Segmental Cholangiectasia of the Intrahepatic Bile Duct: A Case Report

Objective To investigate the expression of Muc1,Muc3A and Muc4 in cultured intrahepatic bile duct(IBD)tissues from different hepatic segments after cold preservation.Methods The IBD tissues of SD rats were obtained by collagen perfusion combining mechanical separation and then were divided into large and small IBD.The 2 parts of IBD were seeded in rat tail collagen gel and were cultured for 48 hours,then the IBD tissues from 10 rats were stored in UW solution at 4℃ for 1 hour(group I,n=5)and 12 hours(group Ⅱ,n=5),respectively,and the IBD tissues from the rest 5 rats were cultured in incubator at 37℃ for 24 hours (control group,n=5).The expressions of Muc1,Muc3A and Muc4 were detected by RT-PCR and Western blot.All data were analysed via ANOVA or LSD test.Results The expressions of Muc1,Muc3 A and Muc4 were detected both in large and small IBD tissues.The mRNA expressions of Muc1,Muc3A and Muc4 were decreased in large IBD as time passed by,which were 0.95±0.14,0.26±0.04 and 0.24±0.06 in group Ⅰ,0.18±0.03,0.14±0.04 and 0.22±0.07 in group Ⅱ,1.00±0.20,1.00±0.09 and 1.00±0.21 in control group,with significant difference among the 3 groups(F=8.8,57.1,10.8,P＜0.05).The mRNA expressions of Muc1 and Muc3A in group Ⅱ were significantly lower than in group Ⅰ(P＜0.05).The protein expressions of Muc1 and Mue3A in large IBD were also decreased as time passed by,which were 0.82±0.13,0.73±0.10 in group Ⅰ,0.56±0.11,0.33±0.04 in group Ⅱ,1.05±0.41,1.06±0.38 in control group,with significant difference among the 3 groups(F=3.9,12.6,P＜0.05).The protein expression of Muc1 of group Ⅱ was significantly lower than in control group(P＜0.05),and the protein expression of Muc4 in group Ⅱ was significantly lower than in group Ⅰ(P＜0.05).The mRNA expressions of Muc3A in small IBD were increased as time passed by,which were 0.15±0.04 in group Ⅰ,0.19±0.05 in group Ⅱ and 0.06±0.03 in control group.Conclusion Decreases of Muc1,Muc3A and Muc4 in IBD after long time cold preservation may weaken the selfprotection of biliary epithelium and case sever injury to bile duct. Key words: Intrahepatic bile duct; Tissue culture; Cold preservation; Mucin

Intrahepatic Biliary Cysts Presenting before Hepatic Portoenterostomy in Biliary Atresia

This Editorial refers to the article by Y. Takagi et al., p 422–430 of this issue.

