery responsible for Ca2+ signals in these cells, as well as experimental models used to investigate cholangiocyte Ca2+ signaling. We will also discuss the role of Ca2+ in the normal and abnormal regulation of secretion and apoptosis in cholangiocytes, two of the best characterized processes mediated by Ca2+ in this cell type.

Here we report a rare case of living Fasciola hepatica in biliary tract. The patient was in acute phase of infection and treated successfully with 10 mg/kg oral triclabendazole after the fluke was extracted using endoscopic retrograde cholangiopancreatography (ERCP).

Choledochal cysts (CDC) have been traditionally classified into five types and subtypes based on the pattern and location of involvement of intra and extra hepatic biliary tree. Herein, we describe a new variant of choledochal cyst which has not been previously described in the English-language medical literature.

Gallbladder carcinoma is the 5th most common gastrointestinal cancer. Gallbladder cancer preferentially metastasizes to regional lymph nodes and liver parenchyma. Bone metastases from gallbladder carcinoma are rare presentation. We report a case of gallbladder carcinoma with solitary metastasis to femur bone with surrounding soft tissue involvement, mimicking as soft tissue tumour involving bone.