Diseases of bile duct in children.

Several diseases originate from bile duct pathology. Despite studies on these diseases, certain etiologies of some of them still cannot be concluded. The most common disease of the bile duct in newborns is biliary atresia, whose prognosis varies according to the age of surgical correction. Other diseases such as Alagille syndrome, inspissated bile duct syndrome, and choledochal cysts are also time-sensitive because they can cause severe liver damage due to obstruction. The majority of these diseases present with cholestatic jaundice in the newborn or infant period, which is quite difficult to differentiate regarding clinical acumen and initial investigations. Intraoperative cholangiography is potentially necessary to make an accurate diagnosis, and further treatment will be performed synchronously or planned as findings suggest. This article provides a concise review of bile duct diseases, with interesting cases.

Biliary microhamartomas (von Meyenburg complexes), liver metastasis simulators: a series of eight cases.

BACKGROUND: Von Meyenburg complexes are benign hamartomatous lesions, they are part of the spectrum of ductal plate malformations. They are rare, reported in 0.35-5.6% of the general population, predominantly in adults, with no clear predilection for sex. OBJECTIVE: To present the clinical characteristics of Von Meyenburg complexes in our region. METHOD: We searched all cases with diagnosis of Von Meyenburg complexes in a period from 2012 to 2022, in our institutions. RESULTS: We identified eight cases, with an average age of 59.25 years, with a predominance of females and with one case associated with gastric carcinoma. CONCLUSIONS: It is important to adequately recognize this entity, since due to its multifocal nature it can easily simulate metastasis, additionally, and its presence does not rule out other synchronous neoplasms.

ANTECEDENTES: Los complejos de Von Meyenburg son lesiones hamartomatosas benignas que forman parte del espectro de las malformaciones de la placa ductal. Son poco frecuentes, se reportan en un 0.35-5.6% de la población general, predominantemente en adultos, sin clara predilección por un sexo. OBJETIVO: Presentar las características clínicas de los complejos de Von Meyenburg en nuestro medio. MÉTODO: Se buscaron todos los casos con diagnóstico de complejos de Von Meyenburg en nuestras instituciones entre 2012 y 2022. RESULTADOS: Identificamos ocho casos, con un promedio de edad de 59.25 años, con predominio por el sexo femenino y con un caso asociado a carcinoma gástrico. CONCLUSIONES: Es importante reconocer y diagnosticar adecuadamente esta afección, ya que por su naturaleza multifocal fácilmente puede simular metástasis, y además su presencia no descarta otros procesos neoplásicos sincrónicos.

Biliary hamartoma: a report of two cases.

Biliary hamartoma, also known as biliary micro hamartoma or Von Meyenburg complex, is a rare benign liver lesion, thought to be a ductal plate malformation rather than a true neoplasm. It is often seen incidentally on imagery or surgery as multiple small subcapsular nodules, scattered throughout the liver, making it likely to be mistaken for metastatic nodules. The histological presentation can also be deceptive, leading to the misdiagnosis of an adenocarcinoma of hepato-biliary differentiation or a metastasis. We hereby present two cases of biliary hamartoma, found incidentally on imagery and surgery, the first one in a 94-year-old woman, and the second in a 48-year-old man, which was initially misdiagnosed as an adenocarcinoma, along with a discussion of key clinical and pathological findings to help avoid this diagnostic pitfall.

Non-invasive diagnosis and follow-up of benign liver tumours.

Hepatocellular benign liver tumours are mainly developed on normal liver and include hepatic hemangioma, focal nodular hyperplasia and hepatocellular adenoma from the most frequent to the less frequent. The diagnosis of hepatic hemangioma and of simple hepatic biliary cysts can be performed using non-invasive criteria using liver ultrasonography or contrast enhanced MRI. Most of the time the diagnosis of focal nodular hyperplasia can be achieved using contrast-enhanced ultrasonography or contrast enhanced MRI with an additional value of hepatobiliary contrast-agent in this setting. Rarely, if a doubt persists, a tumour and non-tumour liver biopsy can be required in order to establish the diagnosis. As hepatic hemangioma, simple hepatic biliary cysts and focal nodular hyperplasia are not associated with complications, they don't require any treatments or follow-up. Hepatocellular adenomas are mainly diagnosed at histology on surgical samples or liver biopsy even if some radiological features are highly suggestive of several subtypes of hepatocellular adenomas. Finally, the management of hepatocellular adenomas should be guided according to the tumour size, gender but also to the molecular subtypes.