A 25-year-old primigravid woman was referred at 30 weeks' gestation to evaluate a fetal intra-abdominal cystic mass. Sonographic examinations revealed a singleton fetus with biometry consistent with 30 weeks of gestation. An intra-abdominal cystic mass measuring 22 × 12 mm was noted. The amniotic fluid volume was normal. Color Doppler ultrasound revealed no flow within the mass. The cyst connected via the cystic duct to the gallbladder, raising the suspicion of a choledochal cyst (Figure 1). Ultrafast magnetic resonance imaging (MRI) scans further depicted a detailed anatomical relationship of the choledochal cyst to the liver, gallbladder and biliary tract (Figure 2). The choledochal cyst consisted of a cystic dilation of the common bile duct without dilation of the gallbladder and intrahepatic bile ducts. A diagnosis of Type IA choledochal cyst was thus made. The size of the cyst remained unchanged during follow-up ultrasound examinations. Sonographic image of a choledochal cyst at 30 weeks' gestation. The choledochal cyst (CC) connects with the gallbladder (GB). Ultrafast magnetic resonance imaging scan of a Type IA choledochal cyst at 30 weeks' gestation. IHD, intrahepatic duct. At 39 weeks' gestation a female infant was born by vaginal delivery with Apgar scores of 10 and 10 at 1 and 5 min, respectively. The postnatal ultrasound and biochemical examinations confirmed the presence of a choledochal cyst. At 7 days of age the cyst was excised and a Roux-en-Y hepaticojejunostomy was performed. At surgery a Type IA choledochal cyst was noted. The histopathological features were consistent with the diagnosis of a choledochal cyst. The infant was discharged 8 days after the operation and was progressing well at 1 month of age. Choledochal cysts are rare anomalies that appear as cystic or fusiform dilations of the extra- or intrahepatic biliary tree or both. Five types of choledochal cysts have been described1. Type I cysts consist of a cystic dilation of the common bile duct (Type IA), a focal segmental dilation of the distal common bile duct (Type IB), or a fusiform dilation of both the common hepatic duct and the common bile duct (Type IC). Type II cysts are true diverticula of the common bile duct. Type III cysts, or choledochoceles, are cystic dilations involving only the intraduodenal portion of the common bile duct. Type IV cysts consist of multiple intra- and extrahepatic cysts (Type IVA) or multiple extrahepatic cysts only (Type IVB). Type V cysts comprise single or multiple cystic dilation of the intrahepatic bile ducts. The differential diagnosis of choledochal cysts includes simple hepatic cysts, biliary atresia, ovarian, omental or mesenteric cysts, duodenal or gallbladder duplications, adrenal cysts, renal cysts, dilated loops of bowel, hydronephrotic renal pelvis and situs inversus. Prenatal diagnosis of choledochal cysts with ultrasound has been well described in the literature2-5. In the preoperative assessment of choledochal cysts, MRI, MR cholangiography, and MR cholangiopancreatography have been used to accurately demonstrate the presence, extent and types of the cysts6-12. Additionally, MRI has the advantages of rapid delineation of tissue borders and easy selection of imaging planes. However, the role of MRI in the prenatal investigation of choledochal cysts remains unclear. MacKenzie et al.13 previously reported the prenatal ultrafast MRI finding of a choledochal cyst in a fetus at 16 weeks' gestation. In their case, the MRI image of the choledochal cyst was presented as a simple intra-abdominal cyst that was not different from that obtained by prenatal ultrasound. In contrast, the present case provides evidence that, in the third trimester, MRI is able to present more precise information than ultrasound in delineating the anatomical relationship of the cyst to the liver, gallbladder and biliary tract. We suggest that ultrafast MRI is a useful tool in the third-trimester investigation of choledochal cysts in addition to prenatal ultrasonography. C.-P. Chen* , S.-J. Cheng?, T.-Y. Chang*, L.-F. Yeh*, Y.-H. Lin*, W. Wang , * Department of Obstetrics and Gynecology, Mackay Memorial Hospital, 92, Section 2, Chung-Shan North Road, Taipei, Taiwan, Republic of China, Department of Medical Research, Mackay Memorial Hospital, Taipei, Taiwan, Republic of China, Department of Nursing, National Yang-Ming University, Taipei, Taiwan, Republic of China, ? Department of Radiology, Mackay Memorial Hospital, Taipei, Taiwan, Republic of China

