UNASSIGNED: Whether a dilated intrahepatic bile duct (IHBD) has any effect on the prognosis of choledochal cyst (CC) remains controversial. We aimed to summarize the clinical characteristics and prognosis of CC with IHBD dilatation.
UNASSIGNED: One hundred ninety-two children diagnosed with CC were identified, including 127 without IHBD dilatation (group A) and 65 with IHBD dilatation (group B). A retrospective analysis was performed to explore the clinical characteristics and prognosis of CC with IHBD dilatation based on clinical indices, symptoms, and complications.
UNASSIGNED: Compared with group A, incidences of jaundice and fever were higher in group B (P = 0.010 and P = 0.033). Preoperative total bilirubin, direct bilirubin, and indirect bilirubin were increased in group B compared to group A (P = 0.005, P < 0.001, and P = 0.014), as were preoperative ALT, AST, γ-GT, and total bile acid (P = 0.006, P = 0.025, P < 0.001, and P = 0.024). The risk of liver fibrosis or cirrhosis was significantly increased for group B compared with group A (P = 0.012) and also occurred earlier in group B (P = 0.006). In the dilated IHBDs, 95.4% (62 of 65) recovered to normal, and more than half of dilated IHBDs (37 of 65) recovered to normal in 1 week.
UNASSIGNED: Most IHBDs can recover to normal postoperatively in a short time, and proactive treatment is recommended for CC patients with IHBD dilatation for significant abnormal liver functions.

Background and Objectives: Gallbladder cancer is a rare type of cancer, with aggressive clinical behavior. Limited treatment options provide poor survival prognosis. We aimed to investigate the incidence, mortality trends, and survival of gallbladder and extrahepatic bile duct cancer in Lithuania between 1998 and 2017. Materials and Methods: The study was based on the Lithuanian Cancer Registry database. The study included all cases of cancer of the gallbladder and extrahepatic bile ducts reported to the Registry in the period 1998-2017. Age-specific and age-standardized incidence rates were calculated. In addition, 95% confidence intervals for APC (Annual Percent Change) were calculated. Changes were considered statistically significant if p was <0.05. Relative survival estimates were calculated using period analysis according to the Ederer II method. Results: Age-standardized rates for gallbladder cancer and extrahepatic bile duct cancer among females decreased from 3.91 to 1.93 cases per 100.000 individuals between 1998 and 2017, and from 2.32 to 1.59 cases per 100.000 individuals between 1998 and 2017 among males. The highest incidence rates were found in the 85+ group with 27.5/100,000 individuals in females and 26.8/100,000 individuals in males. The 1-year as well as 5-year relative survival rates of both genders were 34.29% (95% CI 32.12-36.48) and 16.29% (95% CI 14.40-18.27), respectively. Conclusions: Incidence and mortality from gallbladder and extrahepatic bile duct cancer decreased in both sexes in Lithuania. Incidence and mortality rates were higher in females than in males. Relative 1-year and 5-year survival rates showed a steady increase during the study period among males and females.

Congenital duplication of the extrahepatic bile duct (DEBD) is an unusual anomaly of the biliary system. It occurs due to inability of the embryological duplex biliary system to regress. DEBD has various subtypes depending on the morphology and opening of the aberrant common bile duct. It can have distinct complications. We encountered a 38-year-old lady who experienced pain in the right upper abdomen along with a low-grade fever. Magnetic resonance cholangiopancreatography revealed DEBD with multiple calculi in the right hepatic duct (ductolithiasis) and joining of the right hepatic duct with the left hepatic duct in the intrapancreatic region. Endoscopic retrograde cholangiography failed to clear the calculi from the right duct. They were then managed by common bile duct exploration and roux-en-Y right hepaticojejunostomy for biliary drainage. Her postoperative period was uneventful. She is currently doing well after three months of follow-up. Hence, a proper preoperative delineation of such rare anomalies is essential. It could avoid inadvertent injury to the bile duct and operative complications.