Routine versus selective intraoperative cholangiography during cholecystectomy: systematic review, meta-analysis and health economic model analysis of iatrogenic bile duct injury.

BACKGROUND: Bile duct injury (BDI) is a severe complication following cholecystectomy. Early recognition and treatment of BDI has been shown to reduce costs and improve patients' quality of life. The aim of this study was to assess the effect and cost-effectiveness of routine versus selective intraoperative cholangiography (IOC) in cholecystectomy. METHODS: A systematic review and meta-analysis, combined with a health economic model analysis in the Swedish setting, was performed. Costs per quality-adjusted life-year (QALY) for routine versus selective IOC during cholecystectomy for different scenarios were calculated. RESULTS: In this meta-analysis, eight studies with more than 2 million patients subjected to cholecystectomy and 9000 BDIs were included. The rate of BDI was estimated to 0.36 per cent when IOC was performed routinely, compared with to 0.53 per cent when used selectively, indicating an increased risk for BDI of 43 per cent when IOC was used selectively (odds ratio 1.43, 95 per cent c.i. 1.22 to 1.67). The model analysis estimated that seven injuries were avoided annually by routine IOC in Sweden, a population of 10 million. Over a 10-year period, 33 QALYs would be gained at an approximate net cost of €808 000 , at a cost per QALY of about €24 900. CONCLUSION: Routine IOC during cholecystectomy reduces the risk of BDI compared with the selective strategy and is a potentially cost-effective intervention.

A deep learning-based system for bile duct annotation and station recognition in linear endoscopic ultrasound.

BACKGROUND: Detailed evaluation of bile duct (BD) is main focus during endoscopic ultrasound (EUS). The aim of this study was to develop a system for EUS BD scanning augmentation. METHODS: The scanning was divided into 4 stations. We developed a station classification model and a BD segmentation model with 10681 images and 2529 images, respectively. 1704 images and 667 images were applied to classification and segmentation internal validation. For classification and segmentation video validation, 264 and 517 videos clips were used. For man-machine contest, an independent data set contained 120 images was applied. 799 images from other two hospitals were used for external validation. A crossover study was conducted to evaluate the system effect on reducing difficulty in ultrasound images interpretation. FINDINGS: For classification, the model achieved an accuracy of 93.3% in image set and 90.1% in video set. For segmentation, the model had a dice of 0.77 in image set, sensitivity of 89.48% and specificity of 82.3% in video set. For external validation, the model achieved 82.6% accuracy in classification. In man-machine contest, the models achieved 88.3% accuracy in classification and 0.72 dice in BD segmentation, which is comparable to that of expert. In the crossover study, trainees' accuracy improved from 60.8% to 76.3% (P < 0.01, 95% C.I. 20.9-27.2). INTERPRETATION: We developed a deep learning-based augmentation system for EUS BD scanning augmentation. FUNDING: Hubei Provincial Clinical Research Center for Digestive Disease Minimally Invasive Incision, Hubei Province Major Science and Technology Innovation Project, National Natural Science Foundation of China.

Differences in post-operative complications after reconstruction for congenital biliary dilatation in a single institution-Roux-en-Y hepaticojejunostomy versus hepaticoduodenostomy.

