The postoperative choledochoscopy in the management of the residual hepatolithiasis involving the caudate lobe: A retrospective study.

ABSTRACT: To reveal the role of the postoperative choledochoscopy in treating the residual calculi in the caudate lobe (CL) of the liver.We recruited 66 patients with T-tube/percutaneous transhepatic cholangioscopy tract who still had residual gallstones in the CL at least 6 weeks after the operation. Imaging examinations determined the gallstones' locations in the patients, and all of them underwent the postoperative choledochoscopic examination through the T-tube/percutaneous transhepatic cholangioscopy tract for therapeutic intervention.Among the 66 patients, the residual gallstones were mostly located in the Spiegel lobe (48/66, 72.7%), and the residual gallstones that located in the origin of the CL bile branches were successfully determined in the 57 patients (57/66, 86.4%), the remaining 9 patients were unclear because the proximal ducts were severely narrow or even atresia. The mean frequency of the postoperative choledochoscopy was 3.6 (range, 1-10) times. There were 9 patients with complications, and no mortality occurred. In the origin-proved 57 patients, 6 patients failed to remove the gallstones altogether, and the final residual gallstone clearance rate was 77.3% (51/66). There was no significant difference between the Spiegel lobe and the other parts of the CL in determining the bile duct's origins, gallstone clearance rate, and complications. However, the frequency of choledochoscopy in the other parts of the CL was more than in the Spiegel lobe.The postoperative choledochoscopy, an essential method for treating the residual gallstones in the CL, commands high efficiency for calculi extraction and fewer complications. The main reasons for failing to remove the residual gallstones are that the bile duct's origins could not be determined, and the distal bile ducts are atretic in the CL.

Agenesia de la vesícula biliar / Agenesia of the gallbladder

La agenesia de la vesícula biliar (AVB) es una entidad congénita rara. La mayoría de los pacientes, permanecen asintomáticos, mientras que los que presentan síntomas informan síntomas que imitan el cólico biliar. La evaluación inicial para la sospecha de patología de la vesícula biliar, como la ecografía del cuadrante superior derecho, Puede ser engañosa o no concluyente. En consecuencia, algunos pacientes son finalmente diagnosticados intra-operatoriamente. Por lo tanto, deben mantener la AVB como un diagnóstico diferencial y deben realizarse como la colangiopancreatografía por resonancia magnética (CPRMN) cuando otras pruebas no sean concluyentes. Presentamos una mujer de 39 años que presento síntomas crónicos compatibles con cólico biliar y una ecografía equívoca informada como escleroatrófica con colelitiasis. Se le realizó laparoscopia durante la cual se constató la ausencia de vesícula biliar. La CPRM posoperatoria confirmó el diagnóstico de AG.

Gallbladder agenesis (GA) is a rare congenital entity. Most patients remain asymptomatic, while those with symptoms report symptoms that mimic bile colic. Initial evaluation for suspected gallbladder pathology, such as ultrasound of the right upper quadrant, May be misleading or inconclusive. As a result, some patients are eventually diagnosed intra-operatively. Therefore, GA should be maintained as a differential diagnosis and should be performed as magnetic resonance cholangiopancreatography (MRI) when other tests are inconclusive. We present a 39-year-old woman who has chronic symptoms compatible with biliary colic and an equivocal ultrasound reported as scleroatrofic with cholelithiasis. Laparoscopy was performed during which the absence of gallbladder was found. Postoperative CPRM confirmed the diagnosis of GA.

Management strategies and treatment results of pediatric choledochal malformations in the Nordic countries.

BACKGROUND: Incidence and long-term outcomes of choledochal malformations (CMs) in children remain unclear. METHODS: Clinical characteristics, operative details, complications, and follow-up data were collected from eight pediatric surgical centers in Sweden, Norway, Denmark, and Finland, which also answered a questionnaire addressing management practices. RESULTS: During 2000-2017, 126 pediatric CMs were diagnosed, corresponding an incidence of 1:37,400. Diagnostic, treatment, and follow-up practices varied markedly. Of patients with complete clinical data (n = 119), 85% and 11% had type I and IV CMs and were managed by open hepaticojejunostomy at median age of 2.5 (interquartile range 0.46-5.8) years. Associated malformations were more common in fusiform and type IV (23%) than cystic CMs (8%, p = 0.043). Pancreaticobiliary maljunction was more frequently confirmed in patients presenting with pancreatitis (26% vs. 7%, p = 0.005) and with fusiform CMs (56% vs. 25%, p = 0.001). Cholangitis/pancreatitis episodes, occurring in 12% during postoperative follow-up of 4.0 (2.0-7.9) years, associated with longer surveillance (OR 1.32, 95% CI 1.13-1.54, p < 0.001). However, only two thirds of centers continued follow-up until adulthood. No malignancies were reported. CONCLUSIONS: CM incidence was higher than traditionally reported among Western populations. Although open hepaticojejunostomy carries good short-term outcomes, long-term morbidity is noteworthy. Standardized evidence-based management strategies and long-term follow-up are encouraged.

