Pancreaticobiliary Maljunction Research Articles

Progress in research of pancreaticobiliary maljunction and biliary dilatation

Progress in research of pancreaticobiliary maljunction and biliary dilatation

Helicobacter bilis infection induces oxidative stress in and enhances the proliferation of human cholangiocytes.

Helicobacter bilis, an enterohepatic Helicobacter species, represents a carcinogenic risk factor for cholangiocytes owing to the prevalence of infections in patients with biliary tract cancer, cholecystitis, and pancreaticobiliary maljunction. However, the effect of H.bilis infection on cholangiocytes and the process and mechanism of carcinogenesis are not known. We aimed to determine the effects of H.bilis on cholangiocytes, focusing on inflammation and oxidative stress. Helicobacter bilis and MMNK-1 cells were cocultured for 24 h and inflammatory cytokine secretion was evaluated. Furthermore, MMNK-1 cell proliferation, intracellular reactive oxidant species (ROS) production, and DNA damage caused by ROS were investigated. All factors were compared with and without H.bilis infection. Interleukin (IL)-6 and IL-8 secretion were significantly increased in MMNK-1 cocultures with H.bilis (IL-6, 24.3 ± 12.2 vs. 271.1 ± 286.4pg/ml; IL-8, 167.6 ± 78.7 vs. 1085.1 ± 1047.1 pg/ml, p < .05). MMNK-1 proliferation was also significantly higher in H.bilis cocultures (1.05 ± 0.02 vs. 1.00-fold, respectively; p < .05). Coculturing enhanced the production of ROS in MMNK-1 cells depending on the cell concentration of H.bilis (1.0 vs. 1.17 ± 0.06, p < .05); however, DNA injury was not observed in cocultures with H.bilis (5.35 ± 0.87 vs. 6.08 ± 0.55 pg/μl, p=.06). Helicobacter bilis infection induced ROS production in and enhanced the proliferation of cholangiocytes.

Anomaly of the biliopancreatic junction with associated hepatic duct anomaly – a rare case report

Pancreaticobiliary maljunction (PBM) or anomaly is defined as an anatomical maljunction of the biliary duct and pancreatic duct outside of the duodenal wall beyond the influence of the sphincter of Oddi. Children with this anomaly can present with choledochal cyst with recurrent attacks of acute pancreatitis, and condition can be worse when not treated appropriately. Pancreaticobiliary maljunction or anomaly is considered to be a major risk factor for biliary tract cancer. In patients with this anomaly, free reflux of pancreatic juice into biliary tract may cause biliary tract damage, resulting biliary malignancy can occur. Therefore, in this condition total excision of the extrahepatic bile duct with hepaticojejunostomy is recommended. Early diagnosis of this condition and early surgical treatment is the mainstay to prevent further complications. Proper follow-up is necessary to detect biliary tract malignancy in early post-operative period, especially in patients demonstrating post-operative complications. Here we are describing a young boy with an abnormal pancreaticobiliary junction (PBM) with choledochal cyst associated with hepatic duct anomaly and biliary and pancreatic stones causing recurrent attack of acute pancreatitis.

Combination of UNet++ and ResNeSt to classify chronic inflammation of the choledochal cystic wall in patients with pancreaticobiliary maljunction.

