Hepatolithiasis is a common disease where stones are located in the intrahepatic bile duct. Hepatolithiasis is a disease with regional characteristics. The complication and postoperative recurrence rates of the disease are high. The intrahepatic cholangiocarcinoma and the incidence of liver cirrhosisare the main causes of death in patients with hepatolithiasis. Thus, it is difficult to treat. The majority of biliary stones are readily removed endoscopically, however complex intrahepatic or large refractory extrahepatic stones often require surgical or percutaneous interventions when standard endoscopic methods fail. At present, the main clinical treatment for hepatolithiasis is surgery, of which there are different methods depending on the patient's condition. With the continuous updates and development of medical technology, the treatment of hepatolithiasis has improved. In this paper, several mainstream surgical methods including partial hepatectomy, choledochojejunostomy, biliary tract exploration and lithotomy, percutaneous transhepatic chledochoscopic lithotripsy and liver transplantation used in the clinic are reviewed for clinicians' reference. Depending on the characteristics of each case, a suitable surgical method is chosen to obtain the best treatment effect.

Clonorchis sinensis, Opisthorchis viverrini, and Opisthorchis felineus are the three important liver flukes, infecting approximately 25 million people worldwide. Despite the reporting of the carcinogenesis of these liver flukes, the comprehensive and systematic analysis of the pathogenicity of these parasites in hepatobiliary system is still not sufficient. We conducted a thorough systematic review and search for published articles in MEDLINE, Embase, Cochrane Library, China National Knowledge Infrastructure databases until early 2024. Cohort studies, case-control studies, and cross-sectional studies associated with C. sinensis, O. viverrini, or O. felineus infection were selected. Pooled risk ratio (RR), odds ratio (OR) and their 95 % confidence intervals (95 % CIs) were calculated to assess the risk of hepatobiliary complications due to these liver fluke infections. From a total of 6488 articles, 22 eligible studies and 34,367 participants were included for review. Our results showed C. sinensis, O. viverrini, and O. felineus infections were significantly associated with cholangiocarcinoma, with an overall OR of 4.24 (95 % CI: 3.33-5.39, P < 0.00001) and an overall RR of 10.43 (95 % CI: 2.90-37.47, P = 0.0003). The ORs for the association between cholangiocarcinoma and C. sinensis and O. viverrini infection were 4.49 (95 % CI:3.43-5.87, P < 0.00001) and 3.69 (95 % CI: 2.07-6.55, P < 0.00001) respectively. For the association between cholelithiasis and C. sinensis infection, the OR was 6.46 (95 % CI: 5.15-8.11, P < 0.00001). C. sinensis infection increased the risk of cholecystitis and cirrhosis, with the RR of 21.02 (95 % CI: 17.27-25.58) and an overall RR of 8.77 (95 % CI: 6.79-11.33, P < 0.00001) respectively. C. sinensis infection was also significantly associated with fatty liver, with an overall OR of 2.46 (95 % CI: 1.79-3.37, P < 0.00001). This comprehensive study, reviewing the largest dataset to date, provided an overall risk of hepatobiliary complications due to Clonorchis and Opisthorchis infections, and aids more systematic understanding for the pathogenicity of Opisthorchiidae family parasites.

Objectives: Although peroral cholangioscopy has improved the endoscopic treatment of difficult stones, the treatment of intrahepatic stones remains challenging. The incidence of cholangitis is high when peroral cholangioscopy is used to treat intrahepatic stones. This study aimed to investigate the efficacy and safety of endoscopic treatment with peroral cholangioscopy for intrahepatic and common bile duct stones. Methods: Patients aged ≥20 years, who underwent endoscopic treatment with peroral cholangioscopy for intrahepatic or common bile duct stones at Tottori University Hospital from January 2016 to December 2022, were retrospectively evaluated to determine the efficacy and safety of the treatment. Results: Overall, 70 patients were included in this study: 22 in the intrahepatic stone group and 48 in the common bile duct stone group. Stones were smaller (8 vs. 17.5 mm, p < 0.001) and more numerous (p = 0.016) in the intrahepatic stone group than in the common bile duct stone group. Although the common bile duct stone group exhibited a higher rate of complete stone clearance in the first session, no significant differences were observed in the final results. The intrahepatic stone group had a higher incidence of cholangitis (36% vs. 8%, p = 0.007); however, all cases were mild. Conclusions: Endoscopic treatment with peroral cholangioscopy for intrahepatic stones may be associated with a higher incidence of cholangitis than that for common bile duct stones. Since saline irrigation may contribute to the development of cholangitis, it is important to be aware of intraductal bile duct pressure when performing peroral cholangioscopy.

The term hepatolithiasis describes the presence of biliary stones within the intrahepatic bile ducts, above the hilar confluence of the hepatic ducts. The disease is more prevalent in Asia, mainly owing to socioeconomic and dietary factors, as well as the prevalence of biliary parasites. In the last century, owing to migration, its global incidence has increased. The main pathophysiological mechanisms involve cholangitis, bile infection and biliary strictures, creating a self-sustaining cycle that perpetuates the disease, frequently characterised by recurrent episodes of bacterial infection referred to as syndrome of "recurrent pyogenic cholangitis". Furthermore, long-standing hepatolithiasis is a known risk factor for development of intrahepatic cholangiocarcinoma. Various classifications have aimed at providing useful insight of clinically relevant aspects and guidance for treatment. The management of symptomatic patients and those with complications can be complex, and relies upon a multidisciplinary team of hepatologists, endoscopists, interventional radiologists and hepatobiliary surgeons, with the main goal being to offer relief from the clinical presentations and prevent the development of more serious complications. This comprehensive review provides insight on various aspects of hepatolithiasis, with a focus on epidemiology, new evidence on pathophysiology, most important clinical aspects, different classification systems and contemporary management.