Feline abdominal ultrasonography: what's normal? what's abnormal? Hepatic vascular anomalies.

PRACTICAL RELEVANCE: Abdominal ultrasound plays a vital role in the diagnostic work-up of many cats presenting to general and specialist practitioners. Although hepatic vascular anomalies are less common than disorders of the hepatic parenchyma and biliary tree, our understanding and recognition of these is gradually increasing with advancements in ultrasound technology and image quality. CLINICAL CHALLENGES: Despite ultrasonography being a commonly used modality, many practitioners are not comfortable performing an ultrasound examination or interpreting the resulting images. Even differentiating between normal variation and pathological changes can be challenging for all but the most experienced. In addition, some views may be obscured by overlying structures; for example, the termination of a shunt entering the left phrenic or azygous veins is often difficut to see due to the high probability of lung passing between the shunt and the transducer as the cat breathes. EQUIPMENT: Ultrasound facilities are readily available to most practitioners, although use of ultrasonography as a diagnostic tool is highly dependent on operator experience. AIM: This review, part of an occasional series on feline abdominal ultrasonography, discusses the appearance of various hepatic vascular anomalies. It is aimed at general practitioners who wish to improve their knowledge and confidence in feline abdominal ultrasound and is accompanied by high-resolution images. Ultrasound of the liver and biliary tree were discussed in articles published in January and May 2019, respectively. EVIDENCE BASE: Information provided in this article is drawn from the published literature and the author's own clinical experience.

Intraoperative detection of aberrant biliary anatomy via intraoperative cholangiography during laparoscopic cholecystectomy.

BACKGROUND: Laparoscopic cholecystectomy (LC) is the standard of treatment for symptomatic cholelithiasis. Although intraoperative cholangiography (IOC) is widely used as an adjunct to LC, there is still no worldwide consensus on the value of its routine use. Anatomical studies have shown that variations of the biliary tree are present in approximately 35% of patients with variations in right hepatic second-order ducts being especially common (15-20%). Approximately, 70-80% of all iatrogenic bile duct injuries are a consequence of misidentification of biliary anatomy. The purpose of this study was to assess the adequacy of and the reporting of IOCs during LC. METHODS: IOCs obtained from 300 consecutive LCs between July 2014 and July 2016 were analysed retrospectively by two surgical trainees and confirmed by a radiologist. Biliary tree anatomy was classified from IOC films as described by Couinaud (1957) and correlated with documented findings. The accuracy of intraoperative reporting was assessed. Biliary anatomy was correlated to clinical outcome. RESULTS: A total of 95% of IOCs adequately demonstrated biliary anatomy. Aberrant right sectoral ducts were identified in 15.2% of the complete IOCs, and 2.6% demonstrated left sectoral or confluence anomalies. Only 20.4% of these were reported intraoperatively. Bile leaks occurred in two patients who had IOCs (0.73%) and two who did not (7.4%). CONCLUSION: Surgeons generally demonstrate biliary anatomy well on IOC but reporting of sectoral duct variation can be improved. Further research is needed to determine whether anatomical variation is related to ductal injury.

Prenatal sonographic diagnosis of biliary tract malformations.

Congenital anomalies of the biliary tract include a variety of pathologic conditions, such as biliary atresia, choledochal cysts, gallbladder agenesis, congenital cholelithiasis, and gallbladder duplication. Although most of these malformations are rare and benign conditions, they may occasionally represent a major threat to extrauterine life. Visualization of a normal-sized gallbladder should be a mandatory component of the second-trimester anomaly ultrasound scan. Advances in prenatal sonography enable the detection of biliary tract congenital malformations. In this review, we discuss the detection rates, sonographic features, and prognosis of the most frequently prenatally diagnosed biliary tract malformations.

Dilatation of the common channel in pediatric congenital biliary dilatation remaining after radical operation.

