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The anatomical abnormalities associated with pancreatitis mainly include pancreas anomalies, pancreaticobiliary maljunction and intestinal duplication.Pancreas anomalies are the most common congenital abnormalities in the triggers of pancreatitis, including pancreas divisum, annular pancreas and heterotopic pancreas prevail.In all these anomalies, the mechanism of pancreatitis is likely due to outlet obstruction.Awareness of these anomalies is necessary to arrange the proper strategy for the treatment of patients with pancreatitis.
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Pancreaticobiliary maljunction (PBM) is a congenital disease with pancreatic and bile ducts joining outside of the duodenal wall. The clinical symptoms of PBM are often characterized by a series of gallbladder and pancreatic disease. PBM has also gradually drawn attention due to recurrent of pancreatitis, bile duct stones and the malignant tumors of biliary system. With the innovative development of endoscopic minimally invasive technology, therapeutic endoscopy may provide new treatment options and ideas for PBM patients, and is expected to achieve the goal of endoscopic minimally invasive intervention to correct structural abnormalities in the pancreaticobiliary duct junction. This paper provided an overview of the definition, endoscopy diagnosis, the pathogenesis and therapeutic endoscopy progress of pancreaticobiliary maljunction.
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Resumen La anomalía de la unión biliopancreática (AUBP) es una malformación congénita en la que los conductos pancreáticos y biliares se unen anatómicamente fuera de la pared duodenal. Debido a la excesiva longitud del canal común, la acción del esfínter no afecta la unión pancreatobiliar, lo que permite el reflujo del jugo pancreático hacia la vía biliar. Se asocia con quistes del colédoco y sus manifestaciones incluyen cáncer de la vía biliar, pancreatitis, coledocolitiasis y colangitis. Es una rara patología, especialmente en países occidentales. La colangiopancreatografía por resonancia magnética (CPRM) se ha convertido en el método más utilizado por no ser invasivo, teniendo en cuenta que la colangiopancreatografía retrógrada endoscópica (CPRE) se encuentra contraindicada en casos de pancreatitis aguda y colangitis, además del riesgo de pancreatitis posterior al procedimiento. La distancia entre la unión biliopancreática y la pared duodenal debe ser superior a 9 mm de longitud para diagnosticar AUBP mediante CPRM. En caso de que mida entre 6 y 9 mm y la acción del esfínter afecte la unión pancreatobiliar, se lo denomina unión biliopancreática alta (UBPA) y se debe confirmar mediante CPRE. Describimos los hallazgos en imágenes de siete pacientes jóvenes con clínica abdominal y diagnóstico de AUBP o UBPA por CPRM, y además revisamos la literatura sobre el tema.
Abstract Pancreaticobiliary maljunction (PBM) is a congenital malformation in which the pancreatic and biliary ducts are anatomically joined outside the duodenal wall. Due to the excessive length of the common channel, the action of the sphincter does not affect the pancreaticobiliary junction, allowing the reflux of pancreatic juice into the bile duct. It is associated with cysts of the common bile duct and its manifestations include cancer of the bile duct, pancreatitis, choledocholithiasis and cholangitis. It is a rare pathology, especially in Western countries. Magnetic resonance cholangiopancreatography (MRCP) has become the most used method because it is non-invasive, taking into account that endoscopic retrograde cholangiopancreatography (ERCP) is contraindicated in cases of acute pancreatitis and cholangitis, in addition to the risk of pancreatitis after the procedure. The distance between the biliopancreatic junction and the duodenal wall must be greater than 9 mm in length to diagnose PBM by MRCP. If it is between 6 to 9 mm and the action of the sphincter affects the pancreaticobiliary junction, it is called high confluence of pancreaticobiliary ducts (HCPBD) and should be confirmed by ERCP. We describe the imaging findings of 7 young patients with abdominal symptoms and diagnosis of PBM or HCPBD by MRCP, and also review the literature on the subject.
