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Purpose: To develop machine learning (ML) models to predict the surgical risk of children with pancreaticobiliary maljunction (PBM) and biliary dilatation. Methods: The subjects of this study were 157 pediatric patients who underwent surgery for PBM with biliary dilatation between January, 2015 and August, 2022. Using preoperative data, four ML models were developed, including logistic regression (LR), random forest (RF), support vector machine classifier (SVC), and extreme gradient boosting (XGBoost). The performance of each model was assessed via the area under the receiver operator characteristic curve (AUC). Model interpretations were generated by Shapley Additive Explanations. A nomogram was used to validate the best-performing model. Results: Sixty-eight patients (43.3%) were classified as the high-risk surgery group. The XGBoost model (AUC = 0.822) outperformed the LR (AUC = 0.798), RF (AUC = 0.802) and SVC (AUC = 0.804) models. In all four models, enhancement of the choledochal cystic wall and an abnormal position of the right hepatic artery were the two most important features. Moreover, the diameter of the choledochal cyst, bile duct variation, and serum amylase were selected as key predictive factors by all four models. Conclusions: Using preoperative data, the ML models, especially XGBoost, have the potential to predict the surgical risk of children with PBM and biliary dilatation. The nomogram may provide surgeons early warning to avoid intraoperative iatrogenic injury. [ABSTRACT FROM AUTHOR]

Objective To investigate the relationship between bile duct uneven dilatation and postoperative pancreatitis after ERCP. Methods A total of 247 cases of ERCP+EST + ENBD due to bile duct stone were performed in the Second Affiliated Hospital of Kunming Medical University from January 2018 to December 2021. They were divided into the bile duct dilation group (bile duct diameter ≥10 mm) (group A = 84) and the nondilated group (bile duct diameter < 10 mm) (group B = 163) as control group for statistical processing and comparison. Results There were 84 patients in group A, including 45 females; 9 cases of suspected SOD; there are 30 patients with difficult cannulation, including 22 patients with pancreatic guidewire p assages and 8 patients with development of the pancreatic duct; 9 cases of APBDJ. In group A, there were 10 cases of mild pancreatitis, 8 cases of moderate pancreatitis, and 3 cases of severe pancreatitis. There were 163 patients in group B, including 72 females; 11 cases of suspected SOD; there were 63 patients with difficult cannulation, including 61 pancreatic guidewire passages and 2 patients with development of the pancreatic duct; 1 case of APBDJ. In group B, there are 10 cases of mild pancreatitis, 1 case of moderate pancreastitis, and 4 cases of severe pancreatitis. There were 30 patients in group A and 57 patients in group B who received indomethacin anal embolus. The incidence of PEP was 25% in group A and 9.2% in group B, and the difference between the two groups was statistically significant (P = 0.001) . Logistic univariate regression analysis of 247 patients showed that bile duct dilatation (OR = 3.289, 95%CI = 1.593~6.792, P = 0.001) was an independent risk factor for PEP. Conclusions Female patient with uneven bile duct dilation (bile duct diameter ≥10 mm) is more likely to develop PEP than nondilated patient (bile duct diameter < 10 mm). Therefore, ERCP + EST + ENBD should be carefully handled in these patients, and complex endoscopic surgical operations should be avoided as far as possible. [ABSTRACT FROM AUTHOR]

Abstract Background Biliary dilatation is a rare disease involving intrahepatic and extrahepatic biliary tract abnormalities. With the development of imaging technology, an increasing number of special cases have been diagnosed, which poses a challenge to the traditional classification method. Case presentation A 50-year-old woman was admitted to the hospital due to right upper quadrant pain for more than 10 days. The patient had previous episodes of similar symptoms, which were relieved after symptomatic treatment at a local community hospital. After the symptoms developed, she underwent a computed tomography scan at the local hospital, which showed biliary dilatation; thus, she was referred to our hospital for further treatment. After admission, her magnetic resonance imaging examination also suggested biliary dilatation, but abnormal signals were found in her duodenum. First, a duodenal diverticulum was considered. Later, endoscopic ultrasonography was conducted, and the results suggested that the dilated biliary tract had herniated into the duodenum. This type of lesion is most closely classified as a Todani type III lesion. The patient finally underwent choledochectomy and Roux‑en‑Y hepaticojejunostomy, and the postoperative pathology was consistent with our preoperative diagnosis. The patient was followed up for approximately 2 years, and no obvious postoperative complications were found. Conclusions The manifestations of this case are relatively rare and involve one of the undiscussed categories of the Todani classification system; therefore, this case has certain clinical value. Moreover, there is no report similar to this experience in the previous literature.