BACKGROUND: The standard surgical procedure for congenital biliary dilatation (CBD) consists of excision of extrahepatic bile duct and Roux-en-Y hepaticojejunostomy (HJ). However, alternative reconstructive operations for CBD includes hepaticoduodenostomy (HD). We compared postoperative complications and therapeutic outcomes of these two operations at our institution. METHODS: From 1981 to 2009, there were 23 traceable patients who underwent operation for CBD, They were divided into an HJ Group (n = 15) and an HD Group (n = 8). Demographic and outcome data were compared. RESULTS: There were no significant differences in postoperative complications (cholangitis, pancreatitis, and anastomotic stenosis) and current blood test data (AST, ALT, Total Bilirubin, Direct Bilirubin, Amylase) between the two groups. Current abdominal pain and carcinogenesis were not observed in either group. Intrahepatic stones occurred in one patient in each group, both > 25 years post-operation. CONCLUSION: HD is considered to be an acceptable reconstruction method compared to HJ, based on our results. There has been a suggestion that inadequate diversion in HD might increase the risk of cholangiocarcinoma. To date, in this series, that has not happened.

A Case of Vanishing Bile Duct Syndrome after Drug-Induced Liver Injury Caused by Pelubiprofen.

Vanishing bile duct syndrome (VBDS) is a rare disease characterized by ductopenia and cholestasis, and is linked to immunological damage to the bile duct system. VBDS can be triggered by infection, ischemia, autoimmune diseases, adverse drug reactions, and humoral factors associated with malignancy. A few cases of VBDS associated with nonsteroidal anti-inflammatory drug-related drug-induced liver injury (DILI) have been reported. Here, we report a case of a 29-year-old patient who developed DILI that progressed to VBDS after the administration of pelubiprofen.

Obstructive cholangiopathy patient caused by cytomegalovirus and Kaposi sarcoma in a person living with HIV and hepatitis C.

We present the case of a 28-year-old man with recently-diagnosed human immunodeficiency virus and hepatitis C virus infection. He developed obstructive cholangiopathy secondary to cytomegalovirus and Kaposi sarcoma, both diagnosed by endoscopic retrograde cholangiopancreatography and biopsies. He received antiretroviral therapy, chemotherapy and valganciclovir with full recovery.

The Role of Resolvin D1 in the Differential Diagnosis of the Cholangiocarcinoma and Benign Biliary Diseases.

BACKGROUND: The discrimination of malignant biliary strictures from benign biliary diseases (BBDs) is challenging and complicated. We aimed to investigate whether Resolvin D1 (RvD1) would aid in the discrimination of cholan-giocarcinoma (CCA) from BBDs. METHODS: Thirty-one patients with CCA, 27 patients with BBD, and 30 healthy controls were enrolled in this cross-sectional study. The diagnosis of CCA was based on results obtained from abdominal USG, MRCP, abdominal CT, endosonography, and tumor markers, including CEA and CA 19-9. Histopathological evaluation was performed in the majority of patients, and the final diagnosis was based on surgery or biopsy results. RvD1, CEA, and CA 19-9 were analyzed in all patients with CCA and BBD. RESULTS: RvD1 was significantly lower in those with CCA compared to patients with BBD and healthy controls. In addition, CEA and Ca 19-9 levels were significantly higher in the CCA group than the BBD group (p < 0.001). RvD1 concentration, CA 19-9 concentration, and total bilirubin level were found to be correlated with tumor stage (r = -0.702, 0.390, and 0.569, respectively). ROC curve analysis revealed that an RvD1 concentration of < 380 ng/mL (AUC: 0.783, 95% CI: 0673 - 0.893, p < 0.001) and CA 19-9 concentration of > 94.5 U/mL (AUC: 0.94, 95% CI: 0.898 - 0.998, p < 0.001) could be used to discriminate patients with CCA from those with BBD. CONCLUSIONS: Resolvin D1 and CA 19-9 levels might be used to effectively discriminate between BBD and CCA. Moreover, both RvD1 and CA 19-9 levels are associated with the stage of CCA, indicating that they may also be used in assessing disease progression.

Evolution of bile duct repair in a low and middle-income country (LMIC): a comparison of diagnosis, referral, management and outcomes in repair of bile duct injury after laparoscopic cholecystectomy from 1991 to 2004 and 2005-2017.

