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.

Lesiones de vía biliar poscolecistectomía: conducta terapéutica actual / Post Cholecystectomy Bile Duct Injuries: Current Therapeutic Option

Resumen La colecistolitiasis es una condición común en Chile. La complicación más frecuente de esta condición es la colecistitis aguda. Aproximadamente 60.000 colecistectomías son realizadas anualmente en nuestro país, convirtiéndola en una de las patologías digestivas más frecuentes. El tratamiento de la colelitiasis ha evolucionado las últimas dos décadas. Con el desarrollo de la laparoscopía a final de 1980, la colecistectomía abierta fue definitivamente sustituida en los 90 por la vía laparoscópica. Desafortunadamente, la generalización de este procedimiento produjo un aumento en la incidencia de lesiones de vía biliar, aumentando desde una 0,1-0,2% hasta un 0,5-0,7%. Si bien, con el progresivo entrenamiento de los cirujanos en la cirugía mínimamente invasiva esta cifra ha disminuido nuevamente, la LVB aún constituye una de las complicaciones más graves en cirugía, generando gran impacto en la calidad de vida del paciente, e incluso costos en salud que pueden llegar a ser devastadores. El manejo de una lesión de vía biliar es, para muchos cirujanos, un desafío quirúrgico, que para su resolución requiere de las habilidades y experiencia de un cirujano hepatobiliar y derivación a centros de salud especializados. Sin duda, la acción más importante es extremar de manera rutinaria las medidas de prevención. Sin embargo, una vez que la lesión se ha producido, el futuro del paciente depende, directamente, del buen criterio del cirujano, tanto para realizar un diagnóstico temprano, como del enfrentamiento terapéutico que realice. El objetivo de este trabajo es dar a conocer diferentes conductas preventivas y terapéuticas cuando esta complicación se presenta.

Calculous biliary disease is a common condition in Chile. Over 60.000 cholecystectomies are performed annually, making gallstone disease one of the most common digestive health problems. The treatment of calculous biliary disease has evolved over the last 2 decades. With the development of laparoscopic technology in the late 1980s, new techniques for cholecystectomy were introduced. By the early 1990s, laparoscopic cholecystectomy had replaced open cholecystectomy in the operative management of gallbladder stone disease. Unfortunately, the widespread application of laparoscopic cholecystectomy has led to a concurrent rise in the incidence of major bile duct injuries. Reports have estimated the incidence has risen from 0.1-0.2 to 0.5-0.7%. Although, exponential training of surgeons in minimally invasive surgery has led to decrease this numbers again, bile duct injuries still constitute one of the most serious complications in surgery, generating a great impact on the patient's quality of life, and even health costs that can be devastating. The management of patients following major bile duct injurie is a surgical challenge, often requiring the skills of experienced hepatobiliary surgeons at tertiary referral centers. In this setting, the most important action is to routinely maximize prevention measures. However, once injury has occurred, patient's future depends directly on the correct judgment of the surgeon, both to make an early diagnosis and to choose the best therapeutic confrontation. The objective of this article is to present the different preventive and therapeutic options available when this complication occurs.

[Post-COVID-19 severe cholangiopathy: report of 6 cases]. / Colangiopatía post-COVID-19 grave: reporte de 6 casos.

Coronavirus disease 2019 (COVID-19) is a predominantly respiratory tract infection with the capacity to affect other organs. Liver chemistry abnormalities are a frequent manifestation of COVID-19 but are usually transient. We describe the clinical course and most relevant findings of 6 patients who developed a cholangiopathy after severe COVID-19. The mean age of the patients, 4 men and 2 women, was 56 years and the mean time from COVID-19 diagnosis to diagnosis of cholangiopathy was 138 days. The features most important were the increase of alkaline phosphatase and destructuring and beading of the intrahepatic bile duct in magnetic resonance imaging. Cholangiopathy after severe COVID-19 constitutes a novel entity with unique features and potential for progressive biliary injury and secondary biliary cirrhosis. Further studies are required to understand this disease.

Early versus delayed reconstruction for bile duct injury a multicenter retrospective analysis of a hepatopancreaticobiliary group.