The College of American Pathologists offers these protocols to assist pathologists in providing clinically useful and relevant information when reporting results of surgical specimen examinations. The College regards the reporting elements in the "Surgical Pathology Cancer Case Summary (Checklist)" portion of the protocols as essential elements of the pathology report. However, the manner in which these elements are reported is at the discretion of each specific pathologist, taking into account clinician preferences, institutional policies, and individual practice.The College developed these protocols as an educational tool to assist pathologists in the useful reporting of relevant information. It did not issue the protocols for use in litigation, reimbursement, or other contexts. Nevertheless, the College recognizes that the protocols might be used by hospitals, attorneys, payers, and others. Indeed, effective January 1, 2004, the Commission on Cancer of the American College of Surgeons mandated the use of the checklist elements of the protocols as part of its Cancer Program Standards for Approved Cancer Programs. Therefore, it becomes even more important for pathologists to familiarize themselves with these documents. At the same time, the College cautions that use of the protocols other than for their intended educational purpose may involve additional considerations that are beyond the scope of these documents.This protocol applies to carcinomas of the intrahepatic bile ducts and mixed hepatocellular-cholangiocarcinoma. Hepatocellular carcinoma, hepatoblastoma, and carcinomas of the perihilar bile ducts are not included. The seventh edition TNM staging system for intrahepatic bile ducts of the American Joint Committee on Cancer and the International Union Against Cancer is recommended.Intrahepatic Bile Ducts: Resection (note A)Select a Single Response Unless Otherwise Indicated*Data elements with asterisks are not required. However, these elements may be clinically important but are not yet validated or regularly used in patient management.Specimen (select all that apply)Procedure (select all that apply)Tumor SizeGreatest dimension: ___ cm*Additional dimensions: ___ × ___ cm___ Cannot be determined (see Comment)Tumor Focality (note B)Histologic Type (note C)Histologic Grade (note D)Tumor Growth Pattern (note E)Microscopic Tumor Extension (select all that apply)Margins (select all that apply) (note F)Hepatic Parenchymal MarginBile Duct MarginOther MarginLymph-Vascular InvasionVenous (Major Vessel) Invasion (V) (invasion of right or left portal vein, 1 or more hepatic veins)Small Vessel Invasion (L)*Perineural InvasionPathologic Staging (pTNM) (note G)TNM Descriptors (required only if applicable) (select all that apply)Primary Tumor (pT)Regional Lymph Nodes (pN) (note H)Distant Metastasis (pM)*Additional Pathologic Findings (select all that apply) (note I)*Ancillary Studies*Clinical History (select all that apply) (note J)*Comment(s): ____________________________________* Data elements with asterisks are not required. However, these elements may be clinically important but are not yet validated or regularly used in patient management.This protocol applies only to hepatic resection specimens containing carcinomas arising in the intrahepatic bile ducts. Hepatocellular carcinomas and carcinomas arising in the perihilar bile ducts are staged using separate TNM systems.1 A separate staging system for intrahepatic cholangiocarcinoma is warranted on the basis of biologic differences in tumor behavior and prognostic factors, such as lack of prognostic impact of tumor size for cholangiocarcinoma compared with hepatocellular carcinoma.1Anatomically, the intrahepatic bile ducts extend from the periphery of the liver to the second-order bile ducts (Figure 1). The perihilar bile ducts extend from the hepatic duct bifurcation to include the extrahepatic biliary tree proximal to the origin of the cystic duct. The distal extrahepatic bile duct extends the junction of the cystic duct–bile duct to the ampulla of Vater.1Sections should be prepared from each major tumor nodule, with representative sampling of smaller nodules if macroscopically different in appearance. For purposes of staging, satellite nodules, multifocal primary cholangiocarcinomas, and intrahepatic metastases are not distinguished and are considered multiple tumors.1 In intrahepatic cholangiocarcinoma, multiple tumor deposits have been associated with poorer survival.2,3The protocol recommends the following modified classification of the World Health Organization.4 In the United States, approximately 30% of the primary malignant tumors of the liver are biliary carcinomas.4CholangiocarcinomaCombined hepatocellular and cholangiocarcinomaBile duct cystadenocarcinomaCombined or mixed hepatocellular-cholangiocarcinoma accounts for less than 5% of primary liver carcinomas5 and should show histologic evidence of both hepatocellular differentiation and bile duct differentiation, such as production of mucin. These tumors generally have a poor prognosis and often arise in the setting of cirrhosis.5,6 Recent studies have found genetic changes similar to those seen in cholangiocarcinoma.7For cholangiocarcinomas, definitive criteria for histologic grading have not been established; however, the following quantitative grading system based on the proportion of gland formation within the tumor is suggested.Three tumor growth patterns of intrahepatic cholangiocarcinoma are described: the mass-forming type, the periductal-infiltrating type, and mixed mass-forming/periductal-infiltrating type. Mass-forming intrahepatic cholangiocarcinoma (60% of cases) forms a well-demarcated nodule growing in a radial pattern and invading the adjacent liver parenchyma (Figure 2). In contrast, the periductal-infiltrating type of cholangiocarcinoma (20% of cases) spreads in a diffuse longitudinal growth pattern along the bile duct. The remaining 20% of cases of intrahepatic cholangiocarcinoma grow in a mixed mass-forming/periductal-infiltrating pattern. Limited analyses suggest that the diffuse periductal-infiltrating type is associated with a poor prognosis.2,8The evaluation of margins for total or partial hepatectomy specimens depends on the method and extent of resection. It is recommended that the surgeon be consulted to determine the critical foci within the margins that require microscopic evaluation. The transection margin of a partial hepatectomy may be large, rendering it impractical for complete examination. In this setting, grossly positive margins should be microscopically confirmed and documented. If the margins are grossly free of tumor, judicious sampling of the cut surface in the region closest to the nearest identified tumor nodule is indicated. In selected cases, adequate random sampling of the cut surface may be sufficient. The histologic examination of the bile ducts at the cut margin is recommended to evaluate the lining epithelium for in situ carcinoma or dysplasia. If the neoplasm is found near the surgical margin, the distance from the margin should be reported. For multiple tumors, the distance from the nearest tumor should be reported.The TNM staging system of the American Joint Committee on Cancer and the International Union Against Cancer applies to all primary carcinomas of the intrahepatic bile ducts and mixed hepatocellular-cholangiocarcinomas.1 It does not apply to hepatic sarcomas or to metastatic tumors of the liver.According to the American Joint Committee on Cancer/International Union Against Cancer convention, the designation "T" refers to a primary tumor that has not been previously treated. The symbol "p" refers to the pathologic classification of the TNM, as opposed to the clinical classification, and is based on gross and microscopic examination. pT entails a resection of the primary tumor or biopsy adequate to evaluate the highest pT category, pN entails removal of nodes adequate to validate lymph node metastasis, and pM implies microscopic examination of distant lesions. Clinical classification (cTNM) is usually carried out by the referring physician before treatment during initial evaluation of the patient or when pathologic classification is not possible.Pathologic staging is usually performed after surgical resection of the primary tumor. Pathologic staging depends on pathologic documentation of the anatomic extent of disease, whether or not the primary tumor has been completely removed. If a biopsied tumor is not resected for any reason (eg, when technically unfeasible) and if the highest T and N categories or the M1 category of the tumor can be confirmed microscopically, the criteria for pathologic classification and staging have been satisfied without total removal of the primary cancer.For identification of special cases of TNM or pTNM classifications, the "m" suffix and "y," "r," and "a" prefixes are used. Although they do not affect the stage grouping, they indicate cases needing separate analysis.The "m" suffix indicates the presence of multiple primary tumors in a single site and is recorded in parentheses: pT(m)NM.The "y" prefix indicates those cases in which classification is performed during or after initial multimodality therapy (ie, neoadjuvant chemotherapy, radiation therapy, or both chemotherapy and radiation therapy). The cTNM or pTNM category is identified by a "y" prefix. The ycTNM or ypTNM categorizes the extent of tumor actually present at the time of that examination. The "y" categorization is not an estimate of tumor before multimodality therapy (ie, before initiation of neoadjuvant therapy).The "r" prefix indicates a recurrent tumor when staged after a documented disease-free interval and is identified by the "r" prefix: rTNM.The "a" prefix designates the stage determined at autopsy: aTNM.Intraductal papillary bile duct tumors may be identified in some patients with biliary obstruction and are classified as in situ tumors (Tis).The T classification depends on the number of tumor nodules and the presence or absence of blood vessel invasion.The TNM classification does not discriminate between multiple independent primary tumors, tumor satellite nodules, or intrahepatic metastasis from a single primary carcinoma. Vascular invasion includes either the gross involvement of large vessels or the microscopic involvement of small vessels identified on histologic examination. Major vascular invasion is defined as invasion of the branches of the main portal vein (right or left portal vein) or as invasion of 1 or more of the 3 hepatic veins (right, middle, or left).Direct invasion of adjacent organs, including colon, duodenum, stomach, common bile duct, portal lymph nodes, abdominal wall, and diaphragm, is considered T3 disease, not as metastases.Tumors with periductal growth pattern (diffuse longitudinal growth pattern along the intrahepatic bile ducts on both gross and microscopic examination) or mixed mass-forming and periductal-infiltrating growth pattern are classified as T4.Stage GroupingsLymph-Vascular InvasionLymph-vascular invasion indicates whether microscopic lymph-vascular invasion is identified in the pathology report. Lymph-vascular invasion includes lymphatic invasion, vascular invasion, or lymph-vascular invasion. By the American Joint Committee on Cancer/International Union Against Cancer convention, lymph-vascular invasion does not affect the T category indicating local extent of tumor unless specifically included in the definition of a T category.Lymph node metastases have consistently been identified as an important predictor of outcome for intrahepatic cholangiocarcinoma.1,2,9 Histologic examination of a regional lymphadenectomy specimen usually involves examination of 3 or more lymph nodes.The lymph node involvement pattern for intrahepatic cholangiocarcinomas varies with location in the liver (Figure 3). For biliary carcinomas arising in the right lobe of the liver (segments 5–8), the regional lymph nodes include the hilar (common bile duct, hepatic artery, portal vein, and cystic duct), periduodenal, and peripancreatic lymph nodes. For tumors arising in the left lobe, the regional lymph nodes are the hilar and gastrohepatic lymph nodes. Nodal involvement of the celiac, periaortic, or caval lymph nodes is considered to be distant metastasis (pM1).1Cirrhosis (Ishak score 6) or severe fibrosis (Ishak score 5, marked bridging fibrosis with occasional nodules)10 should be specifically reported because it has an adverse effect on outcome. The presence of underlying disease, such as primary sclerosing cholangitis, should be included in the pathology report.Approximately 10% of intrahepatic cholangiocarcinomas arise in the setting of chronic inflammatory conditions affecting the intrahepatic bile ducts.11 The most common risk factor for intrahepatic cholangiocarcinoma in the United States is biliary cirrhosis, generally in the setting of primary sclerosing cholangitis. In Asian countries, biliary parasites and recurrent pyogenic cholangitis are also etiologic factors. Recent studies suggest that hepatitis C infection, nonalcoholic fatty liver disease, obesity, and smoking are also risk factors for the development of this tumor.12,13The authors have no relevant financial interest in the products or companies described in this article.

