• Classification
• Congenital biliary dilation
• Diagnosis
• Imaging
• Prognosis

• Humans
• Dilatation, Pathologic/congenital
• Male
• Female
• Infant
• Retrospective Studies
• Child, Preschool
• Child
• Bile Duct Diseases/classification
• Bile Duct Diseases/diagnostic imaging
• Bile Duct Diseases/congenital
• Infant, Newborn
• Feasibility Studies

• 81571473 National Natural Science Foundation of China

• Contrast-enhanced harmonic endoscopic ultrasound
• Endoscopic ultrasonography
• Pancreaticobiliary
• Pancreaticobiliary maljunction
• Pancreatobiliary ducts

• Humans
• Male
• Female
• Retrospective Studies
• Endosonography/methods
• Middle Aged
• Adult
• Contrast Media
• Pancreaticobiliary Maljunction/diagnostic imaging
• Aged
• Magnetic Resonance Imaging/methods
• Tomography, X-Ray Computed/methods
• Young Adult
• Pancreatic Ducts/diagnostic imaging
• Pancreatic Ducts/abnormalities
• Adolescent

• Biliary intervention
• Common Bile Duct
• Gastrointestinal surgery
• Paediatric Surgery
• Pancreas and biliary tract

• Humans
• Male
• Spontaneous Perforation/surgery
• Hepatic Duct, Common/surgery
• Hepatic Duct, Common/injuries
• Child
• Abdomen, Acute/etiology
• Abdomen, Acute/surgery
• Laparotomy/methods
• Drainage

• Adult
• Child
• Humans
• Prevalence
• Pancreas/surgery
• Pancreas/abnormalities
• Pancreatic Diseases/epidemiology
• Pancreatic Diseases/surgery
• Pancreatic Diseases/complications
• Duodenal Obstruction/complications
• Duodenal Obstruction/diagnosis
• Duodenal Obstruction/surgery
• Digestive System Abnormalities/epidemiology
• Digestive System Abnormalities/surgery

• anomalous pancreaticobiliary duct union
• chronic pancreatitis
• ercp
• pancreatitis
• recurrent acute pancreatitis

• child
• endoscopic retrograde cholangiopancreatography
• magnetic resonance cholangiopancreatography
• pancreaticobiliary malformation
• pancreatitis

• anomalous
• anomaly
• bile duct
• carcinogenesis
• carcinoma
• gallbladder
• hyperplasia
• junction
• maljunction
• reflux
• union

• Artificial intelligence
• Deep learning
• Fast MR imaging
• Magnetic resonance cholangiopancreatography

• Bile Duct Neoplasms/pathology
• Bile Duct Neoplasms/surgery
• Bile Ducts/abnormalities
• Bile Ducts/pathology
• Bile Ducts/surgery
• Carcinoma/pathology
• Carcinoma/surgery
• Carcinoma in Situ/pathology
• Cell Transformation, Neoplastic/pathology
• Choledochal Cyst/pathology
• Choledochal Cyst/surgery
• Dilatation, Pathologic
• Female
• Humans
• Male
• Middle Aged
• Mucous Membrane/abnormalities
• Mucous Membrane/pathology
• Mucous Membrane/surgery
• Retrospective Studies

Synchronous malignancies involving the gallbladder and the bile duct are exceedingly rare. Moreover, their association with anomalous pancreaticobiliary duct junction (APBDJ) has been reported mostly from the Far Eastern countries. Over time, many studies have suggested the definite risk of malignancy attributable to the ‘carcinogenic anatomical configuration’ of the long common biliopancreatic channel, allowing reflux of pancreatic juices in the biliary tract. In this report, we present a case of an elderly man from South India who was initially diagnosed with synchronous gall bladder with bile duct malignancy; the patient turned out to have an APBDJ on further evaluation.

