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Purpose: We investigated the relationship between bile amylase (AMY) levels and biliary epithelial changes in pancreaticobiliary maljunction (PBM), a congenital anomaly characterized by pancreaticobiliary reflux due to duct fusion outside the duodenal wall. Methods: We enrolled 43 children with congenital biliary dilatation (CBD) of Todani types Ia, Ic, and IVa who underwent surgery at the Hokkaido Medical Center for Child Health and Rehabilitation between November 2007 and June 2023. We defined total AMY exposure in bile as bile AMY levels multiplied by the patient's age (months), representing amount of estimated AMY exposure until surgery. We retrospectively investigated the relationships between bile AMY levels and clinicopathological findings. Results: All patients exhibited hyperplasia in the gallbladder and bile duct epithelium, with dysplasia observed in 13 cases, but no carcinoma. Exposure to bile AMY ≥ 662,400 IU/L × months was an independent risk factor for dysplasia. Conclusion: The amount of estimated AMY exposure in bile rather than AMY levels in the bile is an independent risk factor for dysplasia in the biliary mucosa. [ABSTRACT FROM AUTHOR]

Background: Detection of a common channel outside the duodenal wall is important in diagnosing pancreaticobiliary maljunction (PBM). The present study evaluated the utility of contrast-enhanced harmonic endoscopic ultrasonography (CH-EUS) in diagnosing PBM. Methods: This single-center retrospective study enrolled 45 patients who were diagnosed with PBM or high confluence of pancreatobiliary ducts (HCPBD) between January 2007 and December 2021. The diagnostic sensitivities of contrast-enhanced computed tomography (CE-CT), magnetic resonance imaging (MRI), and CH-EUS for diagnosing PBM were analyzed. Imaging findings were evaluated by two reviewers blinded to the clinicopathological results. Results: Based on diagnostic criteria, 33 patients were diagnosed with PBM and 12 with HCPBD. Compared with the patients with HCPBD, those with PBM had significantly longer common channel (12.5 mm vs. 8.1 mm, P = 0.018) and common bile duct (13.0 mm vs. 8.6 mm, P = 0.049) lengths. The κ-coefficients for differentiating PBM and HCPBD were 0.871 between CE-CT and MRI, 0.330 between CE-CT and CH-EUS, and 0.611 between MRI and CH-EUS. The diagnostic sensitivity of CH-EUS (95.2%) was higher than that of CE-CT (83.3%) and MRI (82.8%), although the differences were not statistically significant. Conclusion: CH-EUS may be useful for the diagnosis of PBM. [ABSTRACT FROM AUTHOR]

Objectives Since neither abdominal pain nor pancreatic enzyme elevation is specific for acute pancreatitis (AP), the diagnosis of AP in patients with pancreaticobiliary maljunction (PBM) may be challenging when the pancreas appears normal or nonobvious on CT. This study aimed to develop a quantitative radiomics-based nomogram of pancreatic CT for identifying AP in children with PBM who have nonobvious findings on CT. Methods PBM patients with a diagnosis of AP evaluated at the Children's Hospital of Soochow University from June 2015 to October 2022 were retrospectively reviewed. The radiological features and clinical factors associated with AP were evaluated. Based on the selected variables, multivariate logistic regression was used to construct clinical, radiomics, and combined models. Results Two clinical parameters and 6 radiomics characteristics were chosen based on their significant association with AP, as demonstrated in the training (area under curve [AUC]: 0.767, 0.892) and validation (AUC: 0.757, 0.836) datasets. The radiomics-clinical nomogram demonstrated superior performance in both the training (AUC, 0.938) and validation (AUC, 0.864) datasets, exhibiting satisfactory calibration (P  > .05). Conclusions Our radiomics-based nomogram is an accurate, noninvasive diagnostic technique that can identify AP in children with PBM even when CT presentation is not obvious. Advances in knowledge This study extracted imaging features of nonobvious pancreatitis. Then it developed and evaluated a combined model with these features. [ABSTRACT FROM AUTHOR]

There is no standard surgical approach for pancreaticobiliary maljunction (PBM) without congenital biliary dilatation (CBD). This study aimed to compare outcomes between therapeutic endoscopic retrograde cholangiopancreatography (ERCP) and laparoscopic hepaticojejunostomy (LH) for pediatric patients of PBM without obvious biliary dilatation (PBM-nonOBD). We retrospectively reviewed demographic and clinical data of pediatric patients with PBM-nonOBD from 2015 to 2021. There were 33 patients in ERCP group and 35 patients in LH group. Primary outcomes included treatment efficiency, postoperative recovery, and postoperative complications. Univariate analysis was further used to explore prognostic factors for ERCP. The mean diameter of the common bile duct in LH group was larger than that in ERCP group (8.6 ± 1.3 mm vs. 6.9 ± 2.1 mm, p = 0.003), while there were no significant differences between the two groups in age, gender, clinical manifestations, complications, and other imaging findings. Compared with LH group, ERCP group had a shorter operation time and postoperative recovery time. The treatment effective rate of ERCP was inferior to that of LH (45.4 % vs. 85.7 %, p＜0.001). For postoperative adverse events, post-ERCP pancreatitis (15.1 %) was most common in the ERCP group. 30.3 % of patients eventually required LH. Intestinal obstruction (5.7 %), recurrent cholangitis (5.7 %), gastrointestinal bleeding (2.8 %), and anastomotic stenosis (2.8 %) were observed in LH group and 8.6 % of patients required a reoperation. A long common channel may be associated with poor prognosis after ERCP. ERCP is associated with less surgical trauma, shorter recovery time, and fewer serious complications than LH, while the treatment effective rate of ERCP is inferior to LH. The indications for endoscopic sphincterotomy and the timing of radical surgery need to be further explored. Ⅲ Retrospective Comparative Study. • Therapeutic endoscopic retrograde cholangiopancreatography (ERCP) is considered beneficial for managing pediatric pancreaticobiliary maljunction (PBM). • Compared to laparoscopic hepaticojejunostomy (LH), ERCP has a lower treatment effective rate. • Younger age and longer common channel length are independent factors associated with poor prognosis in PBM-nonOBD (pancreaticobiliary maljunction without obvious biliary dilatation) patients treated with ERCP. [ABSTRACT FROM AUTHOR]