Background: Biliary dilatation is a rare disease involving intrahepatic and extrahepatic biliary tract abnormalities. With the development of imaging technology, an increasing number of special cases have been diagnosed, which poses a challenge to the traditional classification method.Case Presentation: A 50-year-old woman was admitted to the hospital due to right upper quadrant pain for more than 10 days. The patient had previous episodes of similar symptoms, which were relieved after symptomatic treatment at a local community hospital. After the symptoms developed, she underwent a computed tomography scan at the local hospital, which showed biliary dilatation; thus, she was referred to our hospital for further treatment. After admission, her magnetic resonance imaging examination also suggested biliary dilatation, but abnormal signals were found in her duodenum. First, a duodenal diverticulum was considered. Later, endoscopic ultrasonography was conducted, and the results suggested that the dilated biliary tract had herniated into the duodenum. This type of lesion is most closely classified as a Todani type III lesion. The patient finally underwent choledochectomy and Roux‑en‑Y hepaticojejunostomy, and the postoperative pathology was consistent with our preoperative diagnosis. The patient was followed up for approximately 2 years, and no obvious postoperative complications were found.Conclusions: The manifestations of this case are relatively rare and involve one of the undiscussed categories of the Todani classification system; therefore, this case has certain clinical value. Moreover, there is no report similar to this experience in the previous literature. [ABSTRACT FROM AUTHOR]

IgG4-related sclerosing cholangitis (IgG4-SC) is a relatively newly identified disease that is frequently associated with autoimmune pancreatitis. The differential diagnosis between cholangiocarcinoma, primary sclerosing cholangitis, and IgG4-SC can be challenging due to significant overlap among the clinical and imaging characteristics. We report the case of a 71-year-old woman who was diagnosed with IgG4-related disease based on increased serum IgG4 levels, imaging, and clinical presentation, which showed systemic involvement, including sclerosing cholangitis and kidneys. The patient presented with chronic jaundice. Magnetic resonance imaging revealed bile duct strictures and the dilatation of upstream bile ducts, smooth wall thickening with uniform enhancement in the delayed phase, and no vascular infiltration. Multiple low-density, wedge-shaped areas were identified in both kidneys, which were hypointense on T2-weighted images and hyperintense on diffusion-weighted images. The serum IgG4 levels of this patient were elevated to nearly 10-fold the normal upper limit. A diagnosis of IgG4-SC associated with IgG4-related kidney was made. Based on this case, pre-surgery IgG4 serum treatment in patients with non-malignant bile duct stenosis was recommended to exclude IgG4-SC.

Intraductal papillary neoplasm of the bile duct (IPN-B) is a rare preinvasive intraductal pathology of the biliary tract. It should be differentiated from other more common benign or malignant causes of biliary obstruction and dilatation such as calculi or cholangiocarcinoma because the management and prognosis of this condition differs significantly. This case report describes a case of IPN-B in a 45-year-old female patient who presented with non-specific complaints of chronic abdominal pain without jaundice for three months.

Objective To preliminarily evaluate the application value of SpyGlass direct visualization system in the diagnosis and treatment of biliary stricture after liver transplantation. Methods Clinical data of 4 patients presenting with biliary stricture after liver transplantation who underwent SpyGlass direct visualization system examination were collected. The examination, treatment and prognosis of biliary stricture were analyzed. Results The examination results of color Doppler ultrasound, magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERCP) in 4 patients suggested biliary anastomotic stricture with intrahepatic biliary dilatation, and 2 of them were complicated with intrahepatic biliary calculi. Repeated placement of biliary stent under ERCP yielded poor effect in 3 cases. SpyGlass direct visualization system examination hinted biliary anastomotic stricture in 4 patients, 3 cases of intrahepatic biliary dilatation, 3 cases of intrahepatic biliary calculi, 2 cases of purulent bile and 3 cases of floccules within the biliary tract, 1 case of congestion and edema of biliary tract wall and 2 cases of local epithelial necrosis and stiffness changes of intrahepatic biliary tract wall. The wire could not be inserted in 1 patient due to severe biliary anastomotic stricture. Four patients were treated with biliary stricture resection + biliary stone removal + biliary end-to-end anastomosis, biliary stricture resection + biliary-intestinal anastomosis, ERCP lithotomy + biliary metal stent implantation, and biliary metal stent implantation + percutaneous transhepatic bile duct lithotomy, respectively. Relevant symptoms were relieved without evident complications. All patients survived during the follow-up until the submission date. Conclusions Compared with traditional imaging examination, SpyGlass direct visualization system may more directly display the morphological characteristics of biliary tract wall and structural changes within biliary tract cavity, which is an effective examination tool for biliary stricture after liver transplantation. In addition, individualized treatment methods may be adopted for different biliary tract diseases, which is expected to improve clinical prognosis of patients. [ABSTRACT FROM AUTHOR]