Endoscopic retrograde cholangiopancreatography (ERCP) has been a significant development in gastrointestinal endoscopy. I did my first ERCP at SKIMS on December 5, 1982, and over the last 40 years, I have performed 10,100 ERCP procedures, including 600 Sphincter of Oddi manometries (SOM), and 3200 therapeutic ERCPs. We were confronted with many clinical challenges that needed answers by applying ERCP as a primary diagnostic tool. These studies gave birth to and/or recognition of several clinical syndromes. The hepatobiliary and pancreatic ascariasis (HBPA) as a clinical disease was recognized in 1985. The nematode, Ascaris lumbricoides, was the most common cause of hepatobiliary and pancreatic diseases in Kashmir, and its impact on healthcare, clinical profile, management policies, and control measures was identified. Kashmir was recognized as an endemic zone for recurrent pyogenic cholangitis (RPC), which constituted 12.5% of all biliary diseases. RPC in this population was found essentially to be an aftermath of HBPA. A subset of patients with hepatic hydatidosis with rupture into the biliary tract was recognized at ERCP and primarily treated by endotherapy. Cholangiographic abnormalities in children with portal cavernoma evolved into the recognition of portal biliopathy. Extensive studies of the sphincter of Oddi manometry in patients with unexplained biliary and/or pancreatic pain following cholecystectomy identified the entity of the sphincter of Oddi dyskinesia (SOD). In a cross-over trial, Nifedipine, compared with a placebo, showed a significant clinical response in 20 of 28 such patients. ERCP studies done in patients with tropical calcific pancreatitis showed an anomalous union of bile and pancreatic ducts. Forty of the 220 patients with liver transplantation had biliary complications namely biliary leaks, bile duct strictures, SOD, and recurrence of underlying primary biliary cholangitis. Biliary complications caused considerable morbidity and mortality in patients with liver transplantation.

Recurrent pyogenic cholangitis (RPC) is a condition found almost exclusively in individuals who lived in Southeast Asia. We report a case of a Caucasian veteran diagnosed with RPC after presenting with a 5-year history of recurrent fevers and abdominal pain 20 years after serving in Japan, South Korea, and Guam. Extensive evaluation led to the diagnosis of RPC with improvement after biliary decompression and antibiotics. Although rare, RPC should be considered in individuals who present with recurrent bouts of abdominal pain and fevers regardless of race.

Primary choledocholithiasis (PC) is a common disease in biliary surgery. The treatment is always challenging due to its high recurrence. A systemic review is undertaken to determine the risk factors for recurrence and provide with the individualized management strategy.

Electronic databases PubMed (Medline), Embase and Cochrane Central Register of Controlled Studies were searched for relevant articles on risk factors for PC recurrence. Its therapeutic intervention was also collected and analysed.

A total of 36 articles were eligible for inclusion. The recurrent risk factors include abnormalities of biliary anatomy (peripapillary diverticulum), dynamics (choledochal dilation, sharp angulation and stone number), metabolism (advanced age and hypothyroidism) and bacterial infection (Enterobacter and Helicobacter pylori). These factors eventually induce cholestasis and stone formation. At present, there is no guideline and expertise consensus for PC management. The treatment mainly consists of stone retrieval approaches and internal drainage surgeries. The former are minimally invasive methods: endoscopic sphincterotomy (EST), papillary balloon dilation (EPBD) and laparoscopic common bile duct exploration (LCBDE). The latter include choledochoduodenostomy (CDS) and choledochojejunostomy (CJS) with Roux-en-Y reconstruction. By far, the internal drainage surgeries have significantly lower recurrence than stone retrieval approaches.

Abnormal biliary anatomy, dynamics, metabolism and bacterial infection are the risk factors for PC. Both EST/EPBD and LCBDE can be performed as initial treatment. For recurrent PC, CDS is more suitable to the elderly, while Roux-en-Y CJS reserves for young patients or those in good conditions.

Hepatolithiasis is a disease characterized by intrahepatic stone formation. In this article, we review the features of this disease and explore the established and emerging treatment modalities.

Recent reports show an increasing prevalence of hepatolithiasis, likely owed to increased immigration and shifts in the Western diet. New pharmacotherapy options are limited and are often only supportive. Endoscopic intervention still cruxes on removal of impacted stones, though new techniques such as bile duct exploratory lithotomy and lithotripsy continue to advance management. Although hepatectomy of the effected portion of the liver offers definitive therapy, alternative less invasive modalities such as combined endoscopic/interventional radiology modalities have been utilized in select patients. Additionally, liver transplant serves as an option for otherwise incurable hepatolithiasis with coexisting liver dysfunction. Multiple emerging pharmacologic and procedural interventions may provide novel treatment for hepatolithiasis. While definitive therapy remains resection of affected liver segments, these modalities offer hope for less invasive approaches in the future.

