Acute Pancreatitis as a Complication of Choledochal Cyst

Choledochal cysts (CCs) are rare congenital disorders of the biliary tree which are associated with biliary tract and pancreatic complications. Its etiology is still unknown, but some evidences suggest that CCs are caused by infection during embryogenesis. The laboratory evaluation reveals abnormalities due to pancreatobiliary complications, such as ascending cholangitis, cholecystitis, and pancreatitis. Radiologic examination may delineate the structural abnormalities occured in CCs and confirm the diagnosis. The major mortality is caused by cholangiocarcinoma. This case discussed the diagnosis and treatment of a 20-year-old male with choledochal cyst and its complications. Management consists of therapy on complication and definitive therapy

Congenital cystic lesions of the biliary tree

OBJECTIVE: The purpose of this essay is to illustrate the imaging findings of congenital cystic lesions of the biliary tract. CONCLUSION: Congenital cystic lesions of the biliary tract include ductal plate malformations and choledochal cysts and can be recognized with characteristic imaging findings and basic knowledge of the embryologic development of the biliary tree

Synchronous double primary cancers associated with a choledochal cyst and anomalous pancreaticobiliary ductal union

A 60-year-old female was admitted with epigastric pain lasting a month. Preoperative diagnosis was choledochal cyst with anomalous pancreaticobiliaryductal union (APBDU), C-P type. A papillary mass measuring 2.5 × 1.9 cm was found adjacent to the pancreaticocholedochal junction. Gallbladder (GB) cancer was also observed. Pyloric-preserving pancreaticoduodenectomy (PPPD) was performed. The patient received adjuvant chemotherapy/radiation therapy on the tumor bed. The gallbladder cancer showed serosal invasion, while the bile duct cancer extended into the pancreas. Although common bile duct (CBD) cancer lesion showed focally positive for p53 and the gallbladder cancer lesion showed negative for p53, the Ki-67 labeling index of the CBD cancer and GB cancer were about 10% and 30%, respectively. Nine months after curative resection, a stricture on the subhepatic colon developed due to adjuvant radiation therapy. Localized peritoneal seedings were incidentally found during a right hemicolectomy. The patient underwent chemotherapy and had no evidence of tumor recurrence for two years after PPPD

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

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

Biliary Anomalies in Patients With HNF1B Diabetes

Context: The clinical spectrum of organogenetic anomalies associated with HNF1B mutations is heterogeneous. Besides cystic kidney disease, diabetes, and various other manifestations, odd cases of mainly neonatal and posttransplantation cholestasis have been described. The biliary phenotype is incompletely defined. Objective: To systematically characterize HNF1B-related anomalies in the bile ducts by imaging with magnetic resonance imaging (MRI) or magnetic resonance cholangiopancreatography (MRCP). Setting and Patients: Fourteen patients with HNF1B mutations in the catchment area of the Helsinki University Hospital were evaluated with upper abdominal MRI and MRCP. Blood samples and clinical history provided supplemental data on the individual phenotype. Main Outcome Measure(s): Structural anomalies in the biliary system, medical history of cholestasis, other findings in abdominal organs, diabetes and antihyperglycemic treatment, hypomagnesemia, and hyperuricemia. Results: Structural anomalies of the bile ducts were found in seven of 14 patients (50%). Six patients had choledochal cysts, which are generally considered premalignant. Conclusions: Structural anomalies of the biliary system were common in HNF1B mutation carriers. The malignant potential of HNF1B-associated choledochal cysts warrants further studies.Peer reviewe

A Systematic Review and Meta-Analysis on the Prevalence of Variants in the Pancreaticobiliary Duct Junction and Its Association with Cancer

Background/Objectives: The objective of this study was to describe the anatomical variants of the pancreaticobiliary junction and how its position or structural change could be associated with hepatic, duodenal, and pancreatic clinical complications. Methods: We searched MEDLINE, Scopus, Web of Science (WOS), Google Scholar, CINAHL, and EMBASE databases from their inception up to September 2024. Results: Two authors independently performed the search, study selection, data extraction, and assessed the methodological quality with an assurance tool for anatomical studies (AQUA). Finally, the pooled prevalence was estimated using a random effects model. A total of 59 studies with a total of 22,752 participants were included in this review. The overall prevalence of the anomalous pancreaticobiliary junction (APBJ) variant was 12% (95% CI = 6% to 18%). The prevalence of cancer associated with variants of APBJ was 29% (95% CI = 23% to 34%). Conclusions: In the present anatomical systematic review and meta-analysis, we found that a longer common channel correlated with a higher prevalence of bile duct or gallbladder malignancy, due to the backward flow of bile which occurs as a result of the position and distance of the bile ducts, as well as pancreatic failing. Hence, APBJs are of great interest for gastroduodenal surgeon