BACKGROUND: In congenital biliary dilatation, the protein plug is likely impacted, especially in a dilated common channel. However, nobody has discussed whether this dilatation of common channel remains after radical operation. The aim of this study was to investigate the situation of the dilated common channel after radical operation. METHODS: The status of the common channel was investigated at radical operation and follow-up. We then evaluated whether dilation of the common channel remained postoperatively. Moreover, the association between status of the common channel and postoperative pancreatic complication was evaluated. RESULTS: Postoperative condition of the common channel was able to be evaluated ultrasonographically in 42 patients. The common channel was dilated at the time of operation in 18 patients and remained dilated postoperatively in 11 of these 18 patients. Dilatation of the common channel was not identified in any patients without dilatation at the time of operation. No patients in this series showed any postoperative pancreatic complications, even if a dilated common channel remained. CONCLUSIONS: Dilatation of the common channel can remain postoperatively in congenital biliary dilatation, but does not appear to be associated with postoperative pancreatic complications.

Hippo pathway coactivators Yap and Taz are required to coordinate mammalian liver regeneration.

The mammalian liver has a remarkable capacity for repair following injury. Removal of up to two-third of liver mass results in a series of events that include extracellular matrix remodeling, coordinated hepatic cell cycle re-entry, restoration of liver mass and tissue remodeling to return the damaged liver to its normal state. Although there has been considerable advancement of our knowledge concerning the regenerative capacity of the mammalian liver, many outstanding questions remaining, such as: how does the regenerating liver stop proliferating when appropriate mass is restored and how do these mechanisms relate to normal regulation of organ size during development? Hippo pathway has been proposed to be central in mediating both events: organ size control during development and following regeneration. In this report, we examined the role of Yap and Taz, key components of the Hippo pathway in liver organ size regulation, both in the context of development and homeostasis. Our studies reveal that contrary to the current paradigms that Yap/Taz are not required for developmental regulation of liver size but are required for proper liver regeneration. In livers depleted of Yap and Taz, liver mass is elevated in neonates and adults. However, Yap/Taz-depleted livers exhibit profound defects in liver regeneration, including an inability to restore liver mass and to properly coordinate cell cycle entry. Taken together, our results highlight requirements for the Hippo pathway during liver regeneration and indicate that there are additional pathways that cooperate with Hippo signaling to control liver size during development and in the adult.

Plectin controls biliary tree architecture and stability in cholestasis.

BACKGROUND & AIMS: Plectin, a highly versatile cytolinker protein, controls intermediate filament cytoarchitecture and cellular stress response. In the present study, we investigate the role of plectin in the liver under basal conditions and in experimental cholestasis. METHODS: We generated liver-specific plectin knockout (PleΔalb) mice and analyzed them using two cholestatic liver injury models: bile duct ligation (BDL) and 3,5-diethoxycarbonyl-1,4-dihydrocollidine (DDC) feeding. Primary hepatocytes and a cholangiocyte cell line were used to address the impact of plectin on keratin filament organization and stability in vitro. RESULTS: Plectin deficiency in hepatocytes and biliary epithelial cells led to aberrant keratin filament network organization, biliary tree malformations, and collapse of bile ducts and ductules. Further, plectin ablation significantly aggravated biliary damage upon cholestatic challenge. Coincidently, we observed a significant expansion of A6-positive progenitor cells in PleΔalb livers. After BDL, plectin-deficient bile ducts were prominently dilated with more frequent ruptures corresponding to an increased number of bile infarcts. In addition, more abundant keratin aggregates indicated less stable keratin filaments in PleΔalb hepatocytes. A transmission electron microscopy analysis revealed a compromised tight junction formation in plectin-deficient biliary epithelial cells. In addition, protein profiling showed increased expression of the adherens junction protein E-Cadherin, and inefficient upregulation of the desmosomal protein desmoplakin in response to BDL. In vitro analyses revealed a higher susceptibility of plectin-deficient keratin networks to stress-induced collapse, paralleled by elevated activation of p38 MAP kinase. CONCLUSION: Our study shows that by maintaining proper keratin network cytoarchitecture and biliary epithelial stability, plectin plays a critical role in protecting the liver from stress elicited by cholestasis. LAY SUMMARY: Plectin is a cytolinker protein capable of interconnecting all three cytoskeletal filament systems and linking them to plasma membrane-bound junctional complexes. In liver, the plectin-controlled cytoskeleton mechanically stabilizes epithelial cells and provides them with the capacity to adapt to increased bile pressure under cholestasis.

Selective intraoperative cholangiography during laparoscopic cholecystectomy in children is justified.