Background and Aims: Biliary changes caused by cavernous transformation, which develops after portal vein thrombosis, are referred to as portal biliopathy. It may involve extrahepatic or intrahepatic bile ducts, or both. We aimed to reveal whether findings on Doppler ultrasound are related to changes seen on cholangiography in patients with portal vein thrombosis who developed cavernous transformation. Materials and Methods: A total of 22 patients [15 males, mean age 40,28±12,02 years] with portal biliopathy who underwent endoscopic retrograde cholangiopancreatography procedure for the relief of biliary obstruction were examined with Doppler ultrasound by radiologists who were blinded to the endoscopic retrograde cholangiopancreatography findings. The data from both examinations were compared, particularly the relationship of extrahepatic and intrahepatic main bile ducts with the site of thrombosis and the presence of collaterals. Results: We found biliary strictures in the right hepatic duct in 3 patients, in the left hepatic duct in 2 patients and in the extrahepatic bile ducts in all patients by endoscopic retrograde cholangiopancreatography. On the other hand, Doppler ultrasound showed that a significant number of patients had no biliary stricture even in the presence of dilated collateral veins accompanying the bile ducts (next to the extrahepatic bile duct in all patients, to the right hepatic duct in 12 patients and to the left hepatic duct in 9 patients). Conclusions: We found that there was no relation between the Doppler ultrasound findings of portal veins and collaterals and the site of the stricture detected by endoscopic retrograde cholangiopancreatography. It may be concluded that there are some factors other than compression of collateral veins that play a role in the pathogenesis of portal biliopathy.