Fibropolycystic liver diseases, also known as ductal plate malformations, are a group of associated congenital disorders resulting from abnormal development of the biliary ductal system. These disorders include congenital hepatic fibrosis, biliary hamartomas, polycystic liver disease, choledochal cysts and Caroli disease. Recently, it has been thought to include biliary atresia in this group of diseases, because ductal plate malformations could be implicated in the pathogenesis of this disease. Concomitant associated renal anomalies can also be present, such as autosomal recessive polycystic kidney disease (ARPKD), medullary sponge kidney and nephronophthisis. These disorders can be clinically silent or can cause abnormalities such as cholangitis, portal hypertension, gastrointestinal bleeding and infections. The different types of ductal plate malformations show typical findings at magnetic resonance (MR) imaging. A clear knowledge of the embryology and pathogenesis of the ductal plate plays a pivotal role to understand the characteristic imaging appearances of these complex diseases. Awareness of these MR imaging findings is central to the detecting and differentiating between various fibropolycystic liver diseases and is important to direct appropriate clinical management and prevent misdiagnosis.

Acute cholangitis is bacterial infection of the extra-hepatic biliary system. As it is caused by gallstones blocking the common bile duct in most of the cases, its prevalence is greater in ethnicities with high prevalence of gallstones. Biliary obstruction of any cause is the main predisposing factor. Diagnosis is established by the presence of clinical features, laboratory results and imaging studies. The treatment modalities include administration of intravenous fluid, antibiotics, and drainage of the bile duct. The outcome is good if the treatment is started early, otherwise it could be grave.

Recurrent pyogenic cholangitis (RPC) is an infective process involving the biliary tree typified by pigmented intraductal calculi with dilatation of the intra- and extrahepatic biliary tree. Previously endemic to South-east Asia, RPC can now be seen in Western countries with the increasing access to international travel and immigration. Affected patients are often plagued by recurrent bouts of cholangitis, and commonly suffer from complications such as abscess formation and biliary strictures. In severe cases, cirrhosis with portal hypertension may develop. The disease is also a known risk factor for cholangiocarcinoma, and can be seen in up to 5% of affected patients. Its exact etiology is unknown, but parasitic infections such as Clonorchis sinensis and Ascaris lumbricoides, ascending bacterial infection with gut flora (Escherichia coli) and low socioeconomic status have been associated strongly with it. This paper reviews the imaging features of the disease, as well as the roles of interventional radiology and surgery with respect to management of the condition.

To provide information about the role of liver fluke infection as a risk factor for hepatobiliary pathological changes and promote awareness among the people living in endemic areas, a systematic review and meta-analysis based on published studies was conducted to examine the association between liver fluke infection and hepatobiliary pathological changes.

Relevant original literature was searched in multiple literature databases, including PubMed, Cochrane, Clinical Evidence, Trip Database, Clinical Trials, Current Controlled Trials, Web of Science, the China National Knowledge Infrastructure (CNKI) database, and the Wanfang academic journal full-text database. Studies were selected based on strict screening with inclusion and exclusion criteria. Tests of heterogeneity, sensitivity and publication bias were performed with the Review Manager software, version 5.3, and meta-regression analyses were performed with the Stata software, version 11.0 (Stata Corporation, College Station, TX, USA). Pooled risk ratios (RRs) and odds ratios (ORs) with their 95% confidence intervals (95% CIs) were calculated and used to evaluate the risk of hepatobiliary pathological changes resulting from liver fluke infection. Linear trend analyses were conducted to determine the dose-response relationship using IBM SPSS Statistics 20.0.

A total of 36 studies were included in the meta-analysis. Significant associations were found between liver fluke infection and cholangitis or cholecystitis (RR: 7.80, P<0.001; OR: 15.98, P<0.001), cholelithiasis (RR: 2.42, P = 0.03; OR: 4.96, P = 0.03), hepatocellular carcinoma (OR: 4.69, P<0.001) and cholangiocarcinoma (RR: 10.43, P<0.001; OR: 4.37, P<0.001). In addition, heavier infection was significantly associated with higher incidence of hepatobiliary pathological changes (P<0.05). However, cirrhosis was not significantly associated with liver fluke infection (RR: 3.50, P = 0.06; OR: 5.79, P = 0.08). The statistical heterogeneity was significant, no significant difference was observed in the sensitivity analysis, and no publication bias was found.

The meta-analysis found that liver fluke infection was significantly associated with cholangitis, cholecystitis, cholelithiasis, hepatocellular carcinoma and cholangiocarcinoma and that more severe infection was associated with higher incidence. However, the association between liver fluke infection and cirrhosis was not significant.