Abstract Background Pancreaticobiliary maljunction (PBM) is a known risk factor for biliary tract cancer. However, its association with carcinoma of the papilla of Vater (PVca) remains unknown. We report a case with PVca that was thought to be caused by the hyperplasia–dysplasia–carcinoma sequence, which is considered a mechanism underlying PBM-induced biliary tract cancer. Case presentation A 70-year-old woman presented with white stool and had a history of cholecystectomy for the diagnosis of a non-dilated biliary tract with PBM. Esophagogastroduodenoscopy revealed a tumor in the papilla of Vater, and PVca was histologically proven by biopsy. We finally diagnosed her with PVca concurrent with non-biliary dilated PBM (cT1aN0M0, cStage IA, according to the Union for International Cancer Control, 8th edition), and subsequently performed subtotal stomach-preserving pancreaticoduodenectomy. Pathological findings of the resected specimen revealed no adenomas and dysplastic and hyperplastic mucosae in the common channel slightly upstream of the main tumor, suggesting a PBM related carcinogenic pathway with hyperplasia–dysplasia–carcinoma sequence. Immunostaining revealed positivity for CEA. CK7 positivity, CK20 negativity, and MUC2 negativity indicated that this PVca was of the pancreatobiliary type. Genetic mutations were exclusively detected in tumors and not in normal tissues, and bile ducts from formalin-fixed paraffin-embedded samples included mutated-ERBB2 (Mutant allele frequency, 81.95%). Moreover, of the cell-free deoxyribonucleic acid (cfDNA) extracted from liquid biopsy mutated-ERBB2 was considered the circulating-tumor deoxyribonucleic acid (ctDNA) of this tumor. Conclusions Herein, we report the first case of PVca with PBM potentially caused by a “hyperplasia–dysplasia–carcinoma sequence” detected using immunostaining and next-generation sequencing. Careful follow-up is required if pancreaticobiliary reflux persists, considering the possible development of PVca.

Introduction: 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. Material and methods: 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). Results: 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 PI3KAkt 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). Conclusions: Our results implicate lncRNAs in common bile duct pathogenesis in PBM, and they identify XR_946886 as a potential biomarker for the disease. [ABSTRACT FROM AUTHOR]

Background: Pancreaticobiliary maljunction (PBM) is a known risk factor for biliary tract cancer. However, its association with carcinoma of the papilla of Vater (PVca) remains unknown. We report a case with PVca that was thought to be caused by the hyperplasia–dysplasia–carcinoma sequence, which is considered a mechanism underlying PBM-induced biliary tract cancer. Case presentation: A 70-year-old woman presented with white stool and had a history of cholecystectomy for the diagnosis of a non-dilated biliary tract with PBM. Esophagogastroduodenoscopy revealed a tumor in the papilla of Vater, and PVca was histologically proven by biopsy. We finally diagnosed her with PVca concurrent with non-biliary dilated PBM (cT1aN0M0, cStage IA, according to the Union for International Cancer Control, 8th edition), and subsequently performed subtotal stomach-preserving pancreaticoduodenectomy. Pathological findings of the resected specimen revealed no adenomas and dysplastic and hyperplastic mucosae in the common channel slightly upstream of the main tumor, suggesting a PBM related carcinogenic pathway with hyperplasia–dysplasia–carcinoma sequence. Immunostaining revealed positivity for CEA. CK7 positivity, CK20 negativity, and MUC2 negativity indicated that this PVca was of the pancreatobiliary type. Genetic mutations were exclusively detected in tumors and not in normal tissues, and bile ducts from formalin-fixed paraffin-embedded samples included mutated-ERBB2 (Mutant allele frequency, 81.95%). Moreover, of the cell-free deoxyribonucleic acid (cfDNA) extracted from liquid biopsy mutated-ERBB2 was considered the circulating-tumor deoxyribonucleic acid (ctDNA) of this tumor. Conclusions: Herein, we report the first case of PVca with PBM potentially caused by a "hyperplasia–dysplasia–carcinoma sequence" detected using immunostaining and next-generation sequencing. Careful follow-up is required if pancreaticobiliary reflux persists, considering the possible development of PVca. [ABSTRACT FROM AUTHOR]

The aim of the study is to clarify the clinicopathological and biliary morphological characteristics in reported cases of diverticular congenital biliary dilatation (CBD). Using PubMed and the Japan Medical Abstracts Society, articles on possible diverticular CBD were extracted and the clinical pictures examined. We also sought evidence for definitions of diverticular CBD and the associated condition of pancreaticobiliary maljunction (PBM) using the original articles by Alonso-Lej and Todani. The characteristic biliary morphologies of cases with images were also investigated. Analyses of 211 possible cases superficially demonstrated multiple diverticula in 12 (12%) and single diverticulum in 89 (88%), with diverticula located in the upper (n = 38, 38%), middle (n = 32, 32%), or lower (n = 26, 26%) biliary tract in and presence of intra-diverticular stones, PBM, and biliary carcinoma in 23% (n = 18), 39% (n = 25), and 11% (n = 14), respectively. However, evidence defining diverticular CBD or justifying the lack of associated PBM was not demonstrated even in the original articles. Scrutiny of the biliary anatomy in 59 cases with images showed incorrect inclusions of types I or IV-A with an irregular biliary duct wall or dilated cystic duct, periampullary choledochal diverticula, or even solitary biliary cysts. Authentic diverticular CBD, representing the diverticulum connected to the middle of the common bile duct via a thin, patent stalk was seen in only 6 cases. Real diverticular CBD appears extremely rare. The lack of an objective definition allows wide interpretations of clinical pictures, creating inconsistencies in the diagnosis and treatment of CBD and raising questions regarding the utility of conventional classifications. Level III. • Diverticular CBD is included in the classic Alonso-Lej and Todani classifications of congenital biliary dilatation (CBD) as a Type 2 cyst. • Scrutiny of previous publications on potential diverticulae of the common bile duct showed a lack of evidence for this type suggesting extreme rarity, and rather overdiagnosis and erroneous inclusion of various biliary morphologies. • The inclusion of this diverticular type in conventional classifications of congenital biliary dilatation has created uncertainties and inconsistency and should probably be removed. [ABSTRACT FROM AUTHOR]