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Objective:To explore the clinical characteristics of pancreaticobiliary maljunction (PBM) and its disease spectrum, and to evaluate therapeutic endoscopic retrograde cholangiopancreatography (ERCP).Methods:Data of 52 PBM patients who received therapeutic ERCP procedures for abdominal pain, jaundice and fever in Department of Gastroenterology of Peking University Third Hospital from June 2006 to March 2021 were collected. The clinical characteristics, typing, the change of disease spectrum and ERCP procedures were analyzed.Results:Among 52 PBM patients, female was more common. Abdominal pain and jaundice were the most common clinical manifestations, among which 20 were type Ⅰ, 25 type Ⅱ and 7 type Ⅲ. Half patients had the choledochal cyst. The mean timespan from the first onset to the final diagnosis was 12.2 years. Twenty-four cases (46.2%) had changes in PBM disease spectrum. Among 69 ERCP procedures, 5 (7.2%) failed. Difficult cannulation rate was 34.6% (18/52), and 11 patients underwent advanced cannulation techniques, while it was 15.4% (657/4 275) in the conterpart non-PBM patients in the same period, with significant difference between them ( χ2=14.455, P<0.05). Multiple therapeutic ERCP techniques including endoscopic sphincterotomy, pancreatic stent placement, removal of stones from the duct were applied with the successful rate of 92.8% (64/69). The incidence of post-ERCP pancreatitis was 15.4% (8/52). Conclusion:The chief clinical problem may be changed over time in PBM patients. Although ERCP plays an important role in PBM and its disease spectrum, there may be a higher rate of difficult cannulation and postoperative complications.
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Objective:To study the relationship between pancreaticobiliary maljunction (PBM) with cholangiopancreatic diseases, and to evaluate the efficacy and safety using endoscopic therapy for PBM.Methods:The clinical data of 734 patients treated with ERCP at the Affiliated Hospital of Guizhou Medical University from May 2016 to April 2020 were analyzed retrospectively. Of 31 PBM patients who were finally included in this study, there were 23 patients with benign diseases and 8 patients with malignant diseases. Using the diameter of bile duct, these patients were divided into two groups: dilated bile duct group and the non-dilated bile duct group. The general characteristics of patients, incidences of cholangiopancreatic disease, endoscopic treatment, therapeutic efficacy and follow-up data were analyzed.Results:Of the 31 patients with PBM, 11 were males and 20 were females, aged (56.7±16.2) years. There were 4 patients with choledochal cyst (12.9%) and 6 patients with biliary cancer (19.4%). The incidences were significantly higher than those in non-PBM patients (0.9% and 5.3%, respectively, P<0.05). All 31 patients with PBM underwent endoscopic EST treatment, including 15 patients (48.4%) treated with endoscopic naso-biliary drainage (ENBD), 9 patients (29.0%) with endoscopic retrograde biliary drainage (ERBD), 4 patients (12.9%) with endoscopic papillary balloon dilatation (EPBD)+ ENBD, 1 patient (3.2%) with endoscopic metal biliary endoprothesis (EMBE)+ ENBD, 1 patient with ERBD+ endoscopic retrograde pancreatic drainage (3.2%), and 1 patient with EPBD+ ERBD+ EMBE (3.2%). The operative success rate was 100%. Serum AST, ALT, ALP, GGT, TBil and DBil levels of patients in the benign group and malignant group were significantly decreased postoperatively when compared with the preoperative levels (all P<0.05). One patient (3.2%) developed post ERCP pancreatitis. The preoperative and postoperative NRS scores of the patients in the benign group were 7(6, 8) points compared to 0 (0, 1) points, respectively ( P<0.05). All the 23 patients in the benign group were followed up for (25.13±12.90) months. There were no patients who were loss to follow-up. There was no malignant transformation. Three PMB patients with dilated bile ducts still had attacks of abdominal pain or jaundice. The symptoms of the remaining 20 patients were completely relieved, giving a treatment efficacious rate of 87.0% (20/23). Conclusions:PBM was closely related to choledochal cysts, biliary cancer and other diseases. Endoscopic treatment was efficacious and safe, and provided a safe and feasible treatment in preventing future cholangiopancreatic attacks.
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OBJECTIVE: To identify the best mode for diagnosing and treating the patients with choledochal cysts. METHODS: A retrospective study was performed with medical records of patients diagnosed with choledochal cysts from January 1994 to December 2017. In all cases, the diagnosis was based on ultrasound examination. All the patients underwent cyst resection and were divided in two groups: bile enteric anastomosis in the high portion of the common hepatic duct or in the dilated lower portion. RESULTS: Eighty-one cases were studied. The age of presentation was 4 y 2 mo ± 4 y 1 mo, and the age for the surgical treatment was 5 y 5 mo ± 4 y 6 mo. In 61 cases, US was the only image examination performed. There were 67 cases of Todani type I (82.7%), 13 cases of type IV (16.0%) and one case of type III (1.2%). Nine patients (29.0%) in the first period and 2 patients (4.0%) in the second period presented with postoperative complications (p=0.016). CONCLUSION: In patients with choledochal cysts, US is the only necessary diagnostic imaging. Performing the bile enteric anastomosis in the lower portion of the common hepatic duct is safer and has a lower risk of complications.