The aim of this study was to establish an automatic classification model for chronic inflammation of the choledoch wall using deep learning with CT images in patients with pancreaticobiliary maljunction (PBM). CT images were obtained from 76 PBM patients, including 61 cases assigned to the training set and 15 cases assigned to the testing set. The region of interest (ROI) containing the choledochal lesion was extracted and segmented using the UNet++ network. The degree of severity of inflammation in the choledochal wall was initially classified using the ResNeSt network. The final classification result was determined per decision rules. Grad-CAM was used to explain the association between the classification basis of the network and clinical diagnosis. Segmentation of the lesion on the common bile duct wall was roughly obtained with the UNet++ segmentation model and the average value of Dice coefficient of the segmentation model in the testing set was 0.839 ± 0.150, which was verified through fivefold cross-validation. Inflammation was initially classified with ResNeSt18, which resulted in accuracy = 0.756, sensitivity = 0.611, specificity = 0.852, precision = 0.733, and area under curve (AUC) = 0.711. The final classification sensitivity was 0.8. Grad-CAM revealed similar distribution of inflammation of the choledochal wall and verified the inflammation classification. By combining the UNet++ network and the ResNeSt network, we achieved automatic classification of chronic inflammation of the choledoch in PBM patients and verified the robustness through cross-validation performed five times. This study provided an important basis for classification of inflammation severity of the choledoch in PBM patients. We combined the UNet++ network and the ResNeSt network to achieve automatic classification of chronic inflammation of the choledoch in PBM. These results provided an important basis for classification of choledochal inflammation in PBM and for surgical therapy.

Retrospective Analysis of the Accuracy of High-Frequency Ultrasound for Pancreaticobiliary Maljunction in Pediatrics at a Single Center.

ObjectiveTo determine the clinical value of high-frequency ultrasonography (US) in the evaluation and diagnosis of pancreaticobiliary maljunction (PBM) among children.MethodsThe clinical subjects consisted of 31 pediatric patients who were diagnosed with PBM from January 2015 to May 2021 in Fujian Provincial Maternity and Children's Hospital. The primary outcomes included diagnosis accuracy, imaging characteristics of each type of PBM based on JSPBM, time length of operation, and cost of service. Secondary outcomes were the serum amylase and bilirubin levels.ResultsThe diagnostic accuracy of US was 90.3% and comparable to the other imaging methods—MRCP (82.6%), IOC (79.2%), and ERCP (100%), respectively. The time length of operation and direct cost were significantly lower than other imaging pathways. Stenotic type (A) is associated with a high internal diameter of CBD, and dilated channel type (C) presents increased internal diameter and length of CC as well as internal diameter of PD. There were higher levels of the serum bilirubin seen in type A and of serum amylase in type C compared with others.ConclusionHigh-frequency US is a safe, cost-effective, and non-invasive imaging tool for the diagnosis and evaluation of PBM in pediatrics.

Pancreaticobiliary maljunction and pancreas divisum accompanied with intestinal malrotation: a case report

BackgroundPancreaticobiliary maljunction is a congenital anatomical abnorma l junction of the pancreatic duct and bile duct into a common channel outside the duodenal wall. Pancreas divisum is also a congenital anatomical abnormality characterized by unfused pancreatic ducts. Intestinal malrotation is caused by the failure of bowel rotation and fixation. We reported an optimal surgical intervention for the rare case of pancreaticobiliary maljunction and pancreas divisum accompanied intestinal malrotation.Case presentationA 2-year-old female presented with fever and jaundice. Abdominal ultrasound showed dilated common bile duct and intrahepatic bile ducts; MRCP showed pancreaticobiliary maljunction, pancreas divisum, and dilated biliary system; Abdominal contrast-enhanced CT showed a reversed relationship between the superior mesenteric artery and the superior mesenteric vein. An operation of laparoscopic resection of the extrahepatic bile duct, Roux-en-Y hepaticojejunostomy, and Ladd’s procedure was performed after the inflammation of the biliary system was treated. The post-operative follow-up period was uneventful.ConclusionsThe management of pancreas divisum can be conservative. We present an optimal pattern of Roux-en-Y hepaticojejunostomy to deal with pancreaticobiliary maljunction associated with intestinal malrotation.