• anomalous pancreaticobiliary duct junction
• cholangiocarcinoma
• gallbladder carcinoma
• pancreaticobiliary maljunction

• Biliary
• Management
• Perforation
• Spontaneous

• choledochal cyst
• congenital biliary dilatation (CBD)
• pancreaticobiliary maljunction (PBM)
• pediatric
• perforation

• Recurrent acute pancreatitis
• chronic pancreatitis
• dorsal duct syndrome
• endoscopic retrograde cholangiopancreatography
• idiopathic pancreatitis
• magnetic resonance cholangiopancreatography
• pancreas divisum

Endoscopic retrograde cholangiopancreatography (ERCP) has been widely used in pediatric patients with cholangiopancreatic diseases.

To evaluate the efficacy, safety, and long-term follow-up results of ERCP in symptomatic pancreaticobiliary maljunction (PBM).

A multicenter, retrospective study was conducted on 75 pediatric patients who were diagnosed with PBM and underwent therapeutic ERCP at three endoscopy centers between January 2008 and March 2019. They were divided into four PBM groups based on the fluoroscopy in ERCP. Their clinical characteristics, specific ERCP procedures, adverse events, and long-term follow-up results were retrospectively reviewed.

Totally, 112 ERCPs were performed on the 75 children with symptomatic PBM. Clinical manifestations included abdominal pain (62/75, 82.7%), vomiting (35/75, 46.7%), acholic stool (4/75, 5.3%), fever (3/75, 4.0%), acute pancreatitis (47/75, 62.7%), hyperbilirubinemia (13/75, 17.3%), and elevated liver enzymes (22/75, 29.3%). ERCP interventions included endoscopic sphincterotomy, endoscopic retrograde biliary or pancreatic drainage, stone extraction, etc. Procedure-related complications were observed in 12 patients and included post-ERCP pancreatitis (9/75, 12.0%), gastrointestinal bleeding (1/75, 1.3%), and infection (2/75, 2.7%). During a mean follow-up period of 46 mo (range: 2 to 134 mo), ERCP therapy alleviated the biliary obstruction and reduced the incidence of pancreatitis. The overall effective rate of ERCP therapy was 82.4%; seven patients (9.3%) were lost to follow-up, eight (11.8%) re-experienced pancreatitis, and eleven (16.2%) underwent radical surgery, known as prophylactic excision of the extrahepatic bile duct and hepaticojejunostomy.

ERCP is a safe and effective treatment option to relieve biliary or pancreatic obstruction in symptomatic PBM, with the characteristics of minor trauma, fewer complications, and repeatability.

• Cholangiopancreatic diseases
• Endoscopic retrograde cholangiopancreatography
• Pancreaticobiliary maljunction
• Pancreatitis
• Pediatric patients
• Follow-up

• Adolescent
• Bile Ducts/abnormalities
• Bile Ducts/diagnostic imaging
• Bile Ducts/surgery
• Child
• Child, Preschool
• Cholangiopancreatography, Endoscopic Retrograde/adverse effects
• Cholangiopancreatography, Endoscopic Retrograde/methods
• Female
• Fluoroscopy
• Follow-Up Studies
• Humans
• Infant
• Male
• Pancreatic Ducts/abnormalities
• Pancreatic Ducts/diagnostic imaging
• Pancreatic Ducts/surgery
• Pancreaticobiliary Maljunction/diagnostic imaging
• Pancreaticobiliary Maljunction/surgery
• Postoperative Complications/epidemiology
• Postoperative Complications/etiology
• Retrospective Studies
• Treatment Outcome

Pancreaticobiliary maljunction (PBM) is an uncommon congenital anomaly of the pancreatic and biliary ductal system, defined as a union of the pancreatic and biliary ducts located outside the duodenal wall. According to the Komi classification of PBM, the common bile duct (CBD) directly fuses with the ventral pancreatic duct in all types. Pancreas divisum (PD) occurs when the ventral and dorsal ducts of the embryonic pancreas fail to fuse during the second month of fetal development. The coexistence of PBM and PD is an infrequent condition. Here, we report an unusual variant of PBM associated with PD in a pediatric patient, in whom an anomalous communication existed between the CBD and dorsal pancreatic duct.