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Humans , Child , Bile Ducts/diagnostic imaging , Biliary Tract Surgical Procedures/methods , Choledochal Cyst/surgery , Choledochal Cyst/diagnosis , Postoperative Complications , Choledochal Cyst/classification , Retrospective Studies , Ultrasonography , Treatment OutcomeABSTRACT
Pancreaticobiliary maljunction (PBM) is associated with high risk of epithelial atypical growth and malignant transformation of the bile duct or gallbladder. However, overall changes in genetic expression have not been examined in children with PBM. Genome-wide expression was analyzed using peripheral blood samples from 10 children with PBM and 15 pediatric controls. Differentially expressed genes (DEGs) were identified using microarray. Bioinformatics analysis was conducted using Gene Ontology and KEGG analyses. The top 5 in the up-regulated genes in PBM were verified with qRT-PCR. Receiver operator characteristic curve analysis was conducted to evaluate the predictive accuracy of selected genes for PBM. The microarray experiments identified a total of 876 DEGs in PBM, among which 530 were up-regulated and the remaining 346 were down-regulated. Verification of the top 5 up-regulated genes (TYMS, MYBPC1, FUT1, XAGE2, and GREB1L) by qRT-PCR confirmed the up-regulation of MYBPC1 and FUT1. Receiver operating characteristic curve analysis suggested that FUT1 and MYBPC1 up-regulation could be used to predict PBM, with the area under the curve of 0.873 (95%CI=0.735−1.000) and 0.960 (95%CI=0.891−1.000), respectively. FUT1 and MYBPC1 were up-regulated in children with PBM, and could be used as potential biomarkers for PBM.
Subject(s)
Humans , Male , Infant , Child, Preschool , Child , Pancreatic Ducts/abnormalities , Bile Ducts/abnormalities , Up-Regulation/genetics , Gene Expression Profiling , Fucosyltransferases/genetics , Bile Duct Neoplasms/etiology , Carrier Proteins/genetics , Case-Control Studies , Microarray Analysis , Dilatation, Pathologic/complications , Dilatation, Pathologic/congenital , Gallbladder Neoplasms/etiologyABSTRACT
Objective To summarize our therapeutic experiences on patients with pediatric spontaneous biliary duct perforation.Methods We retrospectively analyzed the clinical data of patients diagnosed as spontaneous biliary duct perforation who were admitted into the Department of Pediatric General Surgery,the Beijing Children Hospital from January 2008 to December 2014,and summarized the therapeutic experiences.Results There were 7 boys and 18 girls,with a average age of 2.4 years (range 11 months to 10 years).Twenty-one patients (84.0％) were diagnosed by ultrasonography.Two patients were treated with conservative therapy and were discharged home.The remaining 23 patients were treated with emergent surgery.Of these patients,9 were treated with cholecystostomy and abdominal drainage (the cholecystostomy group),and the remaining 14 were treated with choledochal drainage and abdominal drainage (the choledochal drainage group).The mean hospitalization stay for the cholecystostomy group was 25.2 days,and 3 patients developed comphcations (33.3％).The mean hospitalization stay for thecholedochal drainage group was 16.1 day,and 2 patients developed complications (14.2％).Twenty-four patients were diagnosed to suffer from congenital choledochal cysts or pancreaticobiliary maljunction by imaging studies during or after surgery.Elective choledochal cystectomy with hepaticojejunostomy were performed on 23 stable patients who developed no severe complications.Conclusions Pediatric spontaneous bile duct perforation is closely related with congenital choledochal cysts,and the pathological basis in diagnosis is pancreaticobiliary maljunction.Bile duct elastic fiber hypogenesis and specific blood supply are important to the onset of perforation.Abdominal ultrasonography plays an important role in the diagnosis.Patients with peritoneal irritation and non-localized perforation should be operated in time,and choledochal drainage with abdominal drainage is a good treatment choice.All patients diagnosed as congenital choledochal cysts or pancreaticobiliary maljunction should undergo elective choledochal cystectomy with hepaticojejunostomy.