AIMS: Intraoperative cholangiograms (IOC) during laparoscopic cholecystectomy have been advocated to detect biliary anomalies and intraductal calculi. However, IOC increases operative time and patient irradiation, and therefore is not universally used. We hypothesise selective IOC may be a valuable tool in children. METHODS: A retrospective case note review was performed of all children who underwent laparoscopic cholecystectomy at a single institution by a single surgeon between January 2011 and March 2017. Demographics, radiological imaging, indications for surgery and IOC, and clinical outcomes were collected. Chi-Squared and Wilcoxon Rank Sum tests were used for comparisons. RESULTS: Sixty-two patients were reviewed. Median follow-up was 2 months (0.1-60), and 53 (85%) had complete symptom resolution following surgery. Twenty-two patients underwent IOC. Six (27%) had anomalies undetected by preoperative imaging. IOC identified common bile duct (CBD) stones in 2 patients which were cleared at laparoscopy. One patient required subsequent ERCP for impacted stones. One patient has a long common channel and pancreatitis. Two patients have CBD strictures. These last 3 are awaiting biliary reconstruction. Presence of CBD dilatation or ductal stones on preoperative ultrasound were significantly associated with positive findings at IOC. No complications resulted from IOC. Patients who did not undergo IOC did not represent with missed anomalies. CONCLUSIONS: Despite using multimodal preoperative imaging, IOC detected biliary anomalies requiring further treatment in 6/62 (10%) of patients undergoing laparoscopic cholecystectomy. Our data support the use of IOC in selective patients with CBD dilatation or suspicion of ductal stones on preoperative imaging. LEVEL OF EVIDENCE: Study of Diagnostic Test: Level III.

Ultrasound of congenital and inherited disorders of the pediatric hepatobiliary system, pancreas and spleen.

Ultrasound is often the initial imaging examination performed of the solid organs of the pediatric abdomen. The sonographic appearance of the hepatobiliary system, pancreas and spleen changes with growth and development. This article reviews the normal US appearance of these organs in children and illustrates, through case examples, congenital and inherited conditions that affect them.

Living-Donor Liver Transplantation With the Use of a Left-Lobe Graft From a Donor With Anomalous Biliary Anatomy in Which B4 Joins the Right Anterior Sectional Duct: A Case Report.

When there is an anatomic anomaly in the biliary tract of the donor for living-donor liver transplantation, the risk of postoperative biliary tract complications increases in both the donor and the recipient. We studied a case of living-donor liver transplantation with a left hepatic lobe graft that had anatomic anomalies, in which the medial segmental branch (B4) joined the anterior segmental branch and the posterior segmental branch formed a common trunk with the lateral segmental branch. A 40-year-old man visited our institution as a candidate organ donor for his mother, who had end-stage liver failure. An anomaly of B4 connecting the anterior segmental branch was suspected on magnetic resonance cholangiopancreatography. On intraoperative cholangiography, confluence of B4 with the anterior segmental branch and connection of the posterior and lateral segmental branches forming a common trunk were confirmed. Accordingly, individual anastomoses of the lateral segmental branch and B4 with the recipient jejunum were planned, and a left-lobe graft was excised. The postoperative recovery was smooth, and the donor was discharged with no complications. Even when an anatomic anomaly is present in the donor bile duct, in urgent cases, accurate evaluation through the use of various modalities may enable living-donor liver transplantation with the use of a graft with an anatomic anomaly.

Classification of pancreaticobiliary maljunction and clinical features in children.

BACKGROUND: In 2015, the Committee on Diagnostic Criteria of the Japanese Study Group on Pancreaticobiliary Maljunction (PBM) proposed a classification of PBM into four types: (A) stenotic type, (B) non-stenotic type, (C) dilated channel type, and (D) complex type. To validate this classification and clarify the clinical features of the four types of PBM, a retrospective multicenter study was conducted. METHODS: The study group of 317 children with PBM was divided into the four types of PBM. Clinical features, preoperative complications, operations, and postoperative pancreatic complications were evaluated. RESULTS: All patients underwent excision of the extrahepatic bile duct. In type A, the age was younger and there was a higher incidence of cystic dilatation. Non-dilatation of the common bile duct was frequently seen in type B. Abdominal pain with hyperamylasemia was frequently seen in types B and C. In particular, the incidence of protein plugs and biliary perforation was high in type C (56.1% and 14.3%, respectively). The overall incidence of acute pancreatitis was 7.3%. Pancreatitis after excisional surgery was rare in the children in this study. Two patients with type D (0.6%) developed chronic pancreatitis postoperatively. CONCLUSIONS: This proposed classification is simple and correlates well with clinical features.