A boy aged 4 years and 2 mo was hospitalized for abdominal pain with nausea and jaundice for 5 d. Abdominal ultrasound showed cholecystitis with cholestasis in the gallbladder, dilated middle-upper CBD, and a strong echo in the lower CBD, indicating biliary stones. The diagnosis was extrahepatic biliary obstruction caused by biliary stones, which is an indication for endoscopic retrograde cholangiopancreatography (ERCP). ERCP was performed to remove biliary stones. During the ERCP, we found a rare communication between the CBD and dorsal pancreatic duct. After clearing the CBD with a balloon, an 8.5 Fr 4-cm pigtail plastic pancreatic stent was placed in the biliary duct through the major papilla. Six months later, his biliary stent was removed after he had no symptoms and normal laboratory tests. In the following 4-year period, the child grew up normally with no more attacks of abdominal pain.

We consider that ERCP is effective and safe in pediatric patients with PBM combined with PD, and can be the initial therapy to manage such cases, especially when it is combined with aberrant communication between the CBD and dorsal pancreatic duct.

• Pancreaticobiliary maljunction
• Pancreas divisum
• Endoscopic retrograde cholangiopancreatography
• Variant
• Communication
• Children
• Case report

• acute relapsing pancreatitis
• pancreas divisum
• pancreatic tumors

• Anatomic Variation
• Humans
• Pancreatic Diseases/etiology
• Pancreatic Ducts/abnormalities
• Pancreatic Ducts/diagnostic imaging
• Risk Factors

This study was designated to evaluate the frequency of anatomic variations of the pancreaticobiliary union.

Our research was observational, comparative and analytical. The investigation was conducted from January 2016-May 2017. This study included 63 patients from Clinic of Gastroenterology and Hepatology – Prishtina, assessed pancreaticobiliary union with Magnetic Resonance cholangiopancreatography.

Union of the common bile duct and the major pancreas was biliary-pancreatic type The angle between common bile duct and the major pancreas duct had different sizes average 35.6°. We did not distinguish significant statistical significance in the size of the pancreaticobiliary angle. In men, the union angle was from the average 36.9°, while in females was average 34.3°. No correlation between the age and size of the angle between common bile duct and the major pancreas duct.

The union of the common bile duct and the major pancreas duct was in most cases B-P Type. The common channel and angle between common bile duct and the major pancreas duct were normal in most cases.

• Diethylnitrosamine
• Hepatocellular carcinoma
• Renin-angiotensin system
• Survival analysis

• Anomaly
• Choledochal cyst
• Congenital biliary dilatation
• Pancreaticobiliary maljunction

• MRCP
• pancreatitis
• secretin

Magnetic resonance cholangiopancreatography (MRCP), MRCP after secretin stimulation (S-MRCP) and endoscopic ultrasonography (EUS) are all selected to diagnose pancreas divisum. However, the accuracies of three diagnosis remain unclear. The aim is to address the diagnostic accuracies of MRCP, S-MRCP and EUS on pancreas divisum. We searched PubMed, MEDLINE and EMBASE databases from inception to January, 2015. Of the 536 citations retrieved, 16 studies were included. For MRCP diagnosis on pancreas divisum, the area under the hierarchical summary receiver-operating characteristic (HSROC) curve was 0.90 (95% confidence interval [CI] 0.87 to 0.92), and for S-MRCP and EUS, 0.99 (95% CI 0.97 to 0.99) and 0.97 (95% CI 0.96 to 0.98). Sensitivity and specificity for MRCP were 0.59 (95% CI 0.45 to 0.71) and 0.99 (95% CI 0.96 to 1.00); for S-MRCP, 0.83 (95% CI 0.66 to 0.92) and 0.99 (95% CI 0.96 to 1.00); for EUS, 0.85 (95% CI 0.67 to 0.94) and 0.97 (95% CI 0.90 to 0.99). Comprehensive comparison of three diagnostic techniques to pancreas divisum, S-MRCP was more reliable than MRCP and EUS on the effect of the diagnostic test.

