Ectopic Opening of Common Bile Duct to Duodenal Bulb: A Single Tertiary Center Experience.

BACKGROUND: The ectopic opening of the common bile duct(CBD) into the duodenal bulb is a rare biliary anomaly. The study aimed to reveal the experience with clinical and endoscopic outcomes in these patients. MATERIALS AND METHODS: This study was conducted on 57 consecutive patients who underwent endoscopic retrograde cholangiopancreatography (ERCP) for ectopic opening of the CBD into the duodenal bulb at our institution between 2010 and 2020. RESULTS: The median age was 59 years (49 males). A total of 146 ERCP procedures were performed (once in 26 patients and 2 or more times in 31 patients). Ten patients had a history of unsuccessful ERCP in an external center. The median follow-up time was 14.6 months. All patients had a slit-like opening of the CBD into the duodenal bulb, apical stenosis, and hook-shaped distal CBD. ERCP findings were CBD stone or dilatation in 55 patients and post-cholecystectomy biliary leakage in 2 patients. Balloon dilatation was performed for apical stenosis in 7 patients and distal CBD stenosis in 26 patients. During the first ERCP session, biliary stent/nasobiliary drainage was placed in 37 patients, and CBD stones were extracted in 19 patients without stenting. Biliodigestive anastomosis was applied to 13 patients, 5 of whom had recurrent cholangitis, 7 required recurrent ERCP, and one was due to the technical difficulty of ERCP. CONCLUSIONS: Ectopic biliary opening should be remembered if the papilla cannot be seen in its usual place in a patient with apical stenosis. ERCP should be performed in experienced hands, and surgery should be considered in the need for recurrent ERCP.

A proposed feasible classification of common bile duct duplications based on a newly described variant and review of existing literature.

BACKGROUND: Duplication of the common bile duct (CBD) is extremely rare among the anatomical variations in the biliary tract system, which presents a septum within the CBD or an accessory CBD. In our study, we report a rare case of duplication of the common bile duct combined congenital biliary dilatation.we present a rare case of a septum in the dilated biliary tract. CASE PRESENTATION: We reported a 5-year-old Asian girl who had history of repeated abdominal pain for 4 days and aggravated for 1 day. Magnetic resonance cholangiopancreatography (MRCP) examination revealed duplicated common bile duct (DCBD) malformation with congenital biliary dilatation and distal cholelithiasis. The patient underwent choledochal cyst resection and biliary tract reconstruction and abdominal cavity irrigation and drainage under general anesthesia. A septum was found within the common bile duct during the operation. The septum divided the extrahepatic bile duct into two parts connected to the left and right hepatic ducts respectively and the gallbladder is attached to the repeated right bile duct which was not previously reported in the literature. CONCLUSIONS: We complement and adjust the classification of common bile duct duplication by reviewing the literature.

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

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.

Pancreatobiliary Maljunction-associated Gallbladder Cancer Is as Common in the West, Shows Distinct Clinicopathologic Characteristics and Offers an Invaluable Model for Anatomy-induced Reflux-associated Physio-chemical Carcinogenesis.

OBJECTIVE: To determine the associations of pancreatobiliary maljunction (PBM) in the West. BACKGROUND: PBM (anomalous union of common bile duct and pancreatic duct) is mostly regarded as an Asian-only disorder, with 200X risk of gallbladder cancer (GBc), attributed to reflux of pancreatic enzymes. Methods: Radiologic images of 840 patients in the US who underwent pancreatobiliary resections were reviewed for PBM and contrasted with 171 GBC cases from Japan. RESULTS: Eight % of the US GBCs (24/300) had PBM (similar to Japan; 15/ 171, 8.8%), in addition to 1/42 bile duct carcinomas and 5/33 choledochal cysts. None of the 30 PBM cases from the US had been diagnosed as PBM in the original work-up. PBM was not found in other pancreatobiliary disorders. Clinicopathologic features of the 39 PBM-associated GBCs (US:24, Japan:15) were similar; however, comparison with non-PBM GBCs revealed that they occurred predominantly in females (F/M = 3); at younger (<50-year-old) age (21% vs 6.5% in non-PBM GBCs; P = 0.01); were uncommonly associated with gallstones (14% vs 58%; P < 0.001); had higher rate of tumor-infiltrating lymphocytes (69% vs 44%; P = 0.04); arose more often through adenoma-carcinoma sequence (31% vs 12%; P = 0.02); and had a higher proportion of nonconventional carcinomas (21% vs 7%; P = 0.03). Conclusions: PBM accounts for 8% of GBCs also in the West but is typically undiagnosed. PBM-GBCs tend to manifest in younger age and often through adenoma-carcinoma sequence, leading to unusual carcinoma types. If PBM is encountered, cholecystectomy and surveillance of bile ducts is warranted. PBM-associated GBCs offer an invaluable model for variant anatomy-induced chemical (reflux-related) carcinogenesis.