Congenital biliary dilatation (CBD) is a congenital malformation of focal dilatation of the extrahepatic bile ducts, including the common bile duct, and is often associated with pancreaticobiliary maljunction (PBM). In this article, we report a CBD case that presented with focal dilation of the common bile duct without PBM (Todani's classification type Ib). The patient was a 32-year-old man who visited a doctor with a chief complaint of abdominal distension. Computed tomography revealed cystic dilatation of the common bile duct, and the patient was referred to our institution. Magnetic resonance cholangiopancreatography showed cystic dilatation of the common bile duct with a maximum diameter of 7 cm; however, evaluating the presence of PBM was challenging. Endoscopic ultrasonography showed small gallstones and debris in the dilated common bile duct and no thickening of the gallbladder wall. Endoscopic retrograde cholangiopancreatography revealed no PBM or markedly elevated bile amylase levels. Based on these findings, the patient was diagnosed with Todani Type Ib CBD. Since this patient did not have pancreatobiliary reflux, it was unclear whether the risk of developing biliary tract cancer was high, and since the treatment was highly invasive, the decision was to follow up without surgical treatment. [ABSTRACT FROM AUTHOR]

Choledochocele is defined as a congenital dilatation of the distal intramural part of the common bile duct protruding into the wall of the descending duodenum, typically without pancreaticobiliary maljunction. However, some cases present with a similar pathophysiology to pancreaticobiliary maljunction, including reciprocal reflux of pancreatic juices and bile, leading to protein plugs, pancreatitis, and biliary tract carcinogenesis. Choledochocele is relatively rare and its anatomy, physiology, pathology, and clinical features are thus not well known. We describe a patient with choledochocele who suffered from repeated severe acute pancreatitis and underwent subtotal stomach-preserving pancreatoduodenectomy, in whom the pathological findings of choledochocele showed hyperplasia. [ABSTRACT FROM AUTHOR]

Objective To explore the efficacy and risk factors of poor outcomes in children of pancreaticobiliary maljunction without obvious biliary dilatation (PBM-nonOBD) after duodenoscopy. Methods From January 2020 to December 2022, retrospective analysis was conducted 1-on children with PBM-nonOBD treated by duodenoscopy in Children's Hospital of Fudan University. They were assigned into two groups of effective (n=24) and ineffective (n=20) treatment. Demographic profiles, clinical symptoms, laboratory tests and imaging studies were collected and the children were followed up. Univariate and multivariate Logistic regression analyses were performed for identifying the risk factors influencing the outcomes after endoscopy. Receiver operating characteristic (ROC) curve was plotted for evaluate the predictive value of the relevant risk factors. Results During an average follow-up period of (19. 7±8. 6) months, the effective rate was 54. 5%(24/44). Post-endoscopic retrograde cholangiopancreatography pancreatitis was the most common postoperative adverse event (7/44, 15. 9%). Ultimately radical surgery (27. 3%) and repeat endoscopy (15. 9%) were required. No significant inter-group differences existed in such preoperative laboratory parameters as serum amylase, transaminases or bilirubin. B/D-type PBM subtype predominated in effective treatment group, accounting for 41. 7%(10/24) and 37. 5%(9/24) respectively. Univariate analysis indicated that younger age, longer common channel length and greater maximal diameter of common bile duct (CBD) were significant factors associated with poor outcomes after endoscopy (P < 0. 05). Multivariable Logistic regression analysis revealed that younger age (OR=1. 645, 95%CI: 1. 645-2. 309) and longer common channel length (OR=0. 720, 95%CI: 0. 720-0. 968) were independent factors associated with poor outcomes (P < 0. 05). Area under the ROC curve (AUC) was 0. 838(95%CI: 0. 719~0. 958) and 0. 731(95%CI: 0. 567~0. 894) and optimal cutoff values were 4. 9 years and 8. 8 mm respectively. Conclusions With minimal surgical trauma and no serious complications, duodenoscopy can effectively relieve symptoms in children of PBM-nonOBD. Young age and longer common channel length may be associated with poor postoperative outcomes after duodenoscopy. [ABSTRACT FROM AUTHOR]

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]

Purpose: Preoperative comprehension of the anatomical variations of the hepatic artery and bile duct is essential for safe laparoscopic surgery for pancreaticobiliary maljunction (PBM). This study aimed to investigate the impact of anatomical variations of the hepatic artery and bile duct on surgical technique and postoperative complications. Methods: We conducted a retrospective review of patients with PBM who underwent laparoscopic surgery at our institution between January 2014 and December 2022 to investigate anatomical variations in the hepatic artery and bile duct, surgical technique, and postoperative complications. Results: We included 112 patients with PBM, with a median age of 4 years (interquartile range, 0–55). Overall, 29 of 112 patients had an aberrant right hepatic artery (ARHA) running ventral to the common hepatic duct (CHD), and they underwent hepaticojejunostomy on the ventral side of the ARHA. Additionally, eight of 112 patients had an aberrant posterior hepatic duct (APHD), which was joined to the CHD in all but one case. The presence of APHD was associated with postoperative bile leak occurrence. Conclusion: Performing hepaticojejunostomy ventral to the ARHA is important to prevent complications. Furthermore, APHD may be a risk factor for postoperative bile leak and requires careful bile duct plasty. [ABSTRACT FROM AUTHOR]

Ultrasonography is an essential part of the diagnostic process of biliary atresia (BA). The characteristic findings of BA include a hilar hyperechoic zone, the triangular cord sign (TCS), an absence of gallbladder contraction after feeding, and gallbladder atrophy. However, approximately 10% of patients with BA have a normal gallbladder. We herein present two cases of BA with normal morphology of the gallbladder as shown by ultrasonography. In the first case, the patient was positive for the TCS, negative for gallbladder atrophy, and positive for contraction of the gallbladder after feeding; the final diagnosis was hilar obstructive BA complicated by pancreaticobiliary maljunction. In the second case, the patient was positive for the TCS, negative for gallbladder atrophy, and negative for contraction of the gallbladder after feeding; the patient also had common bile duct obstruction and stenosis of the hepatic duct in the hilar region. Based on these two cases, we conclude that gallbladder findings are not diagnostic for BA because in some types, the gallbladder may be normal in morphology and even have the capacity for contraction after feeding.