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Objective To investigate the relationship between acute biliary pancreatitis and pancreaticobiliary maljunction and the role of magnetic resonance cholangiopancreatography(MRCP) in evaluation of pancreatico biliary maljunction.Methods To compare the liver function indicators of different groups of acute biliary pancreatitis patients(153 cases) associated with pancreatico biliary maljunction and without pancreatico biliary maljunction before and after the conservative treatment.Results The 32 acute biliary pancreatitis patients with pancreatico biliary maljunction detected by MRCP were compared with the 121 cases without pancreatico biliary maljunction.The ALT,AST,GGT after conservative treatment in both group of pancreatico biliary maljunction and Npancreatico biliary maljunction were significantly decreased (P ＜ 0.05).ALT,AST and GGT of pancreatico biliary maljunction group were higher than that of Npancreatico biliary maljunction group with statistical significance (P ＜ 0.05).Conclusions MRCP as a noninvasive cholangiopancreatography study of pancreatico biliary maljunction is a safe and reliable examination method,pancreatico biliary maljunction is one of the important causes of acute biliary pancreatitis.
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Laparoscopic cholecystectomy is the gold standard for the treatment of gallstones and chronic cholecystitis.A patient with gallstone was admitted to the 88th Hospital of PLA in August 2012 and received laparoscopic cholecystectomy.She had bile leakage after operation,and had to accept reoperation.Pancreaticobiliary maljunction and aberrant duct upon gallbladder bed were found postoperatively by T-tube cholangiography and pancreatic amylase test in the bile,which led to a complicated treatment and a tortuous recovery.This article analyzed the treatment process for this disease and explore the proper diagnosis and treatment.
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Pancreaticobiliary maljunction(PBM)is closely related to many surgical diseases in which are commonly seen in clinical practice.However,diagnosis of PBM is difficult and it is easily missed.The lack of knowledge and awareness of PMB has once made PBM “the forgotten corner of abdominal surgery”.This essay summarized the current knowledge on the diagnosis of PBM by medical imaging.
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The widespread availability of multi-detector spiral CT and its post processing techniques,and MRI,provide us with important tools to carry out anatomical studies on pancreaticobiliary maljunction (PBM).Determining the relationship between PBM and biliary diseases contributes not only to a better understanding of pathogenesis and pathological process of biliary diseases,but it also provides evidence for prophylactic treatment.The present study reviewed the definition,classification,pathological anatomy of PBM,relationship between PBM and pancreaticobiliary diseases,and clinical treatments of PBM.
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Objective To investigate the diagnostic value and feasibility of PTC diagnosis on pancreaticobiliary maljunction (IBM), and to summarize the PTC radiological characteristics of PBM. Methods Clinical findings and cholangiopancreatographic results were analyzed retrospectively for a group of consecutive 363 patients with obstructive jaundice receiving the PTCD therapy. Meanwhile the standard selected for cases and diagnostic conditions were established. The length and diameter of pancreaticobiliary common duct, the diameter of pancreatic duct and common bile duct and the confluence angle were measured respectively. The t test and rank sum test were used to analyze the result statistically. Results Thirty-eight cases were radiologicaUy diagnosed as PBM owing to the reference standard and the detection rate was 10.5% (38/363). The length of common duct was (12.6±7.9)mm. The significant difference existed between it and normal value (6ram) (t=5.15 , P <0.05). The site of duodenal papilla had influence on the length of common duct. The diameter of common bile duct, pancreatic duct and common duct near the confluence are (3.7±1.9 ) mm, (2.4±1.3) mm, (3.3±1.4 ) mm, respectively. There was no statistical difference between them and the normal value (t=1.79,2.85,5.72, P>0.05). Fifteen patients' duodenal papilla located the middle of descending duodenum. The length of common duct was (10.6±9.1)mm , the confluence angle was 51.1°±28.0°, the number of the duodenal papilla locating in the inferior 1/3 of descending duodenum, juncture , horizontal part of duodenum was 10, 8,5, respectively. The length of common duet were (9.9±3.7), ( 18.6±8.9), ( 13.9±3.5 ) mm, respectively. The confluence angle were 54.0°±18.6°、48.7°±12.6°、74.4°±18.5°, respectively . The site of duodenal papilla had significant influence on the length of common duct(X2=14.51, P <0.05). Conclusion PTC is a safe, feasible, method to diagnose PBM, and it demonstrates the characteristic findings of PBM.
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Objective To investigate the possible mechanism of pancreaticobiliary maljunction(PBM) on development of congenital choledochal cyst(CCC).Methods Tweenty-one patients with choledochal cysts initially diagnosed by ultrasonography,then underwent ERCP.The concentrations of amylase(AMS) and C-reactive protein(CRP) in bile removed through selective biliary catheterization was analyzed.Results Each of 21 cases of PBM(including 13 cases of C-P type and 8 cases of P-C type) had significantly higher concentrations of AMS and CRP compared with control group(P