The aim of this study was to assess factors for delineating the pancreaticobiliary junction in the presence of pediatric congenital choledochal cysts (CCC) using Magnetic resonance cholangiopancreatography (MRCP).

Retrospective review of medical records for 48 patients with CCC was conducted, including demographics, biliary amylase and MRCP findings if available. With univariate and multivariate logistic regression, we measured significant factors affecting pancreaticobiliary maljunction(PBM) diagnoses by MRCP.

Of the subjects enrolled with CCC. Twenty-eight cases had PBM according to MRCP. Univariate analysis confirmed that age, cyst diameter > 30 mm and cysts that descended to the introitus pelvis affected junctional delineation and detection of PBM (P<0.05). Stepwise logistic regression analysis confirmed large cysts in the introitus pelvis predicted pancreaticobiliary junctional delineation in MRCP and these data agreed with the literature. A correlation between cyst diameter and the length of the common channel was found as was cyst diameter and biliary amylase although there were no significant differences between them.

Age, cyst diameter >30 mm and descending cysts into the introitus pelvis affected junctional delineation of the pancreatic and bile duct in PBM with MRCP. Large cyst descension into the introitus pelvis was an independent factors affecting PBM detection.

• Adolescent
• Age Factors
• Bile Ducts/abnormalities
• Bile Ducts/diagnostic imaging
• Child
• Child, Preschool
• Cholangiopancreatography, Endoscopic Retrograde
• Cholangiopancreatography, Magnetic Resonance/methods
• Choledochal Cyst/pathology
• Female
• Humans
• Infant
• Infant, Newborn
• Logistic Models
• Male
• Multivariate Analysis
• Pancreatic Ducts/abnormalities
• Pancreatic Ducts/diagnostic imaging
• Retrospective Studies
• Risk Factors

• Acute pancreatitis
• angle
• common bile duct
• magnetic resonance cholangiopancreatography (MRCP)
• pancreatic duct
• pancreaticobiliary junction

AIM: To investigate whether children with congenital common bile duct dilatation (CBDD) differ from children with obstructive CBDD in cholangiographic characteristics.

METHODS: In this retrospective cohort study, the baseline data and the results of imaging analyses were reviewed among children who had endoscopic retrograde cholangiopancreatography (ERCP) due to CBDD. ERCP was performed on all pediatric patients by experienced pediatric endoscopists. The maximal transverse diameter of the common bile duct (CBD) was measured on ERCP. To assess whether age-adjusted CBDD could be used for differential diagnosis, a CBDD severity index (SI) was calculated by dividing the measured CBD diameter by the age-corrected maximal diameter of a normal CBD.

RESULTS: A retrospective medical chart review revealed that 85 consecutive children under 16 years of age with hepatobiliary disease and CBDD were referred to Seoul Asan Medical Center. Fifty-five (64.7%) children had congenital CBDD and 30 (35.3%) had obstructive CBDD. The two groups did not differ significantly in terms of clinical characteristics except for sex. The congenital and obstructive CBDD groups did not differ significantly in terms of mean CBD diameter (19.3 ± 9.6 mm vs 12.2 ± 4.1 mm, P > 0.05). However, congenital CBDD cases had a significantly higher mean SI than obstructive CBDD cases (3.62 ± 1.64 vs 1.98 ± 0.71, P = 0.01). In multivariate analysis, an SI value ≥ 2.32 and comorbidity with anomalous union of pancreaticobiliary duct (APBDU) in ERCP independently predicted congenital CBDD.

CONCLUSION: Measuring the CBD may aid the differential diagnosis of both CBDD and APBDU in children.