[Surgical treatment of common bile duct malformations in children]. / Khirurgicheskoe lechenie mal'formatsii obshchego zhelchnogo protoka u detei.

The review is devoted to the current classifications of common bile duct malformations, authors' opinion regarding surgical approach and reconstruction of the bile ducts. The authors describe the modern modifications of bile duct reconstructions and estimate their effectiveness. PubMed database, Google Scholar and National Scientific Electronic Library eLIBRARY.ru databases were used. We analyzed the complications in children depending on various reconstructions, anatomical form, timing of reconstructive surgery and early diagnosis of this pathology.

Different characteristics of infants diagnosed with congenital choledochal malformation prenatally or postnatally.

The general condition, clinical and pathological characteristics, and treatment regimens of patients prenatally and postnatally diagnosed with congenital choledochal malformation (CM) were analyzed in order to investigate the clinical significance of early diagnosis, treatment, and intervention in CM. We retrospectively analyzed 33 children who were admitted to the Children's Hospital of Soochow University between 1 March 2010 and 31 May 2019, and their diagnosis of CM was confirmed by radiological, surgical and pathological findings. All the patients were under 36 months of age. The patients were divided into prenatally diagnosed and postnatally diagnosed groups. There were 16 and 17 CM patients in the prenatally and postnatally diagnosed groups, respectively, with a preponderance of females in both groups. Compared with the prenatally diagnosed group, the postnatally diagnosed group had a higher incidence of abdominal pain and vomiting (p < 0.05) and higher AST, GGT, and TB levels (p < 0.05). Although postoperative histopathological examination showed inflammation in both groups, congestion in the cyst walls and fibrous tissue hyperplasia were more significant in the postnatally diagnosed group (p < 0.05). In addition, operation time, length of time required to resume a normal diet after surgery, and total length of hospitalization differed between the 2 groups (p < 0.05), with the prenatally diagnosed group having a relatively longer operation time and taking longer to resume a normal diet after surgery. However, the total length of hospitalization in the prenatally diagnosed group was shorter than that in the postnatally diagnosed group. Compared with prenatally diagnosed CM patients, more symptoms, greater severity of symptoms, and more time to recovery after surgery were observed in postnatally diagnosed CM patients.

Anatomy variant symptomatic of the duplication of the common bile duct type IV.

INTRODUCTION: Duplicity of the common bile duct (BCBD) is an unusual congenital disorder. CASE REPORT: A 80-year-old woman with duplication of the common bile duct with retrograde endoscopic cholangiopancreatography (ERCP) who did not resolve the symptoms. DISCUSSION: Our case is a variant of type IV to the classification of DCBC. The MR cholangiography and presurgical ERCP allows assessment of the bile ducts, their caliber, and assessment of abnormalities. The treatment before DCBC will depend on the clinic and the type of opening of the CBCA. CONCLUSIONS: It is important to perform a pre-surgical study and during surgery with CIO.

INTRODUCCIÓN: La duplicidad del conducto biliar común (DCBC) es una alteración congénita insólita. CASO CLÍNICO: Mujer de 80 años con duplicación de la vía biliar común con colangiopancreatografía retrógrada endoscópica (CPRE) que no dilucida la clínica. DISCUSIÓN: Este caso es una variante del tipo IV de la clasificación de DCBC. La colangiorresonancia y la CPRE prequirúrgica permiten valorar las vías biliares, su calibre y las anormalidades. El tratamiento depende de la clínica y el tipo de apertura del conducto biliar común accesorio. CONCLUSIONES: Es importante realizar estudio prequirúrgico y durante la operación con colangiografía intraoperatoria.

Survey of the attitudes of hepatopancreatobiliary surgeons in northern Europe to resection of choledochal cysts in asymptomatic Western adults.

Background: Todani type 1 and 4 choledochal cysts are associated with a risk of developing cholangiocarcinoma. Resection is usually recommended, but data for asymptomatic Western adults are sparse. The aim of this study was to investigate diagnostic interpretation and attitudes towards resection of bile ducts for choledochal cysts in this subgroup of patients across northern European centres. Methods: Thirty hepatopancreatobiliary centres were provided with magnetic resonance cholangiopancreatograms and asked to discuss the management of six cases: asymptomatic non-Asian women, aged 30 or 60 years, with variable common bile duct (CBD) dilatations and different risk factors in the setting of a multidisciplinary team (MDT). The Fleiss κ value was calculated to estimate overall inter-rater agreement. Results: For all case scenarios combined, 83·3 and 86·7 per cent recommended resection for a CBD of 20 and 26 mm respectively, compared with 19·4 per cent for a CBD of 13 mm (P < 0·001). For patients aged 30 and 60 years, resection was recommended in 68·5 and 57·8 per cent respectively (P = 0·010). There was a trend towards recommending resection in the presence of a common channel, most pronounced in the 60-year-old patient. High amylase levels in the CBD aspirate led to recommendations to resect, but only for the 13-mm CBD dilatation. There were no differences related to centre size or region. MDT discussion was associated with recommendations to resect. Inter-rater agreement was 73·3 per cent (κ = 0·43, 95 per cent c.i. 0·38 to 0·48). Conclusion: The inter-rater agreement to resect was intermediate, and the recommendation was dependent mainly on the diameter of the CBD dilatation.