The anatomic variations of the intrahepatic portal vein and bile duct were analyzed to evaluate the potential risk of left hepatectomy. A total of 210 cholangiograms and hepatic arterioportograms were performed in which the ramifications of the intrahepatic portal vein and bile duct were investigated. The orientation of the intrahepatic duct and portal vein were classified into five types. In 175 patients (83.33%), the intrahepatic portal vein and bile duct had the same anatomic classification. In 24 patients (11.43%), the right anterior or posterior intrahepatic duct drained into the left hepatic duct at the umbilical portion (type IV); there were only 15 patients (7.14%) whose portal veins fell into this category. All patients with type IV portal veins had type IV hepatic ducts, but there were 9/49 patients (18.36%) whose hepatic duct distribution belonged to type IV but their portal veins belonged to type II (6 cases) or III (3 cases). Without complete knowledge of the intrahepatic portal and biliary anatomy, insufficient portal perfusion and bile duct complications may result from the left hepatectomy operation. Preoperative portal vein evaluation or left portal vein clamping can provide significant information, but there are still 18.36% of patients where type IV biliary ducts were not detected in those with type II and III portal veins. Cholangiography is of paramount importance in these two groups of patients, as it can prevent inadvertent injury to the right intrahepatic ducts, which drain into the left intrahepatic duct. On the other hand, intraoperative ultrasonography is recommended to identify or exclude an aberrant portal vein if type VI biliary anatomy is detected during intraoperative cholangiography.

Cryptosporidiosis of the gastrointestinal tract associated with sclerosing cholangitis in the absence of documented immunodeficiency: Cryptosporidium parvum and sclerosing cholangitis in an immunocompetent child.