Abstract Background Pancreaticobiliary maljunction (PBM) is a congenital defect, with risk of developing various pancreaticobiliary and hepatic complications. The presentations of PBM in children and adults are believed to be different, but studies on PBM children of different age groups are limited. This study was to evaluate clinicopathologic characteristics and outcomes in PBM children of different ages. Methods A total of 166 pediatric patients with PBM were reviewed retrospectively. Clinicopathological, imaging, laboratory, surgical, and follow-up data were collected and analyzed. The patients were divided into three age groups, namely, group A ( 3 years, n = 72). Results The major clinical manifestation was jaundice in group A and abdominal pain and vomiting in groups B and C. Acute pancreatitis was more often seen in group C than group A. The length of common channel was significantly longer in group C than group A, while the maximum diameter of common bile duct in group C was smaller than that in group A. Cholangitis and cholecystitis were more commonly performed in groups B and C, while hepatic fibrosis in group A. Whether preoperatively or postoperatively, group C was more likely to have elevated serum amylase, while groups A and B were more likely to present with abnormal liver function indicators, including the increase of aspartate transaminase, alanine transaminase, and gamma-glutamyl transpeptidase. Conclusion Presentation of PBM varies among different pediatric age groups, thus suggesting that targeted management should be carried out according to these differences.

Aim: We aimed to assess management, comorbidities, biliary histopathology and outcomes of choledochal malformations (CM) in adults. Methods: All adult CM patients managed during 1987–2021 at the Helsinki University Hospital were retrospectively reviewed. Results: Of the 39 patients (females 79%) identified, 19 (49%) underwent surgery, 5 (13%) therapeutic ERCP with papillotomy for type 3 CM and 15 (38%) were managed conservatively. Twenty-five (66%) patients had ≥1 comorbidity, and five (13%) patients had associated inflammatory bowel disease (IBD); ulcerative colitis (n = 3) and Crohn's disease (n = 2). Most surgically treated patients underwent hepaticojejunostomy (n = 13) or pancreaticoduodenectomy (n = 3) and they were younger at presentation than other patients [29 (21 – 48) vs. 59 (41 – 71), p = 0.001]. Three patients (23%) suffered from pancreatitis following diagnostic ERCP. Early surgical complications occurred in six (32%) patients, including one anastomotic leakage. Biliary epithelial dysplasia was found in three (15%) patients in resected specimens, while no neoplastic changes were observed. Nine patients (47%) had long-term postoperative complications, cholangitis (n = 5) and anastomotic stricture (n = 3) being the most common ones. No evidence of malignant change was observed after median follow-up of 2.3 (0.60 − 4.5) years included all patient cohorts. Conservatively managed patients remained symptomless with unprogressive biliary tract imaging findings and normal liver biochemistry values during follow-up. Nearly half of operated patients developed long-term postoperative complications. A novel association between CMs and IBD was observed. Although no hepatobiliary malignancies regardless of treatment modality were encountered, the number of patients and length of follow-up remained limited. [ABSTRACT FROM AUTHOR]

Ultrasonography is an essential part of the diagnostic process of biliary atresia (BA). The characteristic findings of BA include a hilar hyperechoic zone, the triangular cord sign (TCS), an absence of gallbladder contraction after feeding, and gallbladder atrophy. However, approximately 10% of patients with BA have a normal gallbladder. We herein present two cases of BA with normal morphology of the gallbladder as shown by ultrasonography. In the first case, the patient was positive for the TCS, negative for gallbladder atrophy, and positive for contraction of the gallbladder after feeding; the final diagnosis was hilar obstructive BA complicated by pancreaticobiliary maljunction. In the second case, the patient was positive for the TCS, negative for gallbladder atrophy, and negative for contraction of the gallbladder after feeding; the patient also had common bile duct obstruction and stenosis of the hepatic duct in the hilar region. Based on these two cases, we conclude that gallbladder findings are not diagnostic for BA because in some types, the gallbladder may be normal in morphology and even have the capacity for contraction after feeding. [ABSTRACT FROM AUTHOR]

Background: Pancreaticobiliary maljunction (PBM) is a congenital defect, with risk of developing various pancreaticobiliary and hepatic complications. The presentations of PBM in children and adults are believed to be different, but studies on PBM children of different age groups are limited. This study was to evaluate clinicopathologic characteristics and outcomes in PBM children of different ages. Methods: A total of 166 pediatric patients with PBM were reviewed retrospectively. Clinicopathological, imaging, laboratory, surgical, and follow-up data were collected and analyzed. The patients were divided into three age groups, namely, group A (< 1 year, n = 31), group B (1–3 years, n = 63), and group C (> 3 years, n = 72). Results: The major clinical manifestation was jaundice in group A and abdominal pain and vomiting in groups B and C. Acute pancreatitis was more often seen in group C than group A. The length of common channel was significantly longer in group C than group A, while the maximum diameter of common bile duct in group C was smaller than that in group A. Cholangitis and cholecystitis were more commonly performed in groups B and C, while hepatic fibrosis in group A. Whether preoperatively or postoperatively, group C was more likely to have elevated serum amylase, while groups A and B were more likely to present with abnormal liver function indicators, including the increase of aspartate transaminase, alanine transaminase, and gamma-glutamyl transpeptidase. Conclusion: Presentation of PBM varies among different pediatric age groups, thus suggesting that targeted management should be carried out according to these differences. [ABSTRACT FROM AUTHOR]

Background: Preoperative diagnosis of liver fibrosis in children with pancreaticobiliary maljunction (PBM) is needed to guide clinical decision‐making and improve patient prognosis. Purpose: To develop and validate an MR‐based radiomics‐clinical nomogram for identifying liver fibrosis in children with PBM. Study Type: Retrospective. Population: A total of 136 patients with PBM from two centers (center A: 111 patients; center B: 25 patients). Cases from center A were randomly divided into training (74 patients) and internal validation (37 patients) sets. Cases from center B were assigned to the external validation set. Liver fibrosis was determined by histopathological examination. Field Strength/Sequence: A 3.0 T (two vendors)/T1‐weighted imaging and T2‐weighted imaging. Assessment: Clinical factors associated with liver fibrosis were evaluated. A total of 3562 radiomics features were extracted from segmented liver parenchyma. Maximum relevance minimum redundancy and least absolute shrinkage and selection operator were recruited to screen radiomics features. Based on the selected variables, multivariate logistic regression was used to construct the clinical model, radiomics model, and combined model. The combined model was visualized as a nomogram to show the impact of the radiomics signature and key clinical factors on the individual risk of developing liver fibrosis. Statistical Tests: Mann–Whitney U and chi‐squared tests were used to compare clinical factors. P < 0.05 was considered statistically significant in the final models. Results: Two clinical factors and four radiomics features were selected as they were associated with liver fibrosis in the training (AUC, 0.723, 0.927), internal validation (AUC, 0.718, 0.885), and external validation (AUC, 0.737, 0.865) sets. The radiomics‐clinical nomogram yielded the best performance in the training (AUC, 0.977), internal validation (AUC, 0.921), and external validation (AUC, 0.878) sets, with good calibration (P > 0.05). Data Conclusion: Our radiomic‐based nomogram is a noninvasive, accurate, and preoperative diagnostic tool that is able to detect liver fibrosis in PBM children. Evidence Level: 3. Technical Efficacy: Stage 2. [ABSTRACT FROM AUTHOR]