Antecedentes: Los quistes de colédoco (choledochal cysts, CC) tipo 1 y tipo 4 de Todani se asocian con un riesgo de desarrollar colangiocarcinoma. Generalmente se recomienda la resección de los mismos, pero los datos para pacientes adultos occidentales son escasos. El objetivo del presente estudio fue investigar la interpretación diagnóstica y actitudes respecto a la resección de las vías biliares por CC en este subgrupo de pacientes atendidos en centros del norte de Europa. Métodos: Se proporcionaron imágenes de colangiopancreatografía por resonancia magnética (magnetic resonance cholangiopancreatography, MRCP) a un total de 30 centros especializados en patología hepatobiliar y se les solicitó que discutieran el tratamiento de seis casos: pacientes del sexo femenino no asiáticas asintomáticas, de edad entre 30 y 60 años con dilataciones variables del colédoco (common bile duct, CBD) y con diferentes factores de riesgo en el marco de un equipo multidisciplinario (multidisciplinary team, MDT). Se calculó el índice kappa de Fleiss para estimar el acuerdo global entre los evaluadores. Resultados: Para todos los escenarios de casos combinados, un 83,3% y un 86,7% recomendaron la resección para un CBD de 20 y 26 mm, respectivamente, en comparación con un 19,4% para un CBD de 13 mm (P < 0,001). En el caso de un paciente de 30 y de 60 años, la resección se recomendó en el 68,5% y 57,8%, respectivamente (P = 0,010). Se observaron tendencias hacia recomendar la resección en presencia de un canal pancreático­biliar común, más pronunciado en el paciente de 60 años. Los niveles elevados de amilasa en el aspirado del CBD condujeron a la recomendación de resecar, pero solo en la dilatación del CBD de 13 mm. No hubo diferencias relacionadas con el tamaño del centro o la región. La discusión en el MDT se asoció con recomendaciones para la resección. El acuerdo entre evaluadores fue 73,3% con un índice kappa de 0,43 (i.c. del 95% 0,38­0,48). Conclusión: El acuerdo entre evaluadores para indicar la resección fue intermedio y la recomendación dependió principalmente del diámetro de la dilatación del CBD.

Type Va extrahepatic bile duct duplication: a case report.

BACKGROUND: Extrahepatic bile duct duplication is an extremely rare congenital anomaly in which two common bile ducts exist. There are five different types of this anomaly and we present an unusual variant of duplication of an extrahepatic biliary system of type Va variety. CASE PRESENTATION: This case report describes a 63-year-old women from rural Nepal who presented with type Va of duplicated extrahepatic bile duct, with chronic calculous cholecystitis and choledocholithiasis. She was managed with cholecystectomy with hepatic ductoplasty and hepaticojejunostomy. CONCLUSION: A rare case of double common bile duct (type Va) complicated by choledocholithiasis, cholangitis, and chronic cholecystitis is reported here. Rare cases are sometimes overlooked by modern diagnostic techniques. Correct diagnosis helps appropriate surgical intervention.

Ectopic Opening of the Common Bile Duct into the Duodenal Bulb: Diagnosis and Therapeutic Management and Considerations for Timing of Surgery and Duration of Follow-up After Initial Endoscopic Retrograde Cholangiopancreatography.

INTRODUCTION: Ectopic opening of the common bile duct (EO-CBD) is a rare pathology, and its management is important. To date, only a few studies have been conducted for EO-CBD due to its low incidence. MATERIALS AND METHODS: A retrospective review was conducted on 54 patients with available prospective data. Among them, 30 patients (group 1) underwent choledochoduodenostomy for reasons other than anomalous opening, and 24 patients (group 2) had an EO-CBD. RESULTS: Endoscopic retrograde cholangiopancreatography (ERCP) was performed for 24 patients with duodenal deformity and apical stenosis. EO-CBD was detected in 2.72% of all patients who underwent ERCP (24/880 ERCPs). The occurrence of hyperbilirubinemia (total bilirubin >1.2 mg/dL) was not significantly different between groups: 3.83±4.57 mg/dL for group 1 and 2.26±2.17 mg/dL for group 2. CONCLUSIONS: Failed ERCP, giant stones, and recurrent episodes (>2) of cholangitis requiring ERCP constitute indications for surgical treatment in cases of EO-CBD into the duodenum.