Objective To explore the value of ultrasound in differential diagnosis of hepatic hilar cyst in neonates and infants. Methods The ultrasonographic characteristics of 93 children with hilar cysts under 6 months of age admitted to the General Surgery Department of Beijing Children′s Hospital.Affiliated to Capital Medical University from January 2014 to December 2018 were retrospectively analyzed.According to the surgical results, they were divided into two groups: cystic biliary atresia (CBA) group and congenital choledochal cyst (CC) group, 21 cases in the CBA group and 72 cases in the CC group.The size of cyst in hepatic portal area, the shape of gallbladder in empty stomach, the expansion of intrahepatic and extrahepatic bile ducts, the deposition of biliary sludge and the communication between cyst and intrahepatic bile duct were observed by ultrasound. Results The cyst volume of CBA group was 0.4(0.1, 1.3)cm3, and that of CC group was 18.3(5.4, 50.3)cm3.The cyst volume of CBA group was significantly smaller than that of CC group (P<0.01); 19 cases had abnormal gallbladder morphology in CBA group and 4 cases in CC group (P<0.01); 1 case had biliary sludge in hilar cyst in CBA group and 51 cases had biliary sludge in cyst in CC group (P<0.01); no intrahepatic bile duct dilatation was observed in CBA group, and 54 cases had intrahepatic bile duct dilatation in CC group (P<0.01); no cyst communicated with intrahepatic bile duct in CBA group, and all cysts communicated with intrahepatic bile duct in CC group (P<0.01). Conclusion Gallbladder shape, cyst size, biliary sludge deposition, intrahepatic bile duct dilatation and the communication between cyst and intrahepatic bile duct can be used as sonographic features to differentiate CBA from CC. Key words: Ultrasound; Cystic biliary atresia; Choledochal cyst

IGG4-RELATED DISEASE PRESENTING AS A PULMONARY INFLAMMATORY PSEUDOTUMOR

IGG4-RELATED LUNG DISEASE MASKING LUNG CANCER

Introduction: Hepatolithiasis is defined as the presence of stones in the bile ducts proximal to the confluence of the right and left hepatic ducts, irrespective of the coexistence of stones in the common bile duct and/or gallbladder. The symptoms of hepatolithiasis may include epigastric or right upper quadrant pain, jaundice, fever, and gastrointestinal symptoms. This disease can cause cholestasis, cholangitis, abscesses, postobstructive atrophy, and liver cirrhosis.1 Although the etiology of hepatolithiasis is not exactly known, it is believed that nutrition, environmental and genetic factors, and surgical operations play different roles.2,3 It frequently occurs after a hepatic portoenterostomy (such as a Kasai), with bile stasis and (possibly) bile infections being the main causes of calculi formation. Previous studies have reported a 53% incidence of hepatolithiasis after Kasai operations.3 The aim of hepatolithiasis treatment is to extract the stones and regain biliary drainage. Liver resection as well as less invasive procedures, such as percutaneous transhepatic cholangioscopy, peroral cholangioscopy, and extracorporeal shock wave lithotripsy, may be used for the treatment of hepatolithiasis.4,5 However, these procedures are not always effective, because bile duct anatomy differs, and they may not significantly reduce the intrahepatic stone burden. There is a risk of liver failure after an open liver resection, which is performed if there are multiple large stones, and a stone-free condition cannot be provided for the patient through other treatments. Therefore, an “ultramini percutaneous hepatolithotomy” (UM-PHL) was planned by the Necmettin Erbakan University Stone Diseases Diagnosis and Treatment Center based on decisions made by a urology, interventional radiology, and general surgery clinical team. It was hypothesized that it could provide less morbidity and a higher stone-free condition ratio. Materials and Methods: The patient information details and surgical technique are shown in the video provided. Results and Conclusion: Initially, the left intrahepatic bile ducts were accessed and the obstructing stones were extracted in 126 minutes, whereas the right intrahepatic bile ducts were accessed and treated in 103 minutes. This procedure was performed as one operation, and all of the stones in the patient's intrahepatic bile ducts were removed (stone free). At the 1 month follow-up, liver function tests were performed, which showed normal values, and no stones were seen in the intrahepatic bile ducts through ultrasonography. All of the patient's complaints and symptoms had disappeared, and she did not have any problems during any of her postoperative follow-ups. In postoperative cholangiography and abdominal ultrasonography, there were neither mechanical obstruction findings at the anastomoses nor stones. Pharmacotherapy is not recommended in the evidence-based clinical practice guidelines for cholelithiasis (2016)6 for hepatolithiasis treatment, so this patient was not started on any medications. The previously used methods were not effective in this patient, therefore, UM-PHL was performed. Our hepatolithiasis case is the first case in the literature from whom large stones were extracted using lithotomy with minimal width access, and a complete stone-free state was provided. In hepatolithiasis patients with a high stone burden, an UM-PHL is a safe and effective method for removing stones. No competing financial interests exist. Runtime of video: 9 mins 13 secs