Key points Conventional imaging modalities were not powerful enough to diagnose chronic cholangitis. The radiomics signature based on T2-weighted MR images performed well in diagnosing chronic cholangitis. Associating the radiomics signature with clinical factors improved the diagnosis performance of chronic cholangitis.

Choledochal cyst (CC) or congenital biliary dilatation, has a skewed distribution with hereditary features that is far more common in East Asian females. CC is usually associated with pancreaticobiliary malunion (PBMU) forming a common channel. CC requires early definitive diagnosis, since there is a risk for malignancy occurring in the CC and/or intrahepatic bile ducts (IHBD). Complete CC excision and Roux-en-Y hepaticoenterostomy is required and can be performed by open or minimally invasive surgery with hepatojejunostomy the recommended procedure of choice. Principles of open surgical intervention form the basis of minimally invasive management with laparoscopy and robotic assistance. Current surgical management is associated with fewer early and late complications, such as hepaticoenterostomy anastomotic leakage, cholangitis, anastomosis stricture, and cholangiocarcinoma. Specific features of CC management at Juntendo include: intraoperative endoscopy of the common channel and IHBD for inspecting and clearing debris to significantly reduce post-operative pancreatitis or stone formation; near infra-red fluorescence with indocyanine green for visualizing tissue planes especially during minimally invasive surgery for CC; and a classification system for CC based on PBMU that overcomes inconsistencies between existing classification systems and clinical presentation. [ABSTRACT FROM AUTHOR]

Background: Intracholecystic papillary neoplasm (ICPN) is one of the precursors of gallbladder cancer defined in the 2010 World Health Organization classification of tumors. We herein report ICPN with pancreaticobiliary maljunction (PBM), which is a high-risk factor for biliary cancer. Case presentation: A 57-year-old female presented with abdominal pain. Computed tomography showed a swollen appendix and gallbladder nodules with bile duct dilatation. Endoscopic ultrasonography revealed a gallbladder tumor spreading into the cystic duct confluence accompanying PBM. Based on papillary tumors around the cystic duct detected using the SpyGlass DS II Direct Visualization System (SpyGlass DS), ICPN was suspected. We performed extended cholecystectomy, extrahepatic bile duct resection, and appendectomy with a diagnosis of ICPN and PBM. The pathological diagnosis was ICPN (90 × 50 mm) with high-grade dysplasia spreading into the common bile duct. The absence of residual cancer in the resected specimen was pathologically confirmed. P53 staining was totally negative in both the tumor and normal epithelium. The overexpression of CTNNB1 was not observed. Conclusions: We encountered a patient with a very rare gallbladder tumor, ICPN with PBM. SpyGlass DS contributed to a precise assessment of the extent of the tumor as well as a qualitative diagnosis. [ABSTRACT FROM AUTHOR]

Background/Purpose: Congenital biliary dilatation (CBD) is a disease associated with pancreaticobiliary maljunction. The most frequent postoperative complication is intrahepatic stones, which are caused by hilar bile duct stenosis (HBDS). Methods: We retrospectively reviewed the records of patients who underwent primary surgery for CBD between 2013 and 2021. We evaluated images and videos of HBDS, the laparoscopic technique of releasing the stenosis, and its occurrence rate and compared intraoperative bile duct findings with those of preoperative magnetic resonance cholangiopancreatography (MRCP). Results: There were 87 CBD cases in this study. HBDS occurred in 52% and preoperative MRCP for HBDS showed a 96% sensitivity and 74% specificity in this study. Bile duct plasty was performed in 45% of the cases and videos demonstrated typical methods of laparoscopic bile duct plasty. The mid‐ to long‐term complications were hepatolithiasis in three patients, anastomotic site stricture in three, and postoperative obstruction in two. Conclusions: Our study revealed that preoperative imaging studies are useful for the prediction of HBDS. Our laparoscopic meticulous probing method for finding BDS reveals more intrahepatic BDS through magnification. Therefore, this may reduce the incidence of intrahepatic stones. Preoperative imaging and intraoperative findings can be complemented to plan management that reduces long‐term complications. [ABSTRACT FROM AUTHOR]

Purpose: To develop a model to identify risk factors and predictors of acute pancreatitis in children with pancreaticobiliary maljunction (PBM). Methods: We screened consecutive PBM patients treated at two centers between January, 2015 and July, 2021. For machine learning, the cohort was divided randomly at a 6:4 ratio to a training dataset and a validation dataset. Three parallel models were developed using logistic regression (LR), a support vector machine (SVM), and extreme gradient boosting (XGBoost), respectively. Model performance was judged primarily based on the area under the receiver operating curves (AUC). Results: A total of 99 patients were included in the analysis, 17 of whom suffered acute pancreatitis and 82 did not. The XGBoost (AUC = 0.814) and SVM (AUC = 0.813) models produced similar performance in the validation dataset; both outperformed the LR model (AUC = 0.805). Based on the SHapley Additive exPlanation values, the most important variable in both the XGBoost and SVM models were age, protein plugs, and white blood cell count. Conclusions: Machine learning models, especially XGBoost and SVM, could be used to predict acute pancreatitis in children with PBM. The most important contributing factor to the models were age, protein plugs, and white blood cell count. [ABSTRACT FROM AUTHOR]