Bile Duct Duplication, Choledocholithiasis, and Pancreaticobiliary maljunction

Bile Duct Duplication, Choledocholithiasis, and Pancreaticobiliary maljunction

Multiseptate gallbladder coexisting with pancreaticobiliary maljunction treated by laparoscopic cholecystectomy: report of a pediatric case

BackgroundA multiseptate gallbladder is a very rare congenital malformation in which the lumen is divided into variously sized multiseptal compartments. The pathogenesis and natural history of this disease remain uncertain. We herein describe a pediatric case of a multiseptate gallbladder with pancreaticobiliary maljunction (PBM), which was treated by laparoscopic cholecystectomy.Case presentationA 5-year-old girl was referred to our hospital, because a multiseptate gallbladder had been incidentally detected on abdominal ultrasonography when she presented for transient abdominal pain. Ultrasonography showed hyperechoic septa throughout the lumen of the gallbladder, giving it a honeycomb appearance. The atrophied gallbladder had weak or no contractility. Magnetic resonance cholangiopancreatography performed to detect other coexisting biliary disorders revealed PBM without dilatation of the common bile duct. Although physical examination and laboratory tests revealed no abnormalities, we performed laparoscopic cholecystectomy to prevent cholecystitis and reduce the risk of cancer secondary to the PBM.ConclusionsIn recent pediatric case reports, the indication and timing of cholecystectomy has tended to be determined by the patient’s symptoms and the presence of biliary complications. In the present case, however, the combination of a multiseptate gallbladder and PBM may become problematic in the future. Surgical treatment without delay was appropriate even in this pediatric patient.

Expression of lncRNAs in children with pancreaticobiliary maljunction: functional analysis and potential biomarkers.

Pancreaticobiliary maljunction (PBM) leads to higher rates of complications, including cholangitis, pancreatitis, and malignancies. The aim of the present study was to investigate the expression profile of long non-coding RNAs (lncRNAs) and their potential role as biomarkers in children with pancreaticobiliary maljunction. The differential expression of lncRNAs and messenger RNA (mRNAs) from pediatric patients with pancreaticobiliary maljunction and control subjects was analyzed using a commercial microarray and later validated with qRT-PCR. The potential biological functions of differentially expressed genes were explored based on Gene Ontology and Kyoto Encyclopedia of Genes and Genomes pathway enrichment. The ability of potential lncRNA biomarkers to predict pancreaticobiliary maljunction was assessed based on the area under the receiver operating characteristic curve (AUC). There were 2915 mRNAs and 173 lncRNAs upregulated, and 2121 mRNAs and 316 lncRNAs downregulated in PBM cases compared to controls. The enriched Gene Ontology categories associated with differentially expressed mRNAs were extracellular matrix, extracellular region, and kinetochore. The most enriched Kyoto Encyclopedia pathway was protein digestion and absorption, which was associated with cancer and PI3K-Akt signaling. Analysis of cis- and trans-target genes predicted that a single lncRNA was able to regulate several mRNAs. The qRT-PCR results for NR_110876, NR_132344, XR_946886, and XR_002956345 were consistent with the microarray results, and the difference was statistically significant for NR_132344, XR_946886, and XR_002956345 (p < 0.05). AUC was significant only for XR_946886 (0.837, p < 0.001). Our results implicate lncRNAs in common bile duct pathogenesis in PBM, and they identify XR_946886 as a potential biomarker for the disease.

Synchronous Extrahepatic Cholangiocarcinoma and Gallbladder in Australian Patient Without an Abnormal Pancreatico-biliary Ductal Junction

Synchronous malignancies of bile duct and the gallbladder are rare. These cases are often associated with pancreatico-biliary maljunction which is characterised by a long-shared duct between the sphincter of Oddi, the branches of the common bile duct and the pancreatic duct. This predisposes the biliary epithelium to pancreatic enzyme reflux and makes the development of neoplasia more likely. We describe the case of a 64-year-old Australian female who presented with new jaundice and severe cholecystitis secondary to an impacted gallstone.