Common bile duct injury is a severe complication. It is related to increased rates of morbidity and mortality. Early recognition and on-time diagnosis plus multidisciplinary management of this disease led by a hepatobiliary surgeon show fewer complications rate and best postoperative outcomes. However, no guidelines exist about the proper time of reconstruction. This study aims to describe the experience of a specialized Hepato-Pancreatic-Biliary (HPB) group and to analyze the outcomes regarding the time of bile duct injury (BDI) repair. A multicenter retrospective review of a prospective database was conducted. All the patients older than 18 years old that underwent common bile duct reconstruction between January 2014 and December 2021 were included. Analysis and description of preoperative characteristics and postoperative outcomes were performed. A reconstruction time-based group differentiation was made and analyzed. 44 patients underwent common bile duct reconstruction between January 2014 and December 2021. 56.82% of the patients were female. The mean age was 53.27 years ± 20.7 years. The most common injury was type E2 (29.55%). Hepaticojejunostomy was performed in 81.81% (of the patients. Delayed reconstruction (> 72 h) was performed in the majority of the cases (75.00%) due to delays in the referral centers or poor condition. No statistically significant difference regarding complications in early or delayed BDI reconstruction. The mortality rate was 2.7% (n = 1). 2-year follow-up bilioenteric stenosis was observed in 7 patients. Biloma showed a statistical relationship with complex bile duct injuries (p = 0.02). Bile duct injury is a severe and complex postoperative complication that increases morbidity and mortality rates in the short and long term in patients undergoing cholecystectomy. In our study, there were no statistical differences between the timing of bile duct reconstruction and the postoperative outcomes; we identified the presence of biloma as a statistically related factor associated with complex bile duct injury; however, further prospective or studies with an increased sample size are required to prove our results.

Postoperative liver function tests can predict anastomotic dysfunction after bile duct injury repair.

Liver function tests help in the follow-up of postoperative patients with iatrogenic bile duct injury. There is not clear evidence regarding their predictive role on anastomosis dysfunction. We describe our experience with postoperative liver function tests and a predictive model of long-term patency after repair. This is retrospective cohort study of patients with bilioenteric anastomosis for bile duct injury and their long-term follow-up. A binomial logistic regression model was performed to ascertain the effects of the grade of bile duct injury and liver function test in the postoperative period. A total of 329 patients were considered for the analysis. In the logistic regression model two predictor variables were statistically significant for anastomosis stenosis: type of bilioenteric anastomosis and alkaline phosphatase levels. A ROC curve analysis was made for alkaline phosphatase with an area under the curve of 0.758 (95% CI 0.67-0.84). A threshold of 323 mg/dL was established (OR 6.0, 95% CI 2.60-13.83) with a sensitivity of 75%, specificity of 67%, PPV of 20%, NPV of 96%, PLR of 2.27 and NLR of 0.37. Increased alkaline phosphatase (above 323 mg/dL) after the fourth operative week was found to be a predictor of long-term dysfunction.

Evaluation of the knowledge of the critical view of safety and recognition of the transoperative complexity during the laparoscopic cholecystectomy.

INTRODUCTION: Since the establishment of the Critical view of safety (CVS), different strategies have been created such as bailout procedures (SC, subtotal cholecystectomy), classifications for preoperative and intraoperative complexity (The Parkland grading scale, PGS) and objective evaluation of the CVS (doublet score, DS) to establish a "Culture of Safety in Cholecystectomy, COSIC"; to avoid complications. METHODS: A multiple choice questionnaire was applied to residents and graduated surgeons from different Hospitals in Mexico during different national meetings; evaluating the knowledge of this different concepts (CVS, SC, PGS, DS), univariate logistic regression was used to assess the association of the knowledge with adverse events (AE) like the Bile duct injury. RESULTS: A total of 744 questionnaires were evaluated; 284 (38.17%) women and 460 (61.83%) men; 436 (58.6%) were residents and 308 (41.4%) graduated surgeons. 708 (95.16%) reported knowing the CVS; however, only (51.98%, p ≤ 0.001) defined the concept correctly, while 136 (18.28%) reported knowing the DS, but only 44 (5.91%) defined it correctly. Regarding the PGS, 398 (53.49%) mentioned knowing it, but only 262 defined it correctly. The concept of SC 642 (86.29%) reported knowing it; however, only (56.7%, p ≤ 0.001) correctly defined the techniques, being the reconstituting technique the preferred one (42.37% vs 34.89%). In this survey, the correct knowledge of the CVS (OR 0.47, p < 0.001), the subtotal techniques (OR 0.71 p = 0.07), the DS (OR 0.48 p < 0.001) and of the PGS (OR 0.28, p < 0.001) decreased the risk of presenting BDI. CONCLUSION: Despite the COSIC and the timing of publication of the CVS; the percentage of people who can correctly define basic safety concepts is low among residents and licensed surgeons. Therefore, it is important to emphasize the dissemination of these concepts to obtain safe LC and thus reduce the incidence of complications.