Intraductal papillary neoplasm of the bile duct (IPN-B) is a rare preinvasive intraductal pathology of the biliary tract. It should be differentiated from other more common benign or malignant causes of biliary obstruction and dilatation such as calculi or cholangiocarcinoma because the management and prognosis of this condition differs significantly. This case report describes a case of IPN-B in a 45-year-old female patient who presented with non-specific complaints of chronic abdominal pain without jaundice for three months. [ABSTRACT FROM AUTHOR]

The choledochal cyst (CC) can be better termed as biliary tract malformation because of the close association of embryology and etiology in the causation of CC. Contrary to Babbitt's postulation of reflux, damage and dilatation, reflux was not demonstrable as the causative factor in all varieties of CC. High pressure in the biliary system, otherwise termed ductal hypertension, is put forth as an alternative to explain the evolution of CC. The forme fruste type, which does not find a place in the standard classification, typifies the ductal hypertension hypothesis. Hence a closer, in-depth review would be able to highlight this apt terminology of biliary tract malformation., Competing Interests: Conflict-of-interest statement: No conflict of interest declared., (©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.)

Objective: To investigate the value of intravoxel incoherent motion diffusion-weighed magnetic resonance imaging (IVIM-DWI) in discriminating the pathological grades of hilar cholangiocarcinoma (HC). Patients and methods: Thirty-seven HC patients were enrolled and received routine and advanced DWI scanning with multiple b-values. IVIM-DWI images were obtained using echo-planar imaging sequence. Results: The consistency of the maximum cross-sectional area ROI measuring method was higher than that of the repeated sampling ROI measuring method. ADCslow values were closely correlated with the pathological grades of HC. The degrees of biliary dilatation and MELD scores had no influence on the negative correlation between ADCslow values and the pathological degrees of HC patients. Conclusions: ADCslow values could be applied in indicating the pathological grades of HC, which was independent on the extent of biliary dilatation. [ABSTRACT FROM AUTHOR]

This case report describes a four‐year‐old boy who presented with the diagnosis of LCH with liver involvement. This required a living‐related liver transplant one year later. The primary disease recurred in the transplanted liver 6 months post‐transplant and led to progressive biliary dilatation. A percutaneous trans‐hepatic cholangiogram was performed five years after transplant, showing a pattern of multifocal biliary duct strictures mimicking the pattern of primary sclerosing cholangitis and a stenosis of the biliary‐enteric anastomosis. Despite management with an internal‐external biliary drain, the stenosis of the biliary‐enteric anastomosis evolved to an occlusion one year after drain removal. This was associated with progression of the changes in the biliary tree, this time associated with significant saccular dilatations secondary to the multiple areas of stenosis. Due to these findings and progressive deterioration of the function of the graft, the patient required re‐transplantation. This report illustrates the findings in imaging of the biliary tree secondary to the recurrence of LCH after liver transplantation, which may help to recognize this complication to physicians facing a similar clinical scenario. [ABSTRACT FROM AUTHOR]

Objective·To investigate the application value of lowering of intrapancreatic plate approach in surgical management of intrapancreatic choledochal cyst. Methods·From January 2016 to December 2019, 15 patients with choledochal cyst (Dong type C2) in Renji Hospital, Shanghai Jiao Tong University School of Medicine were retrospectively studied. All patients underwent intrapancreatic choledochal cyst excision through the lowering of intrapancreatic plate approach. Preoperative judgement of junction between choledochal cyst and main pancreatic duct would be verified in operation. The operative success rate, operation time, intraoperative blood transfusion rate, postoperative complications, postoperative pathological results, clinical outcome and follow-up (survival state and long-term complications) were analyzed. Results·Preoperative judgement results of junction between choledochal cyst and main pancreatic duct were confirmed in operation. Main pancreatic duct joined with normal bile duct distal to choledochal cyst in 7 (46.7%) patients, and main pancreatic duct joined with choledochal cyst in 8 (53.3%) patients. All the 15 patients underwent successful intrapancreatic choledochal cyst excision through the lowering of intrapancreatic plate approach without remnant intrapancreatic choledochal cysts and main pancreatic duct damage. The operation time was (170.0±22.7) min, and the blood transfusion rate was 0. Postoperative complications occurred in 8 patients, including pancreatic leakage (grade biochemical), pancreatic leakage (grade B), gastroplegia, pancreatic leakage (grade biochemical) combined with bile leakage, intestinal obstruction, pancreatic leakage (grade biochemical) combined with intestinal obstruction, intraperitoneal effusion with infection and incision infection. Postoperative pathological results showed that all patients were diagnosed as choledochal cyst with chronic inflammation of mucosa. After treatment, all patients were cured and discharged.After 5 - 48 months of follow-up, all patients survived well, without the sign of anastomotic stricture after hepaticojejunostomy, biliary dilatation and carcinogenesis. Conclusion·Intrapancreatic choledochal cyst excision through the lowering of intrapancreatic plate approach is safe and effective. [ABSTRACT FROM AUTHOR]