• Endoscopic retrograde cholangiopancreatography
• Common bile duct
• Choledochal cyst
• Choledolithiasis
• Children

• Adolescent
• Age Factors
• Child
• Child, Preschool
• Cholangiopancreatography, Endoscopic Retrograde
• Choledochal Cyst/diagnostic imaging
• Choledocholithiasis/diagnostic imaging
• Cholestasis/diagnostic imaging
• Common Bile Duct/abnormalities
• Common Bile Duct/diagnostic imaging
• Diagnosis, Differential
• Dilatation, Pathologic
• Female
• Humans
• Male
• Predictive Value of Tests
• Republic of Korea
• Retrospective Studies

• anatomy
• biliary duct
• duodenal papilla
• hepatopancreatic ampulla of Vater
• multislice spiral computed tomography
• pancreatic duct

• Children
• Endoscopic retrograde cholangiopancreatography
• Magnetic resonance cholangiopancreatography
• Pancreaticobiliary maljunction

Tumors of the biliary tree are relatively rare; but their incidence is rising worldwide. There are several known risk factors for bile duct cancers, and these are seem to be associated with chronic inflammation of the biliary epithelium. Herein, 2 risk factors have been discussed, primary sclerosing cholangitis and reflux of pancreatic juice into the bile duct, as seen in such as an abnormal union of the pancreatic-biliary junction because magnetic resonance imaging (MRI) is used widely and effectively in the diagnosis of these diseases. When biliary disease is suspected, MRI can often help differentiate between benignity and malignancy, stage tumors, select surgical candidates and guide surgical planning. MRI has many advantages over other modalities. Therefore, MRI is a reliable noninvasive imaging tool for diagnosis and pre-surgical evaluation of bile duct tumors. Nowadays remarkable technical advances in magnetic resonance technology have expanded the clinical applications of MRI in case of biliary diseases. In this article, it is also discussed how recent developments in MRI contributes to the diagnosis of the bile duct cancer and the evaluation of patients with risk factors affecting bile duct cancer.

• Biliary
• Magnetic resonance imaging
• Malignancy
• Primary sclerosing cholangitis
• Pancreas juice
• Reflux

Magnetic resonance imaging (MRI) techniques for assessment of morphology and function of the pancreas have been improved dramatically the recent years and MRI is very often used in diagnosing and follow-up of chronic pancreatitis (CP) patients. Standard MRI including fat-suppressed T1-weighted and T2-weighted imaging techniques reveal decreased signal and glandular atrophy of the pancreas in CP. In contrast-enhanced MRI of the pancreas in CP the pancreatic signal is usually reduced and delayed due to decreased perfusion as a result of chronic inflammation and fibrosis. Thus, morphological changes of the ductal system can be assessed by magnetic resonance cholangiopancreatography (MRCP). Furthermore, secretin-stimulated MRCP is a valuable technique to evaluate side branch pathology and the exocrine function of the pancreas and diffusion weighted imaging can be used to quantify both parenchymal fibrotic changes and the exocrine function of the pancreas. These standard and advanced MRI techniques are supplementary techniques to reveal morphological and functional changes of the pancreas in CP. Recently, spectroscopy has been used for assessment of metabolite concentrations in-vivo in different tissues and may have the potential to offer better tissue characterization of the pancreas. Hence, the purpose of the present review is to provide an update on standard and advanced MRI techniques of the pancreas in CP.

• Magnetic resonance
• Chronic pancreatitis
• Secretin
• Diffusion weighted imaging
• Exocrine pancreatic function

• Animals
• Cholangiopancreatography, Magnetic Resonance
• Contrast Media
• Humans
• Magnetic Resonance Imaging
• Magnetic Resonance Spectroscopy
• Pancreas/pathology
• Pancreas/physiopathology
• Pancreatic Function Tests
• Pancreatitis, Chronic/diagnosis
• Pancreatitis, Chronic/pathology
• Pancreatitis, Chronic/physiopathology
• Predictive Value of Tests
• Prognosis
• Secretin
• Severity of Illness Index