Rendimiento diagnóstico de las punciones de lesiones biliopancreáticas guiadas por ultrasonido endoscópico con patólogo en sala / Diagnostic performance of endoscopic ultrasound-guided fine needle aspiration of pancreatobiliary lesions with rapid on-site evaluation

Resumen Introducción y objetivos: El ultrasonido endoscópico con punción-aspiración con aguja fina (USE-PAAF) en lesiones neoplásicas biliopancreáticas suele tener un rendimiento alto, que depende de características de la lesión; aspectos técnicos de la USE-PAAF y la experiencia del endoscopista. De los factores menos estudiados es la presencia de patólogo en sala. Se plantea la realización de USE-PAAF con patólogo en sala para disminuir el número de pases, la tasa de muestras inadecuadas y la necesidad de repetir el procedimiento. Material y métodos: Estudio observacional, retrospectivo, con recolección prospectiva de enero de 2018 a junio de 2019, en pacientes adultos sometidos a USE-PAAF. Las muestras obtenidas fueron extendidas y evaluadas en salas de endoscopia por médico patólogo con coloración Diff-Quick y cuando se obtenía una muestra suficiente se enviaba en frasco con formol para bloque celular o biopsias. Resultados: Se realizaron 48 USE-PAAF biliopancreáticas en individuos con una edad mediana de 64 años. Las indicaciones más frecuentes fueron punciones por masa o pseudomasa pancreática (71 % de casos); Se diagnosticaron 35 malignidades (77 % correspondientes a adenocarcinoma, y 14 % a tumores neuroendocrinos). La mediana de tamaño de lesiones fue de 28 mm; el número de pases promedio fue de 3. Se obtuvieron resultados diagnósticos en 89 % frente a 11 % de falsos negativos. Se presentó 1 complicación menor (2,1 %), que fue dolor abdominal. Conclusiones: La USE-PAAF con patólogo en sala tiene alto rendimiento diagnóstico, con escasos resultados falsos negativos. Se requiere una mediana de pases menor, que podría minimizar los riesgos del procedimiento y la necesidad de repetir la prueba.

Abstract Introduction: Endoscopic ultrasound with fine-needle aspiration (EUS-FNA) of pancreatobiliary neoplastic lesions usually has a high performance that depends on the characteristics of the lesion, technical aspects, and expertise of the endoscopist. One of the least studied factors is rapid on-site evaluation with a pathologist in the room. Objective: To perform EUS-FNA with a pathologist in the endoscopy room to reduce the number of passes, the rate of inadequate samples and the need to repeat the procedure. Material and methods: Observational retrospective study with a prospective data collection approach from January 2018 to June 2019 of adult patients undergoing EUS-FNA. The samples obtained were spread and evaluated in endoscopy rooms by a pathologist with Diff-Quick stain, and when a sufficient sample was obtained, it was sent in a vial with formalin for cell block and/or biopsy. Results: 48 pancreatobiliary EUS-FNA were performed in individuals with a median age of 64 years. The most frequent indication was aspiration due to pancreatic mass (71%); 35 malignancies were diagnosed (77% were adenocarcinomas and 14% were neuroendocrine tumors). The median size of the lesions was 28mm, and the average number of passes was 3. Diagnostic results were obtained in 89% vs. 11% of false negatives. There was 1 minor complication (2.1%), which was abdominal pain. Conclusions: EUS-FNA with an in-room pathologist has a high diagnostic performance, with few false negative results. Also, a lower median number of passes is required, minimizing the risks of the procedure and the need for repeating it.