This study aims to evaluate the relation between the cystocholedochal angle (SCA) and choledocholithiasis. The data of 3.350 patients were reviewed retrospectively and a total of 628 patients who met the criteria were included in the study. The patients included in the study were divided into three groups as patients with choledocholithiasis (Group I), patients with only cholelithiasis (Group II), and patients without gallstones as control group (Group III). Measurements of SCA, cystic, bile, and common hepatic ducts (CHDs) were made on magnetic resonance cholangiopancreatography (MRCP) images. Laboratory findings and demographic characteristics of the patients were also recorded. Of the patients included in the study 64.2% were female, 35.8% were male, and their age ranged from 18 to 93 (mean 53.37 ± 18.87 years). While the mean SCA values of all patient groups were 35.44° ± 10.44°, the mean length of cystic, bile and CHDs were 28.91 ± 9.30, 40.28 ± 12.91, 27.09 ± 9.68 mm respectively. All measurements were higher in Group I in comparison to other groups, whereas all measurements of Group II were higher than those of Group III (p < 0.001). Statistical analysis suggests that a SCA of 33.5° and above is an important criterion for diagnosis of choledocholithiasis. Increase of SCA raises the likelihood of choledocholithiasis, as it facilitates the passage of stones from gallbladder into the bile ducts. This is the first study to compare SCA in patients with choledocholithiasis and those with only cholelithiasis. Therefore, we think that this study is important and will be a guide for clinical evaluation.

UNASSIGNED: Cholangiocarcinoma is a malignant neoplasm with a difficult diagnosis and unfavorable prognosis that can appear anywhere in the biliary tree. Anatomically, it can be classified as intra- or extrahepatic, the latter being the most common primary malignancy of the biliary tract. Although bone tissue is the third most common target for metastases in general, metastatic lesions from cholangiocarcinomas are uncommon in the head and neck.
METHODS: A 33-year-old female patient undergoing cancer treatment for cholangiocarcinoma was referred to the Oral and Maxillofacial Surgery and Traumatology Service (CTBMF) of the São Vicente de Paulo Hospital in Passo Fundo, Rio Grande do Sul, Brazil, for interdisciplinary follow-up. She presented trismus, dysphagia, pain, necrosis and drainage in the mental region, and an increased volume on the right side of the mandibular body in the region of teeth 47 and 48. An incisional biopsy was performed in this bulging region. The histopathological diagnosis was poorly differentiated carcinoma. The patient was followed-up weekly by the CTBMF team, receiving analgesia and palliative treatment for the extra- and intraoral lesions, controlling opportunistic infections, and improving food intake.
CONCLUSIONS: Metastatic cholangiocarcinomas in the mandible are rare, hence their diagnosis is often late, which may disfavor the prognosis of these conditions.
CONCLUSIONS: Carefully examining patients with mandibular bone lesions is strongly suggested, especially patients with known or previous concomitant malignancy.

Introduction: Sclerosing Extramedullary Hematopoietic Tumor (SEHT) is a very rare lesion associated with chronic myeloproliferative disorders (CMPD). SEHT can mimic morphologically, both macroscopically and microscopically, a wide variety of tumors/lesions. Case presentation: We present the case of a female patient diagnosed with gallstones for which surgery was decided. Intraoperatively, a malignant tumor of extrahepatic bile ducts was suspected. A frozen section examination raised the suspicion of a mesenchymal tumor or an inflammatory pseudotumor. The histological evaluation of the permanent sections, supplemented with an immunohistochemical investigation (IHC), was the one that established the diagnosis of SEHT, based on the presence of areas of sclerosis, atypical CD31+ megakaryocytes, myeloid and erythroid elements. Conclusions: The authors present the difficulties of a morphological diagnosis on the frozen section and on permanent sections in the absence of relevant clinical information and make a review of the literature data dedicated to the subject.