IL-33 overexpression in gallbladder cancers associated with pancreatobiliary maljunction.

AIMS: To investigate whether genetic or inflammatory pro-oncogenic factors are relevant to the increased risk of gallbladder cancers in patients with pancreaticobiliary maljunction (PBM). METHODS AND RESULTS: Mutations in KRAS exon 2 were examined by a highly sensitive, droplet digital PCR platform using surgically resected specimens of PBM-associated (n = 31) and non-associated gallbladder cancers (n = 49). The tissue expression of IL-6 and IL-33, which are suspected to promote biliary carcinogenesis, was analysed by quantitative real-time PCR and in-situ hybridisation. The incidence of KRAS mutations was similarly low in PBM-associated (five of 32 cases; 16%) and non-associated cancers (four of 49 cases; 8%) (P = 0.272). The tissue expression of IL-33 mRNA, but not IL-6 mRNA, was significantly higher in PBM-associated gallbladder cancers than in gallbladder cancers without PBM (P = 0.004). A similar degree of IL-33 overexpression was also observed in the background non-cancerous mucosa in cases of PBM-associated gallbladder cancers, and was significantly greater than that in PBM cases with cholecystitis alone (P < 0.001). The results of in-situ hybridisation indicated that the source of IL-33 production in PBM-associated carcinomas was the endothelium, cancer cells and non-neoplastic biliary epithelium. In a combined PBM-associated and non-associated cohort, IL-33 overexpression in gallbladder cancers correlated with less aggressive features (e.g. a lower pT stage and longer overall survival), similar to recently reported findings on large-duct cholangiocarcinomas. CONCLUSIONS: KRAS mutations do not appear to be associated with a high risk of malignancy in PBM, while IL-33 overexpression may provide a pro-oncogenic microenvironment in the gallbladder mucosa of patients with PBM.

Intraductal papillary mucinous neoplasm of the main duct in a patient with pancreas divisum.

Pancreas divisum is a congenital anomaly present in 5-10% of the population and is usually asymptomatic. Pancreatic intraductal papillary mucinous neoplasms (IPMN) are mucinous cystic tumors that have malignant potential and are classified according to their location as IPMN of the main duct, branch duct or mixed type. Larger lesions and those originating in the main duct have an increased risk of malignancy. The real incidence is unknown as most lesions are asymptomatic.

Todani Ic cystic dilatation of the bile duct.

The congenital dilation of the bile duct is an infrequent pathology in western countries and is associated with the female sex. It is usually diagnosed clinically with complementary tests and evaluated at an early age and also appears frequently in adults. These dilatations are grouped into five types according to Todani's classification, including type Ic (Figure 1). The treatment of choice for dilatations of the biliary duct Todani type I is the complete excision of the biliary tract due to the susceptibility of malignant degeneration. A reconstruction is performed via a hepaticojejunostomy with a Roux-en-Y loop. Although in non-malignant cases, a papillotomy with prophylactic stent placement using ERCP can be performed as an alternative. We present the case of a 54-year-old female with a history of high blood pressure, she was examined due to generalized abdominal pain which was unrelated to food intake. The blood test did not identify any alterations of interest. Ultrasound identified a fusiform dilation of the common bile duct occupied by lithiasis. ERCP was attempted due to choledocholithiasis, but the procedure was abandoned as it was not feasible to channel the duodenal papilla. The study was completed with NMR cholangiography (transverse plane [Figure 2] and coronal plane [Figure 3]), identifying a diffuse fusiform dilatation of the common bile duct and common hepatic duct, compatible with congenital cystic lesion Todani type Ic. Finally, the patient underwent a hepaticojejunostomy after sectioning of the main bile duct and extraction of choledocholithiasis.

Choledochal malformations: global research, scientific advances and key controversies.

Choledochal malformations (CMs) represent a spectrum of relatively rare and complex congenital anomalies, characterized by abnormal dilatation of the biliary tract in the absence of any acute obstruction. Today, almost 20% of CMs can be detected in-utero using maternal ultrasonography. Formal scientometric analysis was used to identify where modern CM research is taking place and perhaps where our attention should be directed in the future. Thus, this article offers a comprehensive review of recent scientific advances relating to CMs including the current understanding of etiology and classification, whilst also discussing key controversies such as risk of malignant transformation and the role of newer modalities of surgical treatment. Although laparoscopic excision of CMs and biliary reconstruction is nowadays feasible and safe, care should be taken before dispensing with standard open techniques, which have minimal complication rates and proven long-term benefit.