[Ascariasis in the intrahepatic biliary duct: about a case in pediatrics]. / Ascaridiosis en la vía biliar intrahepática: a propósito de un caso pediátrico.

Twenty five percent of the world population is affected by Ascaris lumbricoides. Hepatobiliary ascariasis occurs in areas with high endemicity and great amount of parasitic load, generating intense inflammation to fibrosis. We report a twoyear- old patient that consults about abdominal distension and cough of one month of evolution associated with 72 hours of fever. Abdominal ultrasound is performed, which shows bile duct, stomach, small intestine with ascaris and chest x-ray with interstitial inflammatory infiltrate, associated with hyperleukocytosis with hypereosinophilia and elevated gamma-glutamyl transpeptidase. Antibiotic, anthelminthic treatment is administered, without achieving the elimination of the bile duct parasites, requiring their removal by percutaneous cholangiography.

El 25 % de la población mundial se encuentra infectada por Ascaris lumbricoides. La ascaridiosis hepatobiliar ocurre en zonas con alta endemicidad y gran carga parasitaria, y genera desde intensa inflamación hasta fibrosis. Se presenta a un paciente de 2 años, que consultó por distensión abdominal y tos de 1 mes de evolución asociada a fiebre en las últimas 72 h. Se realizó una ecografía abdominal que evidenció áscaris en la vía biliar, en el estómago y en el intestino delgado, y una radiografía de tórax con infiltrado inflamatorio intersticial, asociado a hiperleucocitosis con hipereosinofilia y gammaglutamiltranspeptidasa elevada. Se administró un tratamiento antibiótico, antihelmíntico, sin lograr la eliminación de los parásitos de la vía biliar, por lo que se requirió su extracción mediante colangiografía percutánea.

[Retrograde cholangiography performed with simple balloon-assisted enteroscopy in patients with altered anatomy by surgery in a private level III clinic]. / Colangiografía retrógrada realizada con enteroscopia asistida por balón simple en pacientes con anatomía alterada por cirugía en una clínica privada nivel III.

OBJECTIVE: To evaluate the usefulness of retrograde cholangiography performed with simple balloon-assisted enteroscopy for the management of bile duct pathology in patients with altered anatomy by surgery at the Anglo American Clinic. MATERIALS AND METHODS: This is a retrospective descriptive study where all patients with altered anatomy who came for bile duct problems to undergo a retrograde cholangiography assisted by simple balloon enteroscopy at the Anglo American Clinic during the January period from 2014 to January 2020. RESULTS: We performed 10 studies of retrograde cholangiography assisted by simple balloon enteroscopy. Cannulation of the bile duct was successful in 80% of our cases, in two cases the bile duct could not be located. The findings of the retrograde cholangiography were biliary stones in 7 cases, stenosis of the biliodigestive anastomosis in 5 cases, and an ampuloma. The most frequent therapeutic procedures were CRE progressive balloon dilation of the bilioenteric anastomosis and extraction of stones with a Dormia basket. There were no complications associated with the procedure. CONCLUSION: Retrograde cholangiography assisted by simple balloon enteroscopy is a safe and effective method to resolve biliary pathology in our patients with altered anatomy.

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.

A practical new strategy to prevent bile duct injury during laparoscopic cholecystectomy. A single-center experience with 5539 cases.