Calcium carbonate gallbladder stones were easily neglected because they were previously reported as a rare stone type in adults. The aim of this study was to investigate the relationship between calcium carbonate stones and Clonorchis sinensis infection. A total of 598 gallbladder stones were studied. The stone types were identified by FTIR spectroscopy. The C. sinensis eggs and DNA were detected by microscopic examination and real-time fluorescent PCR respectively. And then, some egg-positive stones were randomly selected for further SEM examination. Corresponding clinical characteristics of patients with different types of stones were also statistically analyzed. The detection rate of C. sinensis eggs in calcium carbonate stone, pigment stone, mixed stone and cholesterol stone types, as well as other stone types was 60%, 44%, 36%, 6% and 30%, respectively, which was highest in calcium carbonate stone yet lowest in cholesterol stone. A total of 182 stones were egg-positive, 67 (37%) of which were calcium carbonate stones. The C. sinensis eggs were found adherent to calcium carbonate crystals by both light microscopy and scanning electron microscopy. Patients with calcium carbonate stones were mainly male between the ages of 30 and 60, the CO2 combining power of patients with calcium carbonate stones were higher than those with cholesterol stones. Calcium carbonate gallbladder stones are not rare, the formation of which may be associated with C. sinensis infection.

Oriental cholangiohepatitis, or recurrent pyogenic cholangitis is only noted in certain parts of the world, especially South East Asia. Due to increasing immigration the disease is now being seen in western countries also. Treating physicians may face difficulty in managing such cases due to lack of exposure. Furthermore management of such cases is not standardized because of lack of a universally accepted classification system. Here we review the disease and share our long experience with management of these patients.

Ascariasis mainly contributes to the global helminthic burden by infesting a large number of children in the tropical countries. Hepato-biliary ascariasis (HBA) is becoming a common entity now than in the past owing to the frequent usage of ultrasonograms and endoscopic diagnostic procedures in the clinical practice. There are a variety of manifestations in HBA and diagnosis depends on a high index of suspicion in endemic areas coupled with subsequent confirmation by sonographic or endoscopic demonstration of the worm. Most of them present with acute abdomen and jaundice. Oriental or recurrent pyogenic cholangiopathy is possibly the result of HBA, commonly encountered in South-East Asian countries. Conservative treatment with anthelminthic agents is used in the majority. Failure to respond to medical therapy usually indicates the need for endoscopic or surgical interventions. Overall, mortality is low and prognosis is good, but many epidemiological and immunological aspects of Ascaris infection are unclear, meaning our understanding the disease and infection still remains incomplete. Therefore, it is difficult to definitely put down a fixed modality of treatment for HBA. This underscores the need for further studies as ascariasis has the potential to adversely affect the national socio-economy by compromising the health of children and adults alike with its sheer number.

To improve the rate of detection of Clonorchis sinensis infection, we compared different specimens from patients with cholecystolithiasis. Feces, gallbladder bile, and gallbladder stones collected from 179 consecutive patients with cholecystolithiasis underwent microscopic examination, and according to the results, 30 egg-positive and 30 egg-negative fecal, gallbladder bile, and gallbladder stone specimens, respectively, underwent real-time fluorescent PCR. The detection rates of eggs in feces, bile, and gallbladder stones were 30.7%, 44.7%, and 69.8%, respectively, and the differences were statistically significant (P<0.01). The PCR results confirmed that the eggs in the specimens were C. sinensis eggs. Eggs in the feces were "fresh" and in the gallbladder stones were "old." Microscopic examination of gallbladder stones may improve the detection rates of C. sinensis infection, which is important for developing individualized treatments to prevent the recurrence of gallbladder stones and to prevent the occurrence of severe liver damage and cholangiocarcinoma.

The objective of this study was to describe the microstructure of different types of gallbladder stones to better understand the basis of gallbladder stone formation.

Gallbladder stones from 387 patients with cholecystolithiasis were first analyzed by Fourier transform Infrared spectroscopy to identify the type of the gallbladder stone, and they were then examined using scanning electron microscopy to define their microstructure.

Cholesterol stones were mainly composed of plate-like or lamellar cholesterol crystals stacked tightly in a radial, cord-like, or irregular staggered arrangement. A small number of bilirubinate particles were seen occasionally. Pigment stones were mainly composed of loosely arranged bilirubinate particles with different shapes (sphere-like, clumping-like, or amorphous). Calcium carbonate stones were composed of calcium carbonate crystals having many shapes (bulbiform, ellipsoid, fagot-shaped, fusiform, hawthorn-shaped, cuboid, button-shaped, lamellar, broken firewood-shaped, rod-shaped, acicular, or crushed sugar cane-shaped). Bulbiform shaped crystals were the most common. Phosphate stones were mainly composed of different sized echin-sphere-like or rough bulbiform crystals. Bilirubinate particles were always adherent. Calcium stearate stones usually had a network structure with adherent bilirubinate particles. Protein stones usually had a honeycomb or chrysanthemum petal-like structure. Cystine stones were composed of hexagonal cystine crystals, some of which had prominent edges. Mixed stones presented different shapes according to their mixed components.

Different types of gallbladder stones had characteristic shapes and elements. This study provides an objective basis for further research regarding gallbladder stone formation.