Choledochal Cyst (CDC)

Choledochal cysts are congenital bile duct anomalies. These cystic dilatations of the biliary tree can involve the extrahepatic biliary radicles, the intrahepatic biliary radicles, or both. The etiology remains unknown, but choledochal cysts are likely to be congenital in nature. Cyst excision is the definitive treatment of choice for choledochal cyst because of the high morbidity and high risk of carcinoma after internal drainage, a commonly used treatment in the past. CDC is a congenital anomaly involving cystic dilatation of various ducts of biliary tree. The precise etiology of extrahepatic cysts continues to remain unclear. The most commonly accepted theory is an anomalous pancreatobiliary duct junction (APBDJ) and abnormal function of the sphincter of Oddi. Proper imaging plays an essential role in preoperative planning. Proper diagnosis evaluation and management is essential for optimal management. Type I cysts are the most frequently encountered. Choledochal cysts can have variable presentations. Hepatobiliary ultrasound and MRCP are the present day standards for imaging; early diagnosis should be the norm to avoid possible late complications of cholangitis, cirrhosis, hepaticolithiasis and spontaneous perforation. Excision of the cyst with hepaticojejunostomy is the best approach

<Review> Variations of the Union between the Terminal Bile Duct and the Pancreatic Duct in Patients with Pancreaticobiliary Maljunction

Background and Purpose: Pancreaticobiliary maljunction (PBM) is an anomaly with an extramural location of the union between the bile duct and the pancreatic duct system in the duodenum, and is probably caused by disarrangement of the embryonic connection between the two duct systems that presents varied clini- cal manifestations. The aim of this study was to analyze and categorize various patterns of connection between the two duct systems in PBM patients in order to improve the precision of the diagnosis of PBM and to establish safe surgical procedures to treat it. Materials and Methods: We made group classifications of the patterns of connection between the two duct systems in 226 PBM patients based on radiologic or/and anatomical examinations.Results: The 226 cases of PBM were divided into three groups: Group A, 2 patients with a complicated con-nection pattern, Group B, 176 patients with a notch or a small duct segment distal to the terminal bile duct, Group C, 48 patients without a notch or the segment at the terminal bile duct. Conclusion: In Group A, the terminal bile duct was probably connected to one of the multi-branched main ducts of the ventral pancreas, while in Group B, it was probably connected to a second branch of the ventral pancreas, and in Group C, directly to the main pancreatic duct.journal articl

Annular pancreas: endoscopic and pancreatographic findings from a tertiary referral ERCP center

Background and Aims Annular pancreas is a congenital anomaly whereby pancreatic tissue encircles the duodenum. Current knowledge of endoscopic findings of annular pancreas is limited to small case series. The aim of this study was to describe the endoscopic and pancreatographic findings of patients with annular pancreas at a large tertiary care ERCP center. Methods This is a retrospective observational study. Our Institutional Review Board–approved, prospectively collected ERCP database was queried for cases of annular pancreas. The electronic medical records were searched for patient and procedure-related data. Results From January 1, 1994, to December 31, 2016, 46 patients with annular pancreas underwent ERCP at our institution. Index ERCP was technically successful in 42 patients (91.3%), and technical success was achieved in all 46 patients (100%) after 2 attempts, when required. A duodenal narrowing or ring was found in most patients (n = 39, 84.8%), yet only 2 (4.3%) had retained gastric contents. Pancreas divisum was found in 21 patients (45.7%), 18 of which were complete divisum. Pancreatobiliary neoplasia was the indication for ERCP in 7 patients (15.2%). Pancreatographic findings consistent with chronic pancreatitis were noted in 15 patients (32.6%) at the index ERCP. Conclusion This is the largest series describing the endoscopic and pancreatographic findings of patients with annular pancreas. We found that 45.7% of patients had concurrent pancreas divisum. Endoscopic therapy was successful in most patients at our institution after 1 ERCP, and in all patients after a second ERCP. Nearly one-third of patients had findings consistent with chronic pancreatitis at the time of index ERCP. It is unclear whether this may be a feature of the natural history of annular pancreas