Purpose Bile duct injury (BDI) is a catastrophic complication of cholecystectomy, and misidentification of the cystic anatomy is considered to be the main cause. Although several techniques have been developed to prevent BDI, such as the "critical view of safety", the infundibular technique, the rates remain higher during laparoscopic cholecystectomy (LC) than during open surgery. We, here, propose a practical new strategy for ductal identification, that can help to prevent laparoscopic bile duct injury. Methods A retrospective study of 5539 patients who underwent LC from March 2007 to February 2019 at a single institution was conducted. The gallbladder infundibulum was classified by its position located on an imaginary clock with the gallbladder neck as the center point of the dial, 3-o'clock position as cranial, 6-o'clock as dorsal, 9-o'clock as caudal, and 12-o'clock as ventral, as well as the axial position. Patient demographics, pathologic variables and infundibulum classification were evaluated. Detailed analysis of ductal identification based on gallbladder infundibulum position was performed in this study. All infundibulum positions were recorded by intraoperative laparoscopic video or photographic images. Results All the patients successfully underwent LC during the study period. No conversion or serious complications such as biliary injury occurred. Gallbladders with infundibulum of 3-o'clock position, 6-o'clock position, 9-o'clock position, 12-o'clock position, axial position were 12.3%, 23.4%, 28.0%, 4.2%, and 32.1%, respectively. The 3-o'clock and 12-o'clock position were pitfalls that might cause biliary injury. Conclusion The gallbladder infundibulum as a navigator is useful for ductal identification to reduce BDI and improve the safety of LC.

Tumor de Klatskin (colangiocarcinoma hiliar) su morbimortalidad en un servicio de cirugía / Klatskin's tumor (hilar cholangiocarcinoma) his morbi-mortality in a surgery service

RESUMEN Introducción: El tumor de Klatskin es el colangiocarcinoma, más frecuente de la vía biliar siendo responsable de una alta morbimortalidad en los servicios de cirugía. Objetivo: Determinar la morbilidad y la mortalidad por tumor de Klatskin en el Servicio de Cirugía del Hospital Universitario "Manuel Ascunce Domenech". Métodos: Se realizó un estudio descriptivo, prospectivo y observacional de pacientes que ingresaron en el Servicio de Cirugía General con diagnóstico de tumor de Klatskin, entre septiembre de 2018 y enero del 2020. El universo estuvo conformado por 7 pacientes que cumplieron con los criterios de inclusión. Se utilizaron métodos estadísticos descriptivos y cálculos con valores porcentuales. Resultados: La mayor incidencia de los pacientes fue de sexo masculino y de raza blanca, con un 71,4 por ciento y 85,7 por ciento respectivamente. Predominó adenocarcinoma como variedad histológica con un 85,7 por ciento. Tipo II de la clasificación topográfica, el procedimiento de Hess como operación realizada y la bilirragia como complicación prevaleció con un 42,8 por ciento respectivamente. El 85,7 por ciento de los pacientes egresaron vivos y con una cirugía con finalidad curativa. Conclusiones: La mayoría de los pacientes eran masculinos y de color blanco. Más de la mitad de los pacientes fueron clasificados como tipo I y II según clasificación de Bismuth-Corlette. El proceder de Hess, el adenocarcinoma como forma histológica y el estado del egreso vivo predominó en el total de pacientes(AU)

ABSTRACT Introduction: Klatskin's tumor is cholangiocarcinoma, most frequent to occur in the bile duct, being responsible for high morbidity and mortality in surgery departments. Objective: To determine the morbidity and mortality of Klatskin's tumor at the surgery service of Manuel Ascunce Domenech University Hospital. Methods: We carried out a descriptive, prospective and observational study of patients admitted to the general surgery service with a diagnosis of Klatskin's tumor, between September 2018 and January 2020. The study population consisted of seven patients who met the inclusion criteria. Descriptive statistical methods and calculations with percentage values were used. Results: The highest incidence was represented male and white patients, accounting for 71.4 percent and 85.7 percent, respectively. Adenocarcinoma predominated as a histological variety, accounting for 85.7 percent. There was prevalence of type II of topographic classification, the Hess procedure as the performed operation, and bilirrhagia as a complication, accounting for 42.8 percent, respectively. 85.7 percent of the patients were discharged and received surgery for curative purposes. Conclusions: Most of the patients were male and white. More than half of the patients were classified as types I and II, according to the Bismuth-Corlette classification. The Hess procedure, adenocarcinoma as a histological form, and discharge predominated in all patients(AU)