This paper describes typical diseases and morbidities classified in the category of miscellaneous etiology of cholangitis and cholecystitis. The paper also comments on the evidence presented in the Tokyo Guidelines for the management of acute cholangitis and cholecystitis (TG 07) published in 2007 and the evidence reported subsequently, as well as miscellaneous etiology that has not so far been touched on. (1) Oriental cholangitis is the type of cholangitis that occurs following intrahepatic stones and is frequently referred to as an endemic disease in Southeast Asian regions. The characteristics and diagnosis of oriental cholangitis are also commented on. (2) TG 07 recommended percutaneous transhepatic biliary drainage in patients with cholestasis (many of the patients have obstructive jaundice or acute cholangitis and present clinical signs due to hilar biliary stenosis or obstruction). However, the usefulness of endoscopic naso-biliary drainage has increased along with the spread of endoscopic biliary drainage procedures. (3) As for biliary tract infections in patients who underwent biliary tract surgery, the incidence rate of cholangitis after reconstruction of the biliary tract and liver transplantation is presented. (4) As for primary sclerosing cholangitis, the frequency, age of predilection and the rate of combination of inflammatory enteropathy and biliary tract cancer are presented. (5) In the case of acalculous cholecystitis, the frequency of occurrence, causative factors and complications as well as the frequency of gangrenous cholecystitis, gallbladder perforation and diagnostic accuracy are included in the updated Tokyo Guidelines 2013 (TG13). Free full-text articles and a mobile application of TG13 are available via http://www.jshbps.jp/en/guideline/tg13.html.

To assess MR findings and diagnostic performance for differentiating malignant from benign hepatic masses in recurrent pyogenic cholangitis (RPC).

During a recent 6-year period, we performed MRI in 352 patients with RPC. Among them, 58 had confirmed hepatic masses; cholangiocarcinoma (n = 15), abscess (n = 37), inflammatory pseudotumor (n = 3), biloma (n = 3). Two radiologists assessed MR findings including enhancement patterns, intratumoral appearance, peritumoral changes, mass location, and multiplicity. They also graded the malignancy using common MR findings. The receiver operating characteristic analysis and Chi-square test were used. The κ statistics was used to determine interobserver agreement.

The common findings for cholangiocarcinoma were thin and lobulated enhancement at the periphery (n = 8, 53%, P < 0.05); ill-defined enhancement (n = 7, 47%, P < 0.05); slightly high signal on T2 (n = 13, 87%, P < 0.05); mass located in the same lobe of atrophy (n = 11, 73%, P < 0.05) and portal vein thrombosis (n = 15, 100%, P < 0.05). The common findings for benign mass were target-like enhancement (n = 36, 84%, P < 0.05); cluster appearance (n = 15, 35%, P < 0.05); central, fluid-like space (n = 29, 67%, P < 0.05); peritumoral regional high signal on T2 (n = 32, 74%, P < 0.05); multiplicity(n = 21, 49%, P < 0.05). Interobserver agreement was excellent (κ = 0.81-1.000). Area under the curve (Az) for differentiating malignant masses was 0.989, sensitivity was 95.3%, and specificity was 95.3%. There was good interobserver agreement (κ = 0.74).

MR imaging is very useful for differential diagnosis of malignant vs. benign hepatic masses in patients with RPC.

Liver disease is an important source of morbidity among ill returning travelers. Jaundice is one of the most common and obvious symptoms of liver disease, the differential diagnosis of which is extensive, especially in travelers. Jaundice in travelers can arise from both infectious and noninfectious causes. We herein summarize the most common parasitic etiologies that may lead to jaundice in the returned traveler, visitors of friends and relatives, or new immigrants, and describe the etiology, epidemiology, and pathogenesis of clinical features of each.

The objective of this study was to analyze gallbladder stones for direct evidence of a relationship between Clonorchis sinensis infection and gallbladder stones formation.

We investigated one hundred eighty-three gallbladder stones for the presence of Clonorchis sinensis eggs using microscopy, and analyzed their composition using Fourier transform infrared spectroscopy. We confirmed the presence of Clonorchis sinensis eggs in the gallbladder stones using real-time fluorescent PCR and scanning electron microscopy.

Clonorchis sinensis eggs were detected in 122 of 183 gallbladder stones based on morphologic characteristics and results from real-time fluorescent PCR. The proportion of pigment stones, cholesterol stones and mixed gallstones in the egg-positive stones was 79.5% (97/122), 3.3% (4/122) and 17.2% (21/122), respectively, while 29.5% (18/61), 31.1% (19/61) and 39.3% (24/61) in the egg-negative stones. The proportion of pigment stone in the Clonorchis sinensis egg-positive stones was higher than in egg-negative stones (P<0.0001). In the 30 egg-positive stones examined by scanning electron microscopy, dozens or even hundreds of Clonorchis sinensis eggs were visible (×400) showing a distinct morphology. Many eggs were wrapped with surrounding particles, and in some, muskmelon wrinkles was seen on the surface of the eggs. Also visible were pieces of texture shed from some of the eggs. Some eggs were depressed or without operculum while most eggs were adhered to or wrapped with amorphous particles or mucoid matter (×3000).

Clonorchis sinensis eggs were detected in the gallbladder stones which suggests an association between Clonorchis sinensis infection and gallbladder stones formation, especially pigment stones.