Duplication of the common bile duct (DCBD) is a rare congenital anomaly, with no more than 100 published cases in the literature. The malignant rate of this condition may reach 25%. Besides, complicated morbidities could co-exist, which might put surgeons in a difficult situation. We report the case of a 36-year-old Vietnamese man, admitted due to epigastric pain and jaundice. The patient was then diagnosed with type II DCBD with comorbidities (choledocholithiasis, pancreaticobiliary maljunction). Cholecystectomy, lithotripsy, and accessory bile duct removal were performed. The patient was discharged uneventfully 1 week later. To conclude, we would like to emphasize the importance of basic knowledge in complicated hepatobiliary cases, for example, DBCD, to provide the optimal approach for these patients. [ABSTRACT FROM AUTHOR]

Abstract An otherwise healthy 45‐year‐old woman had been experiencing intermittent right upper abdominal pain for the past 1 year. Computed tomography showed pneumobilia and pancreatic duct emphysema despite a normal duodenal papilla. Magnetic resonance cholangiopancreatography and endoscopic ultrasound confirmed bile duct dilation but without a pancreaticobiliary maljunction. Duodenoscopy detected a slightly sunken, unfixed, and spontaneously enlarged duodenal papilla. During the cholangiogram, the Oddi sphincter was relaxed and the catheter could be easily inserted into the bile duct. Further, no findings suggestive of pancreaticobiliary maljunction were observed, and the contrast medium leaked spontaneously from the duodenal papilla. As biliary amylase level was high, we surmised the occurrence of occult pancreaticobiliary reflux due to relaxation of the Oddi sphincter. However, as there are no guidelines on the management of this condition, we did not offer any treatment. Nevertheless, the patient continued to experience similar symptoms and was retested 1 year later with similar results. As occult pancreaticobiliary reflux was reconfirmed, we suggested that the patient undergo laparoscopic extrahepatic bile duct resection and cholecystectomy, which is the standard treatment for pancreaticobiliary maljunction. Pathological evaluation revealed fibrous thickening of the bile duct wall and chronic cholecystitis, which are typical findings of pancreaticobiliary reflux. Even though pancreaticobiliary reflux is mainly observed in pancreaticobiliary maljunction, it has also been reported in normal patients. Here, we describe a novel mechanism of pancreaticobiliary reflux, namely, a relaxed or defective Oddi sphincter.

The prognosis for duodenal atresia (DA) is generally good if radical surgery is performed. However, some cases of DA may be complicated by morphological abnormalities in the pancreaticobiliary system, which may cause problems in the distant period. Here, we report a case of gallbladder cancer (GC) that developed more than 20 years after the initial DA operation. A female infant was admitted to our institution with an anorectal malformation after birth. She had Down syndrome, a ventricular septal defect, and DA. She underwent duodenoduodenostomy for DA, during which a Y-shaped biliary duct was found. The patient grew up well postoperatively, and she was followed up at our institution until the age of 12 years. At the age of 24, she was reintroduced to our institution for multiple hepatic tumors and was diagnosed with GC and multiple liver metastases. Simultaneously, a pancreaticobiliary maljunction (PBM) without a choledochal cyst was also identified. She received palliative care and died two months later. Although reports of combined DA and PBM are rare, the combination should not be overlooked, given the carcinogenic risk of PBM. DA with pancreaticobiliary anomalies requires attention to the possibility of PBM during the postoperative follow-up.

Pancreaticobiliary maljunction (PBM) is a congenital malformation. The reflux of pancreatic juice into the biliary tract caused by PBM plays a significant role in the development of biliary tract cancers (BTCs), such as gallbladder cancer and cholangiocarcinoma. Previous studies have demonstrated a high incidence of BTC in patients with PBM. However, there are only a few reports of patients who developed BTC after a diagnosis of PBM. We report the cases of two patients who developed gallbladder cancer after being diagnosed with PBM. They had refused treatment and were being managed with follow-up observation alone after the diagnosis of PBM and developed gallbladder cancer after several years of observation. Thus, surgical treatment should be recommended for all patients with PBM in order to prevent the development of BTC. Moreover, long-term, close, regular follow-up is necessary to facilitate the early diagnosis of subsequent BTC in patients with untreated PBM. [ABSTRACT FROM AUTHOR]

Herein, we report an extremely rare case of intraductal tubulopapillary carcinoma (ITPC) that was detected due to the pancreatic duct dilatation newly appeared on CT after surgery for gallbladder cancer associated with pancreaticobiliary maljunction. Present case: a 77-year-old female. Extended cholecystectomy, extra-bile duct resection, and hepaticojejunostomy was performed and resected specimen showed that this gallbladder tumor was papillary adenocarcinoma, pT2(ss), pN0, pDM0, pHM0, pEM0. Thereafter, the follow-up CT scan 2 years after surgery detected the dilatation of main pancreatic duct (MPD) and the elevation of carcinoembryonic antigen (CEA) level was pointed out (4.9 to 5.9 ng/ml). Moreover, pancreatic juice cytology revealed adenocarcinoma cells. Thus, distal pancreatectomy was performed based on the diagnosis of pancreatic adenocarcinoma associated with pancreaticobiliary maljunction (PBM). Histologically, proliferation of highly columnar atypical cells in the dilated main pancreatic duct with marked papillary and irregular tubular structures is seen. No mucus production is observed. Based on immunohistochemistry, Mucin (MUC) 1, 2 and 5AC were focal weak positive, negative and negative, respectively. Taken together of these findings, we could diagnose this tumor with ITPC without invasive component. The patient is alive without any recurrence for 36 months after a second surgery. In conclusion, it is essential to be fully aware that PBM is a disease in which there is still a possibility that pancreatic or biliary tract cancer may occur in the future, and that careful routine follow-up for a long period after diversion surgery may lead to early detection of complicated cancers. [ABSTRACT FROM AUTHOR]

Abstract Background Pancreaticobiliary 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 presentation A 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. Conclusions The 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.

Introduction 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. Material and methods 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). Results 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). Conclusions Our results implicate lncRNAs in common bile duct pathogenesis in PBM, and they identify XR_946886 as a potential biomarker for the disease.

Abstract Background A 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 presentation A 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. Conclusions In 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.

Abstract Background Pancreatic 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 presentation A 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. Conclusions The 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.