Colangiografía retrógrada realizada con enteroscopia asistida por balón simple en pacientes con anatomía alterada por cirugía en una clínica privada nivel III / Retrograde cholangiography performed with simple balloon-assisted enteroscopy in patients with altered anatomy by surgery in a private level III clinic

RESUMEN Objetivo : Evaluar la utilidad de la colangiografía retrógrada realizada con enteroscopia asistida por balón simple para el manejo de la patología de la via biliar en pacientes con anatomía alterada por cirugía en la Clínica Anglo Americana. Materiales y métodos : El presente es un estudio descriptivo retrospectivo donde se evalúan a todos los pacientes con anatomía alterada que acudieron por problemas de la via biliar para realizarse una colangiografía retrógrada asistida por enteroscopia con balón simple en la Clínica Anglo Americana durante el periodo de enero del 2014 a enero del 2020. Resultados : Realizamos 10 estudios de colangiografía retrógrada asistida por enteroscopia con balón simple. La canulación de la vía biliar fue exitosa en el 80% de nuestros casos, en dos casos no se pudo localizar la via biliar. Los hallazgos de la colangiografía retrógrada fueron litiasis de via biliar en 7 casos, estenosis de la anastomosis biliodigestiva en 5 casos y un ampuloma. Los procedimientos terapeúticos más frecuentes fueron dilatación con balón progresivo CRE de la anastomosis bilioentérica y extracción de litos con canastilla de Dormia. No se presentaron complicaciones asociadas al procedimiento. Conclusión : La colangiografía retrógrada asistida por enteroscopia con balón simple es un método seguro y eficaz para resolver la patología biliar en nuestros pacientes con anatomía alterada.

ABSTRACT Objective : To evaluate the usefulness of retrograde cholangiography performed with simple balloon-assisted enteroscopy for the management of bile duct pathology in patients with altered anatomy by surgery at the Anglo American Clinic. Materials and methods : This is a retrospective descriptive study where all patients with altered anatomy who came for bile duct problems to undergo a retrograde cholangiography assisted by simple balloon enteroscopy at the Anglo American Clinic during the January period from 2014 to January 2020. Results : We performed 10 studies of retrograde cholangiography assisted by simple balloon enteroscopy. Cannulation of the bile duct was successful in 80% of our cases, in two cases the bile duct could not be located. The findings of the retrograde cholangiography were biliary stones in 7 cases, stenosis of the biliodigestive anastomosis in 5 cases, and an ampuloma. The most frequent therapeutic procedures were CRE progressive balloon dilation of the bilioenteric anastomosis and extraction of stones with a Dormia basket. There were no complications associated with the procedure. Conclusion : Retrograde cholangiography assisted by simple balloon enteroscopy is a safe and effective method to resolve biliary pathology in our patients with altered anatomy.

ABCB4 disease: Many faces of one gene deficiency.

ATP-binding cassette (ABC) subfamily B member 4 (ABCB4), also known as multidrug resistance protein 3 (MDR3), encoded by ABCB4, is involved in biliary phospholipid secretion, protecting hepatobiliary system from deleterious detergent and lithogenic properties of the bile. ABCB4 mutations altering canalicular ABCB4 protein function and expression may have variable clinical presentation and predispose to several human liver diseases. Well-established phenotypes of ABCB4 deficit are: progressive familial intrahepatic cholestasis type 3, gallbladder disease 1 (syn. low phospholipid associated cholelithiasis syndrome), high ɣ-glutamyl transferase intrahepatic cholestasis of pregnancy, chronic cholangiopathy, and adult biliary fibrosis/cirrhosis. Moreover, ABCB4 aberrations may be involved in some cases of drug induced cholestasis, transient neonatal cholestasis, and parenteral nutrition-associated liver disease. Recently, genome-wide association studies have documented occurrence of malignant tumours, predominantly hepatobiliary malignancies, in patients with ABCB4/MDR3 deficit. The patient's age at the time of the first presentation of cholestatic disease, as well as the severity of liver disorder and response to treatment are related to the ABCB4 allelic status. Mutational analysis of ABCB4 in patients and their families should be considered in all individuals with cholestasis of unknown aetiology, regardless of age and/or time of onset of the first symptoms.