Recurrent pyogenic cholangitis (RPC), or oriental cholangiohepatitis, is characterized by intrabiliary pigment stone formation, whose main manifestation consists of recurrent episodes of cholangitis, although other biliopancreatic complications can also occur. RPC develops mainly in Asian patients, in whom this entity is one of the main causes of acute abdominal pain. The differential diagnosis should be established with all other entities associated with intrahepatic stone formation, which is more common in Asian countries compared with the predominance of gallstone formation in the West. The diagnostic and therapeutic approach requires collaboration among gastroenterologists, radiologists and gastrointestinal surgeons. We review the most important clinical, diagnostic and therapeutic features of this entity, which, although clearly predominant in Asia, is increasingly diagnosed in our setting.

The detection of an abnormal hepatic mass with ductal dilatation is highly concerning for malignancy. However, if such patients happen to be immigrants from endemic parts of Asia or South America, further investigations are necessary to rule out oriental cholangiohepatitis, a rare recurrent disease of the hepatobiliary system that can masquerade as cholangiocarcinoma.

We report a case of a patient of South Asian origin who presented to us with acute cholangitis and moderately dilated left hepatic ducts. The findings were highly suspicious for advanced hepatic malignancy; however the laboratory and pathological investigations remained normal. We suspected an unlikely etiology and proceeded with conservative hepatic resection. The histology revealed cholangiohepatitis without any evidence of malignancy.

Cholangiohepatitis is a complex hepatobiliary disease that commonly manifests as recurrent cholangitis or overt biliary sepsis and can rarely present as an abnormal hepatic mass. It results from the development of intrahepatic or extrahepatic strictures that causes stone formation and biliary dilation in the absence of gallbladder disease. Although it is endemic in many parts of the world, it is rare in the western world, and therefore it can present as a significant diagnostic enigma.

Cholangiohepatitis is a rare clinical entity that requires a multi-disciplinary team approach. Surgery plays a dominant role in the management of such patients and therefore surgeons need to be aware of this disease.

There is a wide spectrum of nonneoplastic causes of biliary stricture that can pose a significant challenge to clinicians and radiologists. Imaging plays a key role in differentiating benign from malignant strictures, defining the extent, and directing the biopsy. We describe the salient clinical and imaging manifestations of benign biliary strictures that will help radiologists to accurately diagnose these entities.

Accurate diagnosis and management are based on correlating imaging findings with epidemiologic, clinical, and laboratory data. Cross-sectional imaging modalities permit precise localization of the site and length of the segment involved, thereby serving as a road map to surgery, and permit exclusion of underlying malignancy.

Recurrent pyogenic cholangitis is managed mostly by conservative treatment or by clearance of stones and biliary enteric by-pass procedure. Hepatectomy is rarely needed. We report a case of recurrent pyogenic cholangitis in a 34-year old man, who presented with recurrent upper abdominal pain, mild jaundice, and fever since childhood. Contrast enhanced computed tomography of abdomen and magnetic resonance cholangiopancreatography confirmed the diagnosis and showed significant atrophy of the left lobe of the liver. Patient was treated successfully with left hepatectomy and was well at 9 month follow-up.

To evaluate risk factors for the recurrence of biliary stones after a percutaneous transhepatic biliary stone extraction.

The procedures were performed on 339 patients between July 2004 and December 2008 (54 months). Medical records and images were retrospectively reviewed for 135 patients (mean age, 66.4 years; 83 men and 52 women) who had undergone follow-up for a mean of 13.2 months (range, 3-37 months). To evaluate risk factors for the recurrence of biliary stones, variables were evaluated with univariate and multivariate analyses. Variables included sex, age, stone location, number of stones, stone size, presence of a peripapillary diverticulum, application of antegrade sphincteroplasty, presence of a biliary stricture, largest biliary diameter before the procedure, and gallbladder status.

Thirty-three of the 135 patients (24%) had recurrent symptomatic biliary stones and underwent an additional extraction. The mean time to recurrence was 17.2 months +/- 8.7. Univariate analysis of risk factors for recurrence of biliary stones demonstrated that location, number of stones, stone size, application of antegrade sphincteroplasty, presence of a biliary stricture, and biliary diameter were significant factors (P < .05). With use of multivariate analysis, the number of stones (> or =6; relative risk, 64.8; 95% confidence interval: 5.8, 717.6) and stone size (> or =14 mm; relative risk, 3.8; 95% confidence interval: 1.138, 13.231) were determined to be significant risk factors.

The independent risk factors for recurrence of symptomatic biliary stones after percutaneous transhepatic biliary stone extraction were a stone size of at least 14 mm and the presence of at least six stones.

To investigate the feasibility of the use of gadobenate dimeglumine (also known as Gd-BOPTA) -enhanced T1-weighted MR cholangiography in diagnosis of acute cholecystitis.

This prospectively designed institutional review board-approved HIPAA-compliant study was done between January and November 2007. We included 11 consecutive patients (7 male, mean age 59 years) who presented to the emergency room with acute right upper quadrant pain and with equivocal physical examination and/or ultrasound findings. The control group included 15 patients who underwent liver MRI with Gd-BOPTA. All patients underwent contrast-enhanced (CE) MR cholangiography examinations. CE-MR cholangiography was performed on a 1.5 Tesla magnet using 3D T1-weighted high resolution isotrophic volume examination (THRIVE) obtained at the 90th min after intravenous injection of Gd-BOPTA. Imaging features detected on CE-MR cholangiography were correlated with operative and histopathologic findings.