Abstract Background The 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). Methods We 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. Results The 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

Background/purpose: We evaluated the minimally invasive surgery for congenital biliary dilatation (CBD) in adults and children and analyzed the surgical outcomes, especially in children aged <6 years., Methods: Characteristics and surgical outcomes of patients with CBD who underwent minimally invasive surgery at our hospital between 2013 and 2023 were retrospectively reviewed., Results: Overall, 129 patients (89 children aged <6 years, 9 children aged between 6 and 18 years, and 21 adults) were included in this study. Children exhibited more protein plug presence and abnormal biochemical data than adults. Incidence of postoperative pancreatic fistulas was highest in the adult group (3.4%, 11%, and 33%, respectively, p < .01). In children aged <6 years, postoperative bile leakage and pancreatic fistulas occurred in 9.0% and 3.4% of patients, respectively. Multivariate logistic regression analysis revealed that the Todani IVA was associated with a decrease in postoperative bile leakage (odds ratio: -1.7; 95% confidence interval: (-3.3)-(-0.22), p = .03)., Conclusion: Adults with CBD required prolonged operative times and had more short-term complications than children with CBD. In children aged <6 years, minimally invasive surgery for CBD can be safely performed; however, a small diameter of the bile duct may be associated with bile leakage., (© 2024 The Author(s). Journal of Hepato‐Biliary‐Pancreatic Sciences published by John Wiley & Sons Australia, Ltd on behalf of Japanese Society of Hepato‐Biliary‐Pancreatic Surgery.)

A 70-year-old woman was admitted to the hospital 1 month prior to presentation with acute pancreatitis due to pancreaticobiliary maljunction. After discharge, she was referred for elevated hepatobiliary enzyme levels. She was diagnosed with an acute pancreatitis flare-up. Computed tomography revealed dilation of the common duct compared to the previous admission. Considering the protein plug formation as the cause, endoscopic retrograde cholangiopancreatography (ERCP) was performed after improvement. ERCP revealed a defect in the duct, suspected to be caused by protein plugs, which were removed using a balloon after endoscopic papillary balloon dilatation. An analysis revealed that this component was a protein. No recurrence of pancreatitis was observed after the treatment.

Pancreaticobiliary maljunction (PBM) is associated with the development of neoplasms of bile ducts. Cholecystectomy with diversion of the biliary-pancreatic flow is considered the treatment of choice. To describe the surgical treatment employed for a patient with Komi's type 2 PBM and its long-term results. Laparoscopic common bile duct exploration, intraoperative cholangioscopy, and Roux-en-Y hepatico-jejunostomy were performed. Postoperative evolution was satisfactory. The patient was discharge 72 hours after the surgery. There was no associated morbidity. At 62-month follow-up, clinical examination, laboratory tests, and imaging studies confirmed an adequate patency of bilio-enteric anastomosis. The surgical approach employed was effective and safe, with satisfactory long-term results.

Abstract Background Spontaneous 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 presentation A 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. Conclusion The 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.

Abstract Background Pancreaticobiliary maljunction (PBM) is a condition characterized by chronic inflammation due to refluxed pancreatic juice into the biliary tract that is associated with an elevated risk of biliary tract cancer. DNA double-strand breaks (DSBs) are considered the most serious form of DNA damage. DSBs are provoked by inflammatory cell damage and are recognized as an important oncogenic event in several cancers. This study used γ-H2AX, an established marker of DSB formation, to evaluate the impact of DNA damage on carcinogenesis in PBM. Methods We investigated γ-H2AX expression immunohistochemically in gallbladder epithelium samples obtained from 71 PBM cases and 19 control cases. Results Fourteen PBM cases with gallbladder adenocarcinoma were evaluated at non-neoplastic regions. A wide range of nuclear γ-H2AX staining was detected in all PBM and control specimens. γ-H2AX expression was significantly higher in PBM cases versus controls (median γ-H2AX-positive proportion: 14.4 % vs. 4.4 %, p = 0.001). Among the PBM cases, γ-H2AX expression was significantly higher in patients with carcinoma than in those without (median γ-H2AX-positive proportion: 21.4 % vs. 11.0 %, p = 0.031). Conclusions DSBs occurred significantly more abundantly in the PBM gallbladder mucosa, especially in the context of cancer, indicating an involvement in PBM-related carcinogenesis.

Abstract Choledochocele is a rare subtype of choledochal cyst and is associated with increased prevalence of periampullary cancers. Here, we report an unusual manifestation of infiltrative duodenal cancer arising from a choledochocele, involving superficial spreading (muscularis mucosae) of cancer cells along the duodenum causing gastric outlet obstruction, which clinically mimicked superior mesenteric artery syndrome. Histologically, wide spread of cancer cells was confirmed from periampullary region to duodenojejunal junction showing mismatch with radiologic findings, in which the cancer segment was mainly located in the distal duodenum. Clinical, radiologic, and pathologic findings are discussed with literature reviews.

Background: This study aimed to report our experience with a robot-assisted resection of choledochal cysts (CCs) in pediatric patients, especially focusing on changes in outcomes and operative trends. Methods: We retrospectively reviewed medical records of all 158 patients under 18 years of age who underwent robot-assisted resection of CC in a single tertiary center between July 2008 and January 2021. Patients were divided into the first period (P1, July 2008–March 2016; N = 79) and second period (P2, April 2016–January 2021; N = 79) with equal number of participants. The patients of P2 were compared with those of P1 to assess clinical outcomes with operative details. Operative characteristics and postoperative prognosis were compared for each group. Results: The mean operative time was 383.6 min for the P2 group and 462.6 min for the P1 group (p < 0.001). The mean estimated blood loss was 28 mL in the P2 group and 63 mL in the P1 group (p = 0.025). The rate of emergency department visit after the operation was lower in the P2 group (3.8% vs. 13.9%, respectively, p = 0.047). The two groups showed no significant differences in the rate of late postoperative complications and reoperations. Conclusion: With the increase in the center's experience, robot-assisted resection of CC can be safely adopted and feasible, especially for pediatric patients. Levels of evidence: Treatment Study, Level III. [ABSTRACT FROM AUTHOR]