Pathophysiology and treatment strategy for congenital biliary dilatation and pancreaticobiliary maljunction

Pathophysiology and treatment strategy for congenital biliary dilatation and pancreaticobiliary maljunction

Use of a C-tube to Assess the Pathophysiology of Pancreaticobiliary Maljunction and High Confluence

当院で施行した腹腔鏡下胆嚢摘出術488例の内，C-tubeを留置した271例に対し，術後胆汁アミラーゼ値の測定と胆道造影検査を実施し，膵管・胆管の合流形態異常と胆汁アミラーゼ値の関連性を検討した．膵管が常時描出された合流異常症例4例は全例とも40,000IU/L以上の値であり，膵管が間歇的に描出される高位合流例は中央値9,423IU/L［7-205,900］であった．通常例は中央値10IU/L［1-399,600］で，この結果より胆汁アミラーゼ値の合流異常・高位合流例と通常例の鑑別のためのcut offの検討を行うと，ROC曲線下面積が最大になるアミラーゼ値は6,698IU/Lであった（ROC曲線下面積=0.904）．これをもとに胆汁アミラーゼの臨床的cut off値を6,600IU/L と設定し，このcut off値で検討すると感度85.7％，特異度94.2％で合流異常もしくは高位合流が存在する結果となった．高位合流は合流異常と比較すると胆道癌の発生リスクは低いことは知られているが，その明確な診断基準や治療方針は確立されておらず，今後も症例を積み重ね検討していく必要がある．

Transabdominal Ultrasound Evaluation of Pancreaticobiliary Maljunction in Children.

The development of high-frequency ultrasound made the diagnosis of pancreaticobiliary maljunction (PBM) possible. However, no study has been performed to clarify the sensitivity and specificity of transabdominal ultrasound (TAUS) in the diagnosis of PBM. The purpose of this study was to evaluate the accuracy of TAUS in the diagnosis of pediatric PBM and to assess factors that may influence the accuracy of ultrasound. This was a prospective study and 43 patients with suspected PBM were enrolled. All of these patients underwent TAUS examination to detect the pancreaticobiliary ductal union. Final diagnoses were determined by endoscopic retrograde cholangiopancreatography or intraoperative cholangiography. Sensitivity and specificity were calculated. Fisher exact test was used to analyze the difference of sonographic features between false-negative group and true-positive group. Transabdominal ultrasound demonstrated 77.4% (95% confidence interval, 58.5%-89.7%) sensitivity and 100% (95% confidence interval, 69.9%-100%) specificity for PMB diagnosis. In the false-negative group, infant patients (71.4% vs 16.7%, P = 0.012), cystic dilatation of the common bile duct (CBD) (71.4% vs 16.7%, P = 0.012), and stenosis of the distal CBD (71.4% vs 16.7%, P = 0.012) were more frequently observed than in the true-positive group. On the other hand, the true-positive group showed a higher incidence of protein plugs than the false-negative group (62.5% vs 0%, P = 0.007). Transabdominal ultrasound may serve as a potential alternative detection modality for pediatric patients with suspected PBM. Nondetection of the anomaly may be attributed to factors, such as younger age, cystic dilatation of the CBD, and stenosis of the distal CBD.

Stepwise correlation of TP53 mutations from pancreaticobiliary maljunction to gallbladder carcinoma: a retrospective study

BackgroundThe genetic changes underlying carcinogenesis in patients with risk factors of gallbladder carcinoma (GBC) remains controversial, especially in patients with pancreaticobiliary maljunction (PBM). This study aimed to clarify the association between risk factors of GBC and genetic changes using next-generation sequencing (NGS).MethodsWe retrospectively analyzed resected tissues of 64 patients who were diagnosed with GBC (n = 26), PBM [with GBC (n = 8), without GBC (n = 20)], and chronic cholecystitis, used as a control group (n = 10). DNA was extracted from tumors and their surrounding tissues, which were precisely separated by laser-capture microdissection. Gene alterations of 50 cancer-related genes were detected by NGS and compared with clinical information, including PBM status.ResultsThe most frequent gene alterations in GBC tissues occurred in TP53 (50%), followed by EGFR (20.6%), RB1 (17.6%), and ERBB2 (17.6%). Gene alterations that were targetable by molecular targeted drugs were detected in 20 cases (58.8%). Statistical analysis of gene alterations and risk factors revealed that TP53 alteration rate was higher in GBC patients with PBM than those without PBM (p = 0.038), and the TP53 mutation rates in the epithelium of control patients, epithelium of PBM patients without GBC, peritumoral mucosa of GBC patients with PBM, and tumor tissue of GBC patients with PBM were 10, 10, 38, and 75%, respectively (p < 0.01).ConclusionsTP53 alteration more than KRAS mutation was revealed to underlie carcinogenesis in patients with PBM.