BACKGROUND: There is a paucity of data from the developing world regarding laparoscopic cholecystectomy (LC) bile duct injuries (BDIs), despite the fact that most of the world's population live in a developing country. We assessed how referral patterns, management and outcomes after LC-BDI repair have evolved over time in patients treated at a tertiary referral center in a low and middle-income country (LMIC). METHODS: Patients with LC-BDIs requiring hepaticojejunostomy were identified from a prospective database. Clinical characteristics, geographic distance from referral hospital, timing of referral and repair, and post-operative outcomes were compared in two cohorts treated during 1991-2004 and 2005-2017. RESULTS: Of 125 patients, 32 underwent repair in the early period, 93 in the latter. There was no difference in demographic or clinical characteristics, but a 45.6% increase in geographically distant referrals in the 2005-2017 period. Time from diagnosis to referral and referral to repair increased significantly (p = 0.031, p < 0.001), necessitating more intermediate repairs. Despite this, the number of severe complications decreased (p = 0.022) while long-term outcomes remained unchanged. CONCLUSION: In this study from an LMIC, geographic and logistic constraints necessitated deviation from accepted algorithms devised for well-resourced countries. When appropriately adapted, results comparable to those reported from developed countries are achievable.

Incisionless fluorescent cholangiography (IFC): a pilot survey of surgeons on procedural familiarity, practices, and perceptions.

BACKGROUND: Incisionless fluorescent cholangiography (IFC) has recently been proven feasible, safe, and efficacious as an intraoperative procedure to help identify extrahepatic bile ducts during laparoscopic cholecystectomies (LC). We conducted a pilot survey of 51 surgeons attending an international conference who perform endoscopic cholecystectomies to identify their typical LC practices, and perceptions of IFC. METHODS: An international panel of ten IFC experts, all with > 500 prior IFC procedures and related research publications, convened during the 4th International Congress of Fluorescence-Guided Surgery in Boca Raton, Florida in February 2017. The panel was charged with developing questions about LC practices and experience with IFC, and perceptions regarding its advantages, barriers to use, and indications. These questions then were asked to other congress attendees during one of the didactic sessions using an online polling application. Attendees, who ranged from zero to considerable experience performing IFC, accessed the survey via their portable devices. RESULTS: Of the 51 survey participants, 51% were from North America; 77% identified themselves as general/minimally invasive surgeons, and roughly 60% performed under 50 cholecystectomies/year. Only 12% performed routine intraoperative cholangiography (IOC), while 72.3% routinely performed critical safety reviews. Thirty-five percent estimated that their institution's laparoscopic-to-open surgery conversion rate was > 1% during LC. Roughly 95% of respondents felt that surgeons should have access to a noninvasive method for evaluating extrahepatic biliary structures; 84% felt that the most advantageous characteristic of IFC is the lack of any biliary-tree incision; and 93.3% felt that IFC would have considerable educational value in surgical training programs; and 78% felt that any surgeon who performs LC could benefit. CONCLUSIONS: Surgeons who participated in our survey overwhelmingly recommended the routine use of IFC during laparoscopic cholecystectomy as a complimentary imaging technique. Prospective randomized clinical trials remain necessary to determine whether IFC reduces the incidence of bile duct injuries and other LC complications.

Biliary calprotectin, lactoferrin and dimeric pyruvate kinase after liver transplantation are associated with biliary damage and graft survival in a case-control study.