Abstract Background Metachronous pancreatic and gallbladder cancer is a rare condition and has a dismal prognosis. Herein, we present a patient with triple metachronous primary pancreatic and gallbladder cancer associated with pancreaticobiliary maljunction who achieved long-term survival after undergoing repeat curative surgery. Case presentation A 65-year-old female patient with advanced gallbladder cancer associated with pancreaticobiliary maljunction underwent extended cholecystectomy with extrahepatic bile duct resection. The pathological diagnosis was T3N0M0 stage III A papillary adenocarcinoma with hepatic invasion. During a monthly follow-up, a diffuse hypovascular 2.0 × 1.5-cm mass was detected in the pancreatic head 6.2 years after the initial surgery. Hence, the patient underwent pancreaticoduodenectomy. Histological examination revealed T3N0M0 stage IIA well-differentiated adenocarcinoma without lymph node metastases. Marked inflammatory reaction was observed in the non-cancerous lesions of the proximal pancreatic head parenchyma containing bile pigment within ductular lumens. After 12.5 years from the initial surgery, total pancreatectomy for a 4.0 × 3.0-cm mass in the remnant pancreas was performed. Histological examination revealed T3N1M0 stage IIB moderately differentiated adenocarcinoma with lymph node metastases. Hence, surgical curative resection was achieved. Based on the pathological findings, a definitive diagnosis of triple metachronous pancreatic and gallbladder cancer was made. The pathology suggests no precursor lesions such as pancreatic intraepithelial neoplasia (PanIN) and atypical flat lesions, but marked inflammations in the non-cancerous lesions, strengthening our hypothesis that chronic inflammation induced by the pancreaticobiliary maljunction is related to carcinogenesis of the pancreas. Despite further adjuvant chemotherapy, the patient’s general condition worsened; however, she remained alive 15.2 years after the initial surgery while receiving the best supportive care. Conclusions Repeat curative surgery for triple metachronous cancer was associated with a favorable prognosis. Both the biliary tract and the pancreas should be closely monitored during follow-up among patients with pancreaticobiliary maljunction, which can be managed with curative surgery.

Objective To investigate the effect and safety of endoscopic retrograde cholangiopancreatography (ERCP) in the diagnosis and treatment of pancreaticobiliary maljunction (PBM). Methods The patients with PBM were diagnosed and treated using ERCP and included from December 2007 to August 2020 in Hangzhou First People's Hospital of Zhejiang University School of Medicine. The clinical manifestations of PBM, ERCP treatment, and post ERCP complications were analyzed. Results A total of 219 patients with PBM were in this study including 95 males and 124 females with an average age of (52.11±23.32) (2-89) years. Abdominal pain (77.93%) was the main clinical manifestations of PBM. The success rate of endoscopy intubation was 98.17% (215/219), and the cases with curative ERCP was 96.80% (212/219). Twenty-three of ERCP(7.93%) had post ERCP complications cured all by conservative treatment. Conclusions PBM is more common in women with main manifestation of abdominal pain. ERCP is effective and safe for biliary and pancreatic diseases induced by PBM.

Background: The commonly used anatomical classification of congenital biliary dilatation by Todani is not directly related to types of pancreaticobiliary maljunction (PBM). This work is to investigate clinical presentation and surgical outcomes according to PBM classification proposed by the Japanese Study Group on Pancreaticobiliary Maljunction. Pancreaticobiliary junction angle, common channel length, and diameter of dilatation are studied as well. Materials and Methods: Patients of redo operation, without preoperative images, and lacking clearly documented outcomes were excluded. After exclusion, 79 patients who underwent Roux-en-Y hepaticojejunostomy between January 1994 and December 2019 were enrolled for this retrospective study. Medical records were reviewed, and perioperative parameters were collected. Todani's classification, PBM types, pancreaticobiliary junction angle, common channel length, and dilatation diameter were determined based on magnetic resonance cholangiopancreatography or computed tomography. Clinical presentation and outcome were compared between groups of different anatomical features. Results: PBM type A (stenotic), type B (nonstenotic), and type C (dilated channel) consisted of 48 (60.8%), 18 (22.8%), and 11 (13.9%) patients, respectively; and two patients (2.5%) had no PBM. Patients of PBM type A were younger and had more Todani's type Ia lesion. Patients of PBM type B and C had either Todani's type Ia or Ic lesion, but type IVa had only PBM type B. Longer common channel (1.27 vs. 0.81 cm, P < 0.001) and wider dilatation (4 vs. 2 cm, P < 0.001) were found in patients with right pancreaticobiliary angle (90°). Clinical outcome was similar in different Todani's types, PBM types, and pancreaticobiliary angle. Serum alkaline phosphatase level higher than 675 U/L was associated with major perioperative complications. Preoperative jaundice, mass, and dilatation wider than 5 cm were related to subsequent liver cirrhosis. Conclusion: PBM types and pancreaticobiliary junction groups are feasible for surgical planning, but not related directly to outcome. Palpable abdominal mass suggests higher risk of perioperative and late complications. For subsequent liver cirrhosis, laboratory data of disease onset and dilatation diameter wider than 5 cm are important risk factors.

Background and aims: Pancreaticobiliary maljunction (PBM) is a malformation in which the pancreatic and bile ducts join outside the duodenal wall. It is associated with various biliary and pancreatic diseases. In addition, patients with PBM carry a substantial lifetime risk of developing biliary or gallbladder carcinoma. We aimed to present a multicenter case series of PBM from Turkey. Methods: This study was conducted in adult and pediatric PBM patients who were referred to three tertiary reference centers of Turkey for endoscopic retrograde cholangiopancreatography (ERCP) between July 2007 and May 2020. The clinical presentations, types of PBM, ERCP findings, surgical histories, and the postoperative courses, including the development of biliary malignancies, were retrospectively reviewed. Results: The study group included 47 (31 adult and 16 children) patients. Type D PBM was more frequent (13/41: 27.7%) than that reported in Eastern studies. Type A PBM was more common in the adults (51.6% vs. 12.5%, p < 0.05), whereas type C was more common in pediatric patients (31.3% vs. 13.2%, p < 0.05). Although fusiform anatomy was predominant in both of the groups, cystic dilatation was more common (25.8% vs. 12.5%) in adults and the common bile duct diameter was greater [22 mm (range 11–58) vs. 12 mm (range 5–33)] in adult patients compared to pediatric patients. Resective surgeries were more frequently done in pediatric patients (73.3% vs. 53.6%), whereas cholecystectomy was more frequently performed in adult patients (21.4% vs. 6.7%). Conclusion: Although our findings were compatible with Eastern studies, type D PBM (associated with pancreas divisum) was more frequent in our study population. [ABSTRACT FROM AUTHOR]