Several inflammatory conditions of the bile ducts cause strictures that prevent the drainage of bile into the gastrointestinal tract. Non-pharmacological treatments to re-establish bile flow include plastic or self-expanding metal stents (SEMs) that are inserted in the bile ducts during endoscopic retrograde cholangiopancreatography (ERCP) procedures. The focus of this study was to 3D print an anatomically accurate model of the extrahepatic bile ducts (EHBDs) with tissue-like mechanical properties to improve in vitro testing of stent prototypes. Following generation of an EHBD model via computer aided design (CAD), we tested the ability of Formlabs SLA 3D printers to precisely print the model with polymers selected based on the desired mechanical properties. We found the printers were reliable in printing the dimensionally accurate EHBD model with candidate polymers. Next, we evaluated the mechanical properties of Formlabs Elastic (FE), Flexible (FF), and Durable (FD) resins pre- and post-exposure to water, saline, or bile acid solution at 37 °C for up to one week. FE possessed the most bile duct-like mechanical properties based on its elastic moduli, percent elongations at break, and changes in mass under all liquid exposure conditions. EHBD models printed in FE sustained no functional damage during biliary stent deployment or when tube connectors were inserted, and provided a high level of visualization of deployed stents. These results demonstrate that our 3D printed EHBD model facilitates more realistic pre-clinical in vitro testing of biliary stent prototypes.

BACKGROUND: Intraductal papillary neoplasm of the bile duct (IPNB) is a recently defined entity and its clinical characteristics and classifications have yet to be established. We aimed to clarify the clinical features of IPNB and determine the optimal morphological classification criteria.
METHODS: From 2003 to 2016, 112 patients with IPNB who underwent surgery were included in the analysis. After pathologic reexamination by a specialized biliary-pancreas pathologist, previously suggested morphological and anatomical classifications were compared using the clinicopathologic characteristics of IPNB.
RESULTS: In terms of histologic subtypes, most patients had the intestinal type (n = 53; 48.6%) or pancreatobiliary type (n = 33; 30.3%). The simple \"modified anatomical classification\" showed that extrahepatic IPNB comprised more of the intestinal type and tended to be removed by bile duct resection or pancreatoduodenectomy. Intrahepatic IPNB had an equally high proportion of intestinal and pancreatobiliary types and tended to be removed by hepatobiliary resection. Morphologic classifications and histologic subtypes had no effect on survival, whereas a positive resection margin (75.9% vs. 25.7%; P = 0.004) and lymph node metastasis (75.3% vs. 30.0%; P = 0.091) were associated with a poor five-year overall survival rate. In the multivariate analysis, a positive resection margin and perineural invasion were important risk factors for survival.
CONCLUSIONS: IPNB showed better long-term outcomes after optimal surgical resection. The \"modified anatomical classification\" is simple and intuitive and can help to select a treatment strategy and establish the proper scope of the operation.

A novel type of interstitial tissue cells in the biliary tree termed telocytes (TCs), formerly known as interstitial Cajal-like cells (ICLCs), exhibits very particular features which unequivocally distinguish these cells from interstitial cells of Cajal (ICCs) and other interstitial cell types. Current research substantiates the existence of TCs and ICCs in the biliary system (gallbladder, extrahepatic bile duct, cystic duct, common bile duct and sphincter of Oddi). Here, we review the distribution, morphology and ultrastructure of TCs and ICCs in the biliary tree, with emphasis on their presumptive roles in physiological and pathophysiological processes.

Adenomyomatous hyperplasia is a reactive malformation or non-neoplastic tumor-like lesion frequently observed in the gallbladder, stomach, duodenum and jejunum, but rare in the extrahepatic bile duct. A 42-year-old man with epigastric discomfort had a stricture in the common bile duct on initial CT scans. Initially, it was regarded as a malignant lesion with some evidence, but histopathologic examinations of multiple biopsies obtained by multiple sessions of endoscopic retrograde cholangiopancrea-tography showed no evidence of malignancy. The patient had undergone the pylorus preserving pancreaticoduodenectomy because of the possibility of malignancy; however, the final diagnosis was adenomyomatous hyperplasia. It is important to distinguish a malignancy from benign biliary stricture with endoscopic biopsies. Surgery for suspected biliary malignancy often reveals benign lesions. Therefore, a correct diagnosis is important before deciding upon treatment of bile duct stricture. In conclusion, in younger patients with bile duct stricture where there is no evidence of histologic malignancy despite multiple biopsies, the possibility of benign disease such as adenomyomatous hyperplasia should be considered, to avoid unnecessary radical surgery.