In the control group, GD-BOPTA was visualized within the gallbladder in all subjects. For the study group, gallstones were present in nine patients (n = 7 both in gallbladder and cystic duct, n = 1 only in gallbladder, n = 1 only in cystic duct) on MRCP. Hydropic gallbladder was detected in seven patients, significant wall thickening in seven patients, and pericholecystic free fluid in 6 patients. On delayed phase CE cholangiography, significant enhancement of gallbladder wall was seen in 10 patients, and contrast agent excretion into gallbladder was absent in all patients. Surgery was performed in 10 patients, and cholecystostomy was done in 1 patient. Surgery and histopathology findings were consistent with cholecystitis in all patients.

In addition to anatomical assessment, Gd-BOPTA-enhanced MR cholangiography can provide functional evaluation similar to HIDA scintigraphy in diagnosing acute cholecystitis in patients with acute right upper quadrant pain and equivocal findings.

Suppurative cholangitis is characterized by obstruction, inflammation, and pyogenic infection of the biliary tract. This disease represents a true emergency. The purpose of this study was to compare the computed tomography (CT) findings between acute calculous suppurative and nonsuppurative cholangitis and to determine if there are findings that assist in the differential diagnosis.

Fifteen patients with acute suppurative cholangitis were enrolled in this study. Findings at endoscopic retrograde cholangiopancreaticography (ERCP) were the standard of reference for suppurative cholangitis. To compare the findings of suppurative cholangitis with those of nonsuppurative cholangitis, 35 patients with nonsuppurative cholangitis were randomly selected. The following findings were evaluated: the presence of papillitis, the presence of stones in the ampulla, the presence of intrahepatic stones, the presence of early inhomogeneous enhancement of the liver, the degree of bile duct dilatation, the degree of bile duct wall thickening and presence of cholecystitis. Sensitivity and specificity for each of the individual findings were calculated. Statistical analyses were performed the Pearson chi(2) test, Fisher's exact test and the Mann-Whitney U test.

Papillitis showed the highest specificity 86% with 60% sensitivity. Marked inhomogeneous enhancement of the liver during the arterial phase showed 80% specificity with 60% sensitivity. In multivariate logistic analysis, papillitis and marked early inhomogeneous enhancement of the liver were the most significant predictors of acute suppurative cholangitis. The combination of these two CT findings improved specificity (97% specificity) for the diagnosis of suppurative cholangitis.

Papillitis and marked early inhomogeneous enhancement of the liver were found to be the most discriminative CT findings for the diagnosis of acute suppurative cholangitis and the differentiation between suppurative and nonsuppurative cholangitis.

To investigate prevalence of Clonorchis sinensis in patients with gastrointestinal symptoms, and the relation of the infection to hepatobiliary diseases in 26 hospitals in Korea.

Consecutive patients who had been admitted to the Division of Gastroenterology with gastrointestinal symptoms were enrolled from March to April 2005. Of those who had been diagnosed with clonorchiasis, epidemiology and correlation between infection and hepatobiliary diseases were surveyed by questionnaire.

Of 3080 patients with gastrointestinal diseases, 396 (12.9%) had clonorchiasis and 1140 patients (37.2%) had a history of eating raw freshwater fish. Of those with a history of raw freshwater fish ingestion, 238 (20.9%) patients had clonorchiasis. Cholangiocarcinoma was more prevalent in C. sinensis-infected patients than non-infected patients [34/396 (8.6%) vs 145/2684 (5.4%), P = 0.015]. Cholangiocarcinoma and clonorchiasis showed statistically significant positive cross-relation (P = 0.008). Choledocholithiasis, cholecystolithiasis, cholangitis, hepatocellular carcinoma, and biliary pancreatitis did not correlate with clonorchiasis.

Infection rate of clonorchiasis was still high in patients with gastrointestinal diseases in Korea, and has not decreased very much during the last two decades. Cholangiocarcinoma was related to clonorchiasis, which suggested an etiological role for the parasite.

Cholangiocarcinoma is the second most common primary malignant hepatobiliary neoplasm, accounting for approximately 15% of liver cancers. Diagnosis of cholangiocarcinoma is challenging and the prognosis is uniformly poor, with recurrence rates of 60%-90% after surgical resection. A wide spectrum of neoplastic and nonneoplastic conditions of the biliary tract may masquerade as cholangiocarcinoma, adding to the complexity of management in patients suspected to have cholangiocarcinoma. Mimics of cholangiocarcinoma constitute a heterogeneous group of entities that includes primary sclerosing cholangitis, recurrent pyogenic cholangitis, acquired immunodeficiency syndrome cholangiopathy, autoimmune pancreatitis, inflammatory pseudotumor, Mirizzi syndrome, xanthogranulomatous cholangitis, sarcoidosis, chemotherapy-induced sclerosis, hepatocellular carcinoma, metastases, melanoma, lymphoma, leukemia, and carcinoid tumors. These entities demonstrate characteristic histomorphology and variable clinicobiologic behaviors. The imaging findings of these disparate entities are protean and may be indistinguishable from those of cholangiocarcinoma. In most cases, a definitive diagnosis can be established only with histopathologic examination of a biopsy specimen.

A high prevalence of intrahepatic stones in some areas of East Asia has been believed to be related with Clonorchis sinensis infection. The authors conducted a hospital-based case-control study to evaluate the role of Clonorchis sinensis infection as a risk factor for the development of gallstones in Korea.