BACKGROUND: After liver transplantation (LT), biliary complications are associated with reduced graft survival. We tested inflammation markers for their association with biliary damage and graft loss in bile. MATERIAL AND METHODS: The study design was a retrospective case-control study. Calprotectin, lactoferrin and pyruvate kinase were measured in endoscopically retrieved bile with ELISA. RESULTS: Calprotectin and lactoferrin were significantly higher in bile of ischemic-type biliary lesions and donor duct non-anastomotic strictures than in control, bile leakage, Cytomegalovirus infection, anastomotic stricture or acute cellular rejection patients (p<0.001) independent of serum liver values at endoscopy. Calprotectin (p=0.02) was independently associated with retransplantation free survival in multivariate analysis, as was γGT (p=0.03) but not ERC radiographic classification of the bile duct or cold ischemia time. CONCLUSION: Calprotectin and lactoferrin are bile markers for biliary damage and are associated with re-transplantation free survival. They can differentiate progressive biliary damage from non-biliary liver value alterations after LT.

Multicenter database registry for endoscopic retrograde cholangiopancreatography: Japan Endoscopic Database Project.

BACKGROUND AND AIM: Few studies have reported on a national, population-based endoscopic retrograde cholangiopancreatography (ERCP) database. Hence, in 2015, we established a multicenter ERCP database registry, the Japan Endoscopic Database (JED) Project in preparation for a nationwide endoscopic database. The objective the present study was to evaluate this registry before the establishment of a nationwide endoscopic database. METHODS: From 1 January 2015 to 31 March 2017, we collected and analyzed the ERCP data of all patients who underwent ERCP in four participating centers in the JED Project based on the JED protocol. RESULTS: Four centers carried out 4104 ERCP on 2173 patients. Data entry of ERCP information (age, 100%; gender, 100%; American Society of Anesthesiologists Physical Status Classification System, 74.5%; scope, 92.7%; time to ERCP, 100%; antithrombotic drug information, 55.0%; primary selective common bile duct [CBD] cannulation methods, 73.0%; number of attempts at primary selective CBD cannulation, 67.6%; overall selective CBD cannulation methods, 68.9%; ERCP procedure time, 66.3%; fluoroscopy time, 65.1%; adverse events, 74.9%; serum amylase levels 1 day post-ERCP, 36.5%) was accurately extracted from the four centers. Success rate of CBD cannulation by level of ERCP difficulty was 98.5%, 99.0%, and 96.4% in grades 1, 2, and 3, respectively. Complication rate by overall selective CBD cannulation method was 5.6%, 7.6%, and 10.5% in the contrast-assisted technique, guidewire-assisted technique, and cross-over method, respectively. CONCLUSION: Data from this evaluation of the JED Project, a multicenter ERCP database registry, suggest the feasibility of establishing a nationwide ERCP database and its challenges.

Risk factors for complications after endoscopic retrograde cholangiopancreatography A referral Center experience.

AIM: Endoscopic retrograde cholangiopancreatography (ERCP) is an important diagnostic and therapeutic tool in patients with hepato-pancreatobiliary diseases. In the present study, we sought to determine predictors of post-ERCP complications at our institution. METHODS: A retrospective analysis of patients who underwent ERCP in between January 2010 and November 2011 was done. Demographics, indications, ERCP findings, success rate, complications and the need of repeat procedure were evaluated with special emphasis on the difficulty in cannulation procedure, the primary etiology of the disease (benign/malign) and age. Chi-square analysis was applied for statistical analysis. The differences were considered statistically significant, if the p value was less than 0.05. RESULTS: A total of 112 ERCP was performed in 81 patients. Thirty-eight were male (46.9 %) and 43 were female (53.1%). Mean age was 61.3 (range 17-88), and 31 patients was seventy years and older (38.3 %). Complications were detected in 28 patients (34.6 %). Nine cases with difficult or unsuccessful cannulation (69.2 %) had complications (p=0.001). Patients with benign diseases showed less complications (21/70), in comparison with those with malignancies (7/11) (30 % vs. 63.6 %, respectively; p<0.05). Complication rate in patients 70 years and older was 32.2 % (n=10) compared to 35.3 % in patients younger than 70 (n= 18) (p<0.05). CONCLUSION: Risk factors such as difficult or incomplete cannulation and malignancy are considered as possible predisposing factors for complications. Age is an independent factor. KEY WORDS: Endoscopic cholangiopancreatography, Risk factors.