IgG4-related sclerosing cholangitis (IgG4-SC) is a relatively newly identified disease that is frequently associated with autoimmune pancreatitis. The differential diagnosis between cholangiocarcinoma, primary sclerosing cholangitis, and IgG4-SC can be challenging due to significant overlap among the clinical and imaging characteristics. We report the case of a 71-year-old woman who was diagnosed with IgG4-related disease based on increased serum IgG4 levels, imaging, and clinical presentation, which showed systemic involvement, including sclerosing cholangitis and kidneys. The patient presented with chronic jaundice. Magnetic resonance imaging revealed bile duct strictures and the dilatation of upstream bile ducts, smooth wall thickening with uniform enhancement in the delayed phase, and no vascular infiltration. Multiple low-density, wedge-shaped areas were identified in both kidneys, which were hypointense on T2-weighted images and hyperintense on diffusion-weighted images. The serum IgG4 levels of this patient were elevated to nearly 10-fold the normal upper limit. A diagnosis of IgG4-SC associated with IgG4-related kidney was made. Based on this case, pre-surgery IgG4 serum treatment in patients with non-malignant bile duct stenosis was recommended to exclude IgG4-SC. [ABSTRACT FROM AUTHOR]

Ampullary Gangliocytic paraganglioma is a rare gastro-entero-pancreatic neuroendocrine tumor with a characteristic histologic appearance involving epitheliod, spindle and ganglion cells. Endoscopic ultrasound is useful to determine depth of invasion and regional metastasis. Endoscopic resection, Ampullectomy and pancreato-duodenenectomy have been described as management options in literature but no consensus has been established. The index case describes an asymptomatic 42 y/o lady with a background of plexiform neurofibromatosis referred for cholestatic derangement of liver function. She was found to have a dilated biliary system with a periampullary mass which proved to be a Gangliocytic Paraganglioma without local lymph node metastasis. She underwent successful endoscopic ampullectomy with complete normalization of LFT and decompression of the biliary system with no clinical or endoscopic recurrence at 2 years follow up. We advocate that Partial Ampullectomy could be done safely as a treatment of localized Ampullary GP allowing long term resolution of symptoms.

Purpose: Various studies have shown the benefit of three-dimensional (3D) systems over two-dimensional (2D) systems in endoscopic surgery. However, few studies have focused on pediatric endosurgery. The purpose of this study was to assess the impact of 2D and 3D environments on the time taken and forceps manipulation by comparing experts and trainees using a laparoscopic hepaticojejunostomy simulator. Methods: We have developed a simulator of laparoscopic hepaticojejunostomy for congenital biliary dilatation. Seventeen participants of pediatric surgeons (4 experts and 13 trainees) performed hepaticojejunostomy using our simulator in both 2D and 3D environment. We evaluated the required time, total path length, and average velocities of bilateral forceps in both situations. Results: Obtained results show the findings for the required time (seconds; 2D, experts: 810.43 ± 321.64 vs. trainees: 1136.02 ± 409.96, P = .17) (seconds; 3D, experts: 660.21 ± 256.48 vs. trainees: 1017 ± 280.93, P = .039), total path length of right forceps (mm; 2D, experts: 38838.23 ± 30686.63 vs. trainees: 52005.47 ± 31675.02, P = .48)(mm; 3D, experts: 24443.09 ± 12316.32 vs. trainees: 45508.09 ± 26926.27, P = .16), total path length of left forceps (mm; 2D, experts: 131635.54 ± 71669.94 vs. trainees: 245242.48 ± 130940.25, P = .48)(mm; 3D, experts: 88520.42 ± 56766.63 vs. trainees: 230789.75 ± 107315.85, P = .024), average velocities of right forceps (mm/second; 2D, experts: 44.80 ± 21.85 vs. trainees: 44.37 ± 18.92, P = .97) (mm/second; 3D, experts: 37.28 ± 16.49 vs. trainees: 42.58 ± 14.40, P = .54), average velocities of left forceps (mm/second; 2D, experts: 156.65 ± 38.69 vs. trainees: 202.58 ± 63.80, P = .20) (mm/second; 3D, experts: 125.64 ± 44.30 vs. trainees: 219.42 ± 43.82, P < .01). Conclusion: The experts performed more effectively when using the 3D system. Using 3D, the total path length of the left forceps of expert pediatric surgeons was significantly shorter than trainee pediatric surgeons, and the average velocities of the left forceps tip of expert pediatric surgeons was significantly slower than trainee pediatric surgeons. These results suggest that training of assisting hand is necessary for advanced pediatric endosurgery to avoid organ injury. [ABSTRACT FROM AUTHOR]