Fatal pancreatic fistula after laparoscopic distal pancreatectomy for intraductal papillary mucinous carcinoma with pancreaticobiliary maljunction and sphincterotomized papilla: a case report

BackgroundPancreatic juice is constantly activated by contaminated bile in patients with pancreaticobiliary maljunction (PBM). Here, we report a case of laparoscopic distal pancreatectomy for a patient with PBM and sphincterotomized papilla, resulting in fatal pancreatic fistula.Case presentationA 79-year-old man was diagnosed with pancreatic intraductal papillary mucinous neoplasm and common bile duct stones. Endoscopic sphincterotomy was performed prior to surgery. The pancreatic duct was simultaneously visualized when the contrast agent was injected into the common bile duct. Sudden bleeding was observed from the abdominal drain on postoperative day (POD) 6. Emergent stent graft placement and coil embolization were performed for bleeding from the splenic artery. On POD 9, the drainage fluid changed to yellowish in color with bile contamination. For internal drainage of the digestive fluid, endoscopic retrograde biliary tube and pancreatic drainage tube were placed. On POD 24, second emergent coil embolization was performed for bleeding from the left gastric artery. On POD 25, open abdominal drainage was performed. On POD 32, third emergent coil embolization was performed for bleeding from the gastroduodenal artery. Subsequently, remnant pancreatic resection was performed. On POD 39, massive bleeding was again observed from the abdominal drain. Emergency arterial portography revealed bleeding in the right wall of the superior mesenteric vein. The patient died of hemorrhagic shock on the same day.ConclusionsThe extreme risk of severe pancreatic fistula after distal pancreatectomy should be considered in patients with PBM and sphincterotomized papilla. In this extraordinary situation, surgeons should promptly decide whether to resect the remnant pancreas to prevent losing the patient.

Application of Embedding Hepaticojejunostomy in Children with Pancreaticobiliary Maljunction Without Biliary Dilatation.

Background: To investigate the effect of embedded hepaticojejunostomy in children with pancreaticobiliary maljunction (PBM) without biliary dilatation. Materials and Methods: The clinical data of 10 patients with nondilated PBM from February 2017 to July 2020 were retrospectively analyzed. Perioperative liver function indexes were compared. Results: All patients were diagnosed by magnetic resonance cholangiopancreatography (MRCP) combined with intraoperative cholangiography. There were 5 cases of Komi type I and 5 cases of type II; the diameter of the common bile duct was 4-9 mm (median: 6 mm); and the length of the common channel was 5-15 mm (median: 9.25 mm). The procedure for one patient with common duct stones was converted to open surgery. Laparoscopic cholecystectomy, common bile duct resection, and embedded hepaticojejunostomy were successfully performed in all 10 cases. The average operation time was 225 ± 96.64 min, and the intraoperative blood loss was 2-5 mL. The mean time to oral intake was 3.5 ± 1.65 days (range: 2-5 days), and the mean hospitalization duration was 6.2 ± 2.44 days (range: 5-8 days). The differences in liver function indexes in the perioperative period were statistically significant (P < .05). The patients were followed-up for 13 to 54 months (median: 40 months). All patients grew well and there was no bile duct dilatation, calculus, or cirrhosis on B-ultrasound examinations. Conclusions: The clinical manifestations of nondilated PBM are often concealed, and preoperative MRCP was important for obtaining a diagnosis. Laparoscopic cholecystectomy, common bile duct resection, and embedded hepaticojejunostomy are feasible for treating nondilated PBM.