A practical new strategy to prevent bile duct injury during laparoscopic cholecystectomy. A single-center experience with 5539 cases

Abstract Purpose Bile duct injury (BDI) is a catastrophic complication of cholecystectomy, and misidentification of the cystic anatomy is considered to be the main cause. Although several techniques have been developed to prevent BDI, such as the "critical view of safety", the infundibular technique, the rates remain higher during laparoscopic cholecystectomy (LC) than during open surgery. We, here, propose a practical new strategy for ductal identification, that can help to prevent laparoscopic bile duct injury. Methods A retrospective study of 5539 patients who underwent LC from March 2007 to February 2019 at a single institution was conducted. The gallbladder infundibulum was classified by its position located on an imaginary clock with the gallbladder neck as the center point of the dial, 3-o'clock position as cranial, 6-o'clock as dorsal, 9-o'clock as caudal, and 12-o'clock as ventral, as well as the axial position. Patient demographics, pathologic variables and infundibulum classification were evaluated. Detailed analysis of ductal identification based on gallbladder infundibulum position was performed in this study. All infundibulum positions were recorded by intraoperative laparoscopic video or photographic images. Results All the patients successfully underwent LC during the study period. No conversion or serious complications such as biliary injury occurred. Gallbladders with infundibulum of 3-o'clock position, 6-o'clock position, 9-o'clock position, 12-o'clock position, axial position were 12.3%, 23.4%, 28.0%, 4.2%, and 32.1%, respectively. The 3-o'clock and 12-o'clock position were pitfalls that might cause biliary injury. Conclusion The gallbladder infundibulum as a navigator is useful for ductal identification to reduce BDI and improve the safety of LC.

Robotic Left Hepatectomy and Roux-en-Y Hepaticojejunostomy After Bile Duct Injury.

BACKGROUND: Bile duct injuries after cholecystectomy remain a major concern because their incidence has not changed through the years despite technical advances. This video presents a robotic left hepatectomy and Roux-en-Y hepaticojejunostomy as a treatment for a complex bile duct injury after laparoscopic cholecystectomy. METHODS: A 52-year-old man underwent laparoscopic cholecystectomy at another institution 8 years previously, which resulted in a bile duct injury. His postoperative period was complicated by jaundice and cholangitis. He was treated with endoscopic retrograde cholangiopancreatography and multiple endoprostheses for 3 years, after which the endoprostheses were removed, and he was sent to the authors' institution. Computed tomography showed that the left liver had signs of disturbed perfusion and dilation of the left intrahepatic bile duct. The patient was asymptomatic and refused any further attempt at surgical correction of the lesion. He was accompanied for 5 years. Magnetic resonance imaging showed progressive atrophy of the left liver. Finally, 3 months before this writing, he presented with intermittent episodes of cholangitis. A multidisciplinary team decided to perform left hepatectomy with Roux-en-Y hepatojejunostomy via a robotic approach. The left liver was atrophied, and left hepatectomy was performed. Fluorescence imaging was used to identify the right bile duct. At opening of the right bile duct, small stones were found and removed. Antecolic Roux-en-Y hepaticojejunostomy then was performed. RESULTS: The operative time was 335 min. Recovery was uneventful, and the patient was discharged on postoperative day 4. CONCLUSIONS: Robotic repair of bile duct injuries is feasible and safe, even when liver resection is necessary. This video may help oncologic surgeons to perform this complex procedure.

Biliary Paracoccidioidomycosis: An Unusual Infection Simulating Malignant Neoplasm.

Paracoccidioidomycosis is a systemic granulomatous disease caused by the dimorphic fungus Paracoccidioides brasiliensis and is restricted to Latin America. It normally affects lungs, skin and lymph nodes. Abdominal organs are usually not involved. In rare cases paracoccidioidomycosis may simulate neoplasm. Herein we describe our experience with four cases of paracoccidioidomycosis mimicking cholangiocarcinoma. To the best of our knowledge, this is the largest case series on this subject produced in English. Paracoccidioidomycosis must be considered as a differential diagnosis of cholangiocarcinoma, especially in individuals who come from endemic areas.