Autosomal recessive polycystic kidney disease (ARPKD) is a potentially lethal multi-organ disease affecting both the kidneys and the liver. Unfortunately, there are currently no non-invasive methods to monitor liver disease progression in ARPKD patients, limiting the study of potential therapeutic interventions. Herein, we perform an initial investigation of T1 relaxation time as a potential imaging biomarker to quantitatively assess the two primary pathologic hallmarks of ARPKD liver disease: biliary dilatation and periportal fibrosis in the PCK rat model of ARPKD. T1 relaxation time results were obtained for five PCK rats at 3 months of age using a Look-Locker acquisition on a Bruker BioSpec 7.0 T MRI scanner. Six three-month-old Sprague-Dawley (SD) rats were also scanned as controls. All animals were euthanized after the three-month scans for histological and biochemical assessments of bile duct dilatation and hepatic fibrosis for comparison. PCK rats exhibited significantly increased liver T1 values (mean ± standard deviation = 935 ± 39 ms) compared with age-matched SD control rats (847 ± 26 ms, p = 0.01). One PCK rat exhibited severe cholangitis (mean T1 = 1413 ms), which occurs periodically in ARPKD patients. The observed increase in the in vivo liver T1 relaxation time correlated significantly with three histological and biochemical indicators of biliary dilatation and fibrosis: bile duct area percent ( R = 0.85, p = 0.002), periportal fibrosis area percent ( R = 0.82, p = 0.004), and hydroxyproline content ( R = 0.76, p = 0.01). These results suggest that hepatic T1 relaxation time may provide a sensitive and non-invasive imaging biomarker to monitor ARPKD liver disease. Copyright © 2015 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Ampullary Gangliocytic paraganglioma is a rare gastro-entero-pancreatic neuroendocrine tumor with a characteristic histologic appearance involving epitheliod, spindle and ganglion cells. Endoscopic ultrasound is useful to determine depth of invasion and regional metastasis. Endoscopic resection, Ampullectomy and pancreato-duodenenectomy have been described as management options in literature but no consensus has been established. The index case describes an asymptomatic 42 y/o lady with a background of plexiform neurofibromatosis referred for cholestatic derangement of liver function. She was found to have a dilated biliary system with a periampullary mass which proved to be a Gangliocytic Paraganglioma without local lymph node metastasis. She underwent successful endoscopic ampullectomy with complete normalization of LFT and decompression of the biliary system with no clinical or endoscopic recurrence at 2 years follow up. We advocate that Partial Ampullectomy could be done safely as a treatment of localized Ampullary GP allowing long term resolution of symptoms. [ABSTRACT FROM AUTHOR]

A 7 year old girl infected with the zoonotic trematode, Fasciola gigantica is reported because of the extreme rarity of this condition in our region. Because of the overlap in symptomatology and radiological features, the more common amebic/pyogenic liver abscess in the initial hepatic migratory phase and later choledochal cyst/biliary ascariasis when the parasite was finally located in the extrahepatic bile ducts, were thought of delaying effective treatment. The diagnosis was confirmed only by surgical exploration. The characteristic contrast enhanced computed tomography scan features retrospectively identified were multiple clustered hypodense lesions in the liver with peripheral enhancement in the acute hepatic migratory phase, and periportal tracking in the previously affected areas of the liver with biliary dilatation and a linear hypointense lesion within the common bile duct in the chronic phase. Although a known association, she did not have eosinophilia. This child, who became symptomatic at the age of 5.5 years, also appears to be one of the youngest patients reported with Fasciola gigantica . [ABSTRACT FROM AUTHOR]