Biliary complications after liver transplantation; recent developments in etiology, diagnosis and endoscopic treatment.

Biliary complications are considered to be the Achilles' heel of liver transplantation. The most common complications are leaks and bile duct strictures. Strictures can arise at the level of the anastomosis (anastomotic strictures; AS) or at other locations in the biliary tree (non-anastomotic strictures; NAS). Endoscopic treatment via endoscopic retrograde cholangiopancreatography (ERCP) is considered to be the preferred therapy for these complications. This review will focus on the diagnostic modalities, new insights in etiology of biliary complications and outcomes after different endoscopic therapies, in both deceased donor transplantation and living-donor liver transplantations. Advances in recent therapies, such as the use of self-expendable metal stents (SEMS) and endoscopic therapy for patients with a bilio-digestive anastomosis will be discussed.

Anomalous biliary and vascular anatomy-Potential pitfalls during cholecystectomy.

Laparoscopic cholecystectomy is usually a low-risk procedure associated with a short stay and a low rate of conversion to open surgery. Complications are sometimes associated with anomalous vascular or biliary anatomy. Outlined below are the variations in vascular and biliary anatomy which may result in complications either due to involvement in the inflammatory process or inadvertent division during dissection. Clin. Anat. 30:1103-1106, 2017. © 2017 Wiley Periodicals, Inc.

Biliary Anomalies in Patients With HNF1B Diabetes.

Context: The clinical spectrum of organogenetic anomalies associated with HNF1B mutations is heterogeneous. Besides cystic kidney disease, diabetes, and various other manifestations, odd cases of mainly neonatal and posttransplantation cholestasis have been described. The biliary phenotype is incompletely defined. Objective: To systematically characterize HNF1B-related anomalies in the bile ducts by imaging with magnetic resonance imaging (MRI) or magnetic resonance cholangiopancreatography (MRCP). Setting and Patients: Fourteen patients with HNF1B mutations in the catchment area of the Helsinki University Hospital were evaluated with upper abdominal MRI and MRCP. Blood samples and clinical history provided supplemental data on the individual phenotype. Main Outcome Measure(s): Structural anomalies in the biliary system, medical history of cholestasis, other findings in abdominal organs, diabetes and antihyperglycemic treatment, hypomagnesemia, and hyperuricemia. Results: Structural anomalies of the bile ducts were found in seven of 14 patients (50%). Six patients had choledochal cysts, which are generally considered premalignant. Conclusions: Structural anomalies of the biliary system were common in HNF1B mutation carriers. The malignant potential of HNF1B-associated choledochal cysts warrants further studies.

Gallstones.

Gallstones grow inside the gallbladder or biliary tract. These stones can be asymptomatic or symptomatic; only gallstones with symptoms or complications are defined as gallstone disease. Based on their composition, gallstones are classified into cholesterol gallstones, which represent the predominant entity, and bilirubin ('pigment') stones. Black pigment stones can be caused by chronic haemolysis; brown pigment stones typically develop in obstructed and infected bile ducts. For treatment, localization of the gallstones in the biliary tract is more relevant than composition. Overall, up to 20% of adults develop gallstones and >20% of those develop symptoms or complications. Risk factors for gallstones are female sex, age, pregnancy, physical inactivity, obesity and overnutrition. Factors involved in metabolic syndrome increase the risk of developing gallstones and form the basis of primary prevention by lifestyle changes. Common mutations in the hepatic cholesterol transporter ABCG8 confer most of the genetic risk of developing gallstones, which accounts for ∼25% of the total risk. Diagnosis is mainly based on clinical symptoms, abdominal ultrasonography and liver biochemistry tests. Symptoms often precede the onset of the three common and potentially life-threatening complications of gallstones (acute cholecystitis, acute cholangitis and biliary pancreatitis). Although our knowledge on the genetics and pathophysiology of gallstones has expanded recently, current treatment algorithms remain predominantly invasive and are based on surgery. Hence, our future efforts should focus on novel preventive strategies to overcome the onset of gallstones in at-risk patients in particular, but also in the population in general.

Three-Dimensional Sonography of Biliary Tract Disorders.

In this pictorial essay, we review the 3-dimensional sonographic appearance of the normal gallbladder and biliary tree, as well as the appearance of congenital and acquired biliary tract disorders. Using examples, we demonstrate the advantage of 3-dimensional scanning compared with 2-dimensional sonography and, even in certain cases, magnetic resonance cholangiopancreatography.