Pancreaticobiliary maljunction (PBM) is an unusual anomalous condition in which the pancreatic duct and bile duct merge outside the duodenal wall and form a long common channel. Pancreas divisum (PD) is a congenital anomaly in which the dorsal and ventral pancreatic ducts fail to fuse. Endoscopic retrograde cholangiopancreatography (ERCP) is the gold standard for diagnosing PD and magnetic resonance cholangiopancreatography (MRCP) is the non-invasive choice. In this study, four cases of patients with unusual PBM in addition to PD are described. The patients presented with abdominal pain, which was caused by distal biliary stricture diagnosed by MRCP. The patients received ERCP and had a good prognosis.

• diagnosis
• pancreas divisum
• pancreaticobiliary maljunction
• therapy

• pancreas divisum
• pancreatitis
• endoscopic ultrasound
• magnetic resonance cholangiopancreatography
• endoscopic retrograde cholangiopancreatography
• computer tomography

Gall bladder carcinoma is the most common biliary tract cancer. Delayed presentation and early spread of tumor make it one of the lethal tumors with poor prognosis. Considering that simple cholecystectomy for T1 disease could offer a potential cure, it is increasingly needed to identify it at early stages. Identification of high-risk cases and offering prophylactic cholecystectomy can decrease the incidence of gallbladder carcinoma. With advances in diagnostic tools like contrast-enhanced endoscopic ultrasound, elastography, multidetctor CT, MRI, and PET scan, we can potentially diagnose gallbladder carcinoma at early stages. This paper reviews the various diagnostic modalities available and an algorithmic approach to early diagnosis of gallbladder carcinoma.

• pancreaticobiliary maljunction
• pediatric
• imaging
• intraoperative cholangiography

AIM: To clarify the strategy for early diagnosis of pancreaticobiliary maljunction (PBM) without biliary dilatation and to pathologically examine gallbladder before cancer develops.

METHODS: The anatomy of the union of the pancreatic and bile ducts was assessed by using endoscopic retrograde cholangiopancreatography (ERCP). Patients with a long common channel in which communication between the pancreatic and bile ducts was maintained even during sphincter contraction were diagnosed as having PBM. Of these, patients in which the maximal diameter of the bile duct was less than 10 mm were diagnosed with PBM without biliary dilatation. The process of diagnosing 54 patients with PBM without biliary dilatation was retrospectively investigated. Histopathological analysis of resected gallbladder specimens from 8 patients with PBM without biliary dilatation or cancer was conducted.

RESULTS: Thirty-six PBM patients without biliary dilatation were diagnosed with gallbladder cancer after showing clinical symptoms such as abdominal or back pain (n = 16) or jaundice (n = 12). Radical surgery for gallbladder cancer was only possible in 11 patients (31%) and only 4 patients (11%) survived for 5 years. Eight patients were suspected as having PBM without biliary dilatation from the finding of gallbladder wall thickening on ultrasound and the diagnosis was confirmed by ERCP and/or magnetic resonance cholangiopancreatography (MRCP). The median age of these 8 patients was younger by a decade than PBM patients with gallbladder cancer. All 8 patients underwent prophylactic cholecystectomy and bile duct cancer has not occurred. Wall thickness and mucosal height of the 8 resected gallbladders were significantly greater than controls, and hyperplastic changes, hypertrophic muscular layer, subserosal fibrosis, and adenomyomatosis were detected in 7 (88%), 5 (63%), 7 (88%) and 5 (63%) patients, respectively. Ki-67 labeling index was high and K-ras mutation was detected in 3 of 6 patients.

CONCLUSION: To detect PBM without biliary dilatation before onset of gallbladder cancer, we should perform MRCP for individuals showing increased gallbladder wall thickness on ultrasound.

• Pancreaticobiliary maljunction
• Pancreatobiliary reflux
• Ultrasound
• Gallbladder cancer
• Endoscopic ultrasonography