Diagnosis and Management of Primary Sclerosing Cholangitis

IgG4-related disease is characterized by IgG4-positive plasmacyte infiltration into various organs, but its etiology is not unknown. To elucidate the etiology of IgG4-related disease. We experienced an interesting case of IgG4-related lung disease complicated by chronic EB virus infection. A 70-year-old male visited our hospital due to failure of pneumonia treatment. Chest computed tomography (CT) showed consolidation in the right middle field and slight mediastinal lymphadenopathy in the subcarinal region. Lung consolidation improved with antibiotics; subcarinal lymphadenopathy progressed after 4 months. Malignant lymphoma was suspected given elevated sIL2-R levels (1862 U/mL). Patchy ground glass opacities appeared in the bilateral lung field just before surgical biopsy. He was diagnosed with IgG4-related lung disease after inspection of a pathological specimen obtained from the right upper lung and right hilar lymph node. EB virus-infected cells were also detected in the lymph node. Blood examination revealed EB virus viremia, but the patient did not present with symptoms or organ involvement. This led to a diagnosis of asymptomatic chronic EB virus infection. Recent studies have suggested an association between EB virus infection and IgG4-related diseases in the pathological exploration of surgically resected lymph nodes. Our case is the first case of IgG4-related lung disease in which EB virus infection was both pathologically and clinically proved. The present case is of particular interest in view of this newly reported association, and may serve as a fundamental report for future studies connecting EB virus infection with IgG4-related diseases.

Immunoglobulin (Ig) G4 (IgG4) related disease is a recently described clinical entity that can involve multiple organs. It is an autoimmune disorder characterized by a dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. A key distinguishing factor is its dramatic response to steroid therapy. It is crucial to differentiate IgG4-related cholangiopathy from its mimickers such as primary biliary cholangitis (PBC), secondary biliary cholangitis (SBC), primary sclerosing cholangitis (PSC), secondary sclerosing cholangitis (SSC) and cholangiocarcinoma, as treatment modalities and outcomes of IgG4-related cholangiopathy differ significantly from these disorders. A 72 year-old man presented as a transfer with obstructive jaundice and was found to have elevated total bilirubin (5 mg/dL), alkaline phosphatase (315 mg/dL) and liver enzymes (AST of 77 mg/dL and ALT of 45 mg/dL). MRI of the abdomen revealed contrast filling the biliary tree and ERCP showed a stricture extending from the bifurcation of the hepatic duct into the right hepatic duct. He also had sausage-like strictures of the intra hepatic ducts, which were concerning for cholangiocarcinoma. Percutaneous transhepatic cholangiogram revealed a hepatic duct bifurcation stricture that involved the right intra hepatic duct. The differential diagnosis included cholangiocarcinoma, primary biliary cholangitis (PBC), secondary biliary cholangitis (SBC), PSC, inflammatory myofibroblastic tumor, and IgG4-related cholangiopathy. Due to concerns for hilar cholangiocarcinoma by cholangiography, the patient underwent right hepatectomy and left hepaticojejunostomy. Shown are gross images of the right lobe of the liver (Figures 1A and 1B), with an arrow indicating the intra-hepatic bile duct and arrowhead indicating the hilar mass (Figure 1B). On microscopy, there was periductal fibrosis (arrow), edema (arrowhead) and a rich lymphoplasmacytic inflammatory infiltrate (black arrow) (Figure 1C). A portal vein was found to be obscured by inflammatory cells (Figure 1D). There were areas of inflammatory cell encroachment upon portal veins. Obliterative phlebitis, or occlusion of portal veins by inflammatory cell infiltrates were evident. There was storiform fibrosis with notable eosinophils. This particular case demonstrated bile duct involvement and morphologically greater than 40% of plasma cell infiltrates were positive for IgG4. The patient subsuquently improved with oral steroid therapy.FigureTable: Table. Potential treatment regimens for IgG4-related disease