The cases of 138 patients with gallstones (intrahepatic 44, gallbladder 67, and extrahepatic 27) and matched controls underwent microscopy for C. sinensis, serological tests for C. sinensis using enzyme-linked immunosorbent assay, radiological examinations, and interviews concerning the history of eating raw freshwater fish. We assessed a relationship of three types of gallstones and variables regarding C. sinensis by using univariate and multivariate statistical analyses.

Univariate statistical analyses showed that radiological evidence of C. sinensis and recent history of eating raw freshwater fish were related to an increased risk of intrahepatic stones (P = 0.0002 and 0.0039, respectively). According to multivariate statistical analyses, radiological evidence of C. sinensis was the only risk factor for intrahepatic stones (odds ratio = 7.835; 95% confidence interval = 1.671-36.724). Any evidence regarding C. sinensis was not related to an increased risk of either gallbladder or extrahepatic stones.

Radiological evidence of C. sinensis was significantly associated with intrahepatic stones.

Choledocholithiasis (bile duct stones) occurs in 10% to 15% of patients with cholelithiasis. Endoscopic retrograde cholangiopancreatography and biliary sphincterotomy, combined with the use of a variety of available accessories (eg, mechanical lithotriptors), are highly effective in managing most common bile duct stones. The combination of biliary sphincterotomy and papillary balloon dilation allows removal of many stones without the need for mechanical lithotripsy. Large stones may require additional expertise and the use of cholangioscopy with intraductal lithotripsy. Some patients, especially those with intrahepatic stones, require the help of an interventional radiologist or a biliary surgeon. The availability of endoscopists, radiologists, and surgeons interested in and experienced with biliary diseases will allow successful and safe therapy to be tailored to the individual patient.

Hepaticolithiasis is a rare disease of the intrahepatic bile ducts endemic to east Asia which also is known as oriental cholangiohepatitis. Beside strictures and metabolic disturbance, the main cause of intrahepatic stones is acquired or congenital malformation of the intrahepatic biliary ducts. In the following we show a case of hepaticolithiasis and concurrent pancreatic metaplasia.

Among several diagnostic tools for clonorchiasis (Clonorchis sinensis infection), radiologic examinations are commonly used in clinical practices. During the 2 past decades, many reports regarding imaging findings of clonorchiasis were introduced. The basic imaging finding of clonorchiasis is diffuse dilatation of the peripheral intrahepatic bile ducts, without dilation of the large intrahepatic or extrahepatic bile ducts. By this finding, however, active clonorchiasis cannot be differentiated from cured infection. Some recent radiologic studies suggested specific findings of active clonorchiasis. Besides direct demonstration of worms, increased periductal echogenicity on sonography and periductal enhancement on dynamic contrast-enhanced CT or MR imaging possibly represent active clonorchiasis. Those images of the liver clonorchiasis are known to be correlated with worm burdens (EPG counts) in their frequency and also severity. The images of cholangiocarcinoma associated with clonorchiasis show both the tumor with obstruction images and diffuse dilatation of the peripheral intrahepatic bile ducts. Radiological images can be a good practical alternative diagnostic method of clonorchiasis.

In this essay, we present the spectrum of intrahepatic and extrahepatic bile duct abnormalities seen on MRCP in patients with recurrent pyogenic cholangitis.

MRCP is a promising, noninvasive alternative to more invasive direct cholangiography for evaluating the intrahepatic and extrahepatic bile ducts in patients with recurrent pyogenic cholangitis.

Unusual cases of acute cholecystitis and cholangitis include (1) pediatric biliary tract infections, (2) geriatric biliary tract infections, (3) acalculous cholecystitis, (4) acute and intrahepatic cholangitis accompanying hepatolithiasis (5) acute biliary tract infection accompanying malignant pancreatic-biliary tumor, (6) postoperative biliary tract infection, (7) acute biliary tract infection accompanying congenital biliary dilatation and pancreaticobiliary maljunction, and (8) primary sclerosing cholangitis. Pediatric biliary tract infection is characterized by great differences in causes from those of adult acute biliary tract infection, and severe cases should be immediately referred to a specialist pediatric surgical unit. Because biliary tract infection in elderly patients, who often have serious systemic conditions and complications, is likely to progress to a serious form, early surgery or biliary drainage is necessary. Acalculous cholangitis, which often occurs in patients with serious concomitant conditions, such as those in intensive care units (ICUs) and those with disturbed cardiac, pulmonary, and nephric function, has a high mortality and poor prognosis. Cholangitis accompanying hepatolithiasis includes recurrent pyogenic cholangitis, an epidemic disease in Southeast Asia. Biliary tract infections, which often occur after a biliary tract operation and treatment of the biliary tract, may have a fatal outcome, and should be carefully observed. The causes of acute cholangitis associated with pancreaticobiliary maljunction differ before and after operation. Direct cholangiography is most useful in the diagnosis of primary sclerosing cholangitis. If cholangiography visualizes a typical bile duct, differentiation from acute pyogenic cholangitis is easy. This article discusses the individual characteristics, diagnostic criteria, treatment guidelines, and prognosis of these unusual types of biliary tract infection.