Utility of endoscopic retrograde cholangiopancreatography in infant patients with conservational endoscopy.

BackgroundThe indications for endoscopic retrograde cholangiopancreatography (ERCP) in infant patients (age <1 year) differ from those in adults. A paucity of data and concerns about the potential lower effectiveness and more adverse effects limit its utility, even in tertiary care centres. In this study, we retrospectively analysed the indications, success rates, and adverse effects of ERCP in these groups.MethodsFrom June 2014 to March 2018, 17 ERCPs were performed in 15 children [median age: 10.4 months (6–12 months); median weight: 6.6 kg (3.3–10.7 kg)]. A conventional duodenoscope was utilized in all procedures. All patients were followed up as inpatients.ResultsFifteen therapeutic and two diagnostic procedures were managed by licensed paediatric endoscopist. Successful cannulation was obtained in all patients (100%). A high proportion (47.1%) of pancreaticobiliary maljunction (PBM) and therapeutic procedures (88.2%) were identified. Two cases of mild pancreatitis were recorded, accounting for 12.5% of the post-ERCP pancreatitis (PEP) rate. By multivariable analysis, recurrent acute pancreatitis and pancreatic duct (PD) cannulation/injection were identified as PEP-related risk factors. All complications were managed conservatively.ConclusionsERCP in infant patients (0.5< age <1 year) could be safely completed by conservational endoscopy. A high proportion of PBM and therapeutic procedures were identified in our study cohort. The overall adverse-event rate was acceptable, and no serious complication occurred. The PEP-related independent risk factors included recurrent acute pancreatitis and PD cannulation/injection.

S3487 Pancreaticobiliary Maljunction Presenting as Mimic of Primary Sclerosing Cholangitis

S3487 Pancreaticobiliary Maljunction Presenting as Mimic of Primary Sclerosing Cholangitis

A Case of Pancreaticobiliary Maljunction with Repeated Pancreatitis Accompanied by Intestinal Malrotation

A Case of Pancreaticobiliary Maljunction with Repeated Pancreatitis Accompanied by Intestinal Malrotation

Spontaneous common bile duct perforation due to choledocolithiasis accompanied with pancreaticobiliary maljunction in an adult: a case report

BackgroundSpontaneous common bile duct (CBD) perforation is an extremely rare disease in adults. We report an adult case of CBD perforation due to choledocolithiasis accompanied with pancreaticobiliary maljunction, which is, to our knowledge, the first such case report based on a search using PubMed.Case presentationA 71-year-old woman with consciousness disorder was transported to the emergency department of another hospital. She was diagnosed as having severe peritonitis with septic shock and transferred to our hospital for emergency surgery. Enhanced computed tomography (CT) revealed supraduodenal CBD dilation similar to a diverticulum and a defect of bile duct wall continuity. Furthermore, CT showed a long common channel of the pancreaticobiliary duct, so she was diagnosed as having spontaneous CBD perforation with pancreaticobiliary maljunction. Emergency surgery was performed that revealed a necrotic diverticulum-like change on the supraduodenal part, and a 2.5 × 1 cm perforation was found on the anterolateral wall of the CBD. Peritoneal lavage was performed, and CBD perforation was resolved with a T-tube. The patient suffered refractory intra-abdominal and retroperitoneal abscess formation and bleeding from the abdominal wall, which required a long period of postoperative management. The T-tube was removed on day 136, and the patient was transferred on day 153.ConclusionThe cause of CBD perforation is commonly considered to be increased intraductal pressure or weakness of the bile duct wall. In this case, pancreaticobiliary maljunction may have significantly influenced onset and the postoperative course. This case suggests that early surgical intervention and appropriate drainage are important to ensure survival.