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BACKGROUND A choledochal cyst (CC), or biliary cyst, is a congenital or acquired anomaly of the biliary tree. Pancreas divisum (PD) is a rare congenital anomaly due to incomplete fusion of pancreatic ducts, which can complicate the clinical course of choledochal cysts. This rare combination is a surgical management challenge. This report presents the diagnosis and management of a 23-year-old woman with a combined choledochal cyst and pancreas divisum treated with pancreaticoduodenectomy. CASE REPORT This article presents the case of a 23-year-old woman who presented with severe, stabbing abdominal pain radiating to the back and epigastric tenderness and was diagnosed with pancreatitis. Initial imaging revealed a choledochal cyst, prompting further investigation with ERCP that showed concomitant PD. She was treated via pancreaticoduodenectomy. During the following 9 years, she was hospitalized over 2 dozen times for recurrent pancreatitis. CONCLUSIONS This report presents a complex case of a combined choledochal cyst and pancreas divisum, which was surgically managed by pancreaticoduodenectomy. The association of CC with PD should be suspected in patients with recurrent acute pancreatitis and/or cholangitis with no identifiable cause. Surgical treatment of CC with PD depends on the classification of the CC, and complications can include recurrent pancreatitis, though prognosis is often favorable. The purpose of this manuscript is to emphasize that pancreaticoduodenectomy is unlikely to provide favorable outcomes for CC with PD, especially considering there is evidence that less extensive surgical interventions produce better outcomes.

We report the case of a 91-year-old female with acute cholangitis and long-standing symptoms of recurrent colic- related abdominal pain after cholecystectomy. She was diagnosed by abdominal CT of saccular dilation of the intramural bile duct in the duodenum suggesting choledococele. ERCP was performed in which the presumptive diagnosis was confirmed, and choledochotomy and choledochoplasty were done with excellent subsequent progress. Choledococele is the least common type of biliary cyst. It consists of a cystic dilation of the intramural portion of the common bile duct that protrudes into the duodenal lumen and causes symptoms of recurrent abdominal pain and biliary events. ERCP is a key test in both diagnosis and treatment except when they cannot be approached by this technique or malignancy is suspected.

Background: Choledochal cyst with perforation (CC with perforation) rarely occurs, early diagnosis and timely treatment plan are crucial for the treatment of CC with perforation. This study aims to forecast the occurrence of CC with perforation., Methods: All 1111 patients were conducted, who underwent surgery for choledochal cyst at our hospital from January 2011 to October 2022. We conducted univariate and multivariate logistic regression analysis to screen for independent predictive factors for predicting CC with perforation, upon which established a nomogram. The predictive performance of the nomogram was evaluated using receiver operating characteristic (ROC) curves, calibration plots, and decision curve analysis (DCA) curves., Results: The age of children with choledochal cyst perforation is mainly concentrated between 1 and 3 years old. Logistic regression analysis indicates that age, alanine aminotransferase, glutamyl transpeptidase, C-reactive protein, vomiting, jaundice, abdominal distension, and diarrhea are associated with predicting the occurrence of choledochal cyst perforation. ROC curves, calibration plots, and DCA curve analysis curves demonstrate that the nomogram has great discriminative ability and calibration, as well as significant clinical utility., Conclusion: The age of CC with perforation is mainly concentrated between 1 and 3 years old. A nomogram for predicting the perforation of choledochal cyst was established., (© 2024. The Author(s).)

Cholangiocarcinoma in patients with Choledochal cysts is rare in childhood; however, it seriously affects the prognosis of the disease. The key to addressing this situation lies in completely removing the extrahepatic cyst. We herein present a case report of a 3-year-old boy with cholangiocarcinoma associated with a choledochal cyst (CDC). Preoperative 3D simulation, based on CT data, played an important role in the treatment of this patient., (© 2024. The Author(s).)

The objective of this study was to analyse the prevalence, risk factors and need for intervention in a sample of Indian children with choledochal cyst (CDC) complicated by pancreatitis with a special focus on chronic pancreatitis. A retrospective review of medical records of children admitted with CDC over 11 years was done and pancreatitis identified using INSPPIRE guidelines. Children were divided into two groups-one having choledochal cyst alone and the other choledochal cyst along with pancreatitis to determine associated risk factors. 40.2% of children with CDC had pancreatitis based on elevation of enzymes or radiological imaging. Age, total bilirubin and indirect bilirubin, requirement of intervention was significantly higher in the group with pancreatitis. 47% of those with radiological features of pancreatitis had imaging features of chronic pancreatitis. Chronic pancreatitis has not been reported previously in children with CDC and maybe peculiar to the Indian subcontinent., (© 2024 European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

• Complications after choledochal cyst resection are common, mainly anastomotic stricture, bowel obstruction, biliary fistula and pancreatic cyst remnant. • Reoperation is the optimal method for patients with pancreatic cyst remnant and hepaticojeju-nal anastomotic stricture. • ERCP, balloon dilation should be considered as a supporting tools to reduce the risk and mor-bidity of surgery. Choledochal cyst is a rare benign congenital dilation of the bile duct, which causes recurring disturbing symptoms without totally resection. Nonetheless, postoperative complications are still a common issue. A step up management for patients with complex complications is required to address the problem. We report a 10-year-old child who suffered complex postoperative complications after choledochal cyst resection at the age of 5, including cholangitis, bilioenteric stenosis and cystolithiasis in remnant intrapancreatic duct cyst. She occasionally endured episodes of epigastric pain, fever and jaundice afterwards. As the symptoms and recurrent rate were worsen over time, the patient was admitted multiple times and various approaches (balloon dilation, percutaneous transhepatic biliary drainage, endoscopic retrograde cholangiopancreatography and laparoscopic surgery) were applied. Afterwards, patient recovered and discharged without any complications. Our case presented sophisticated complications relating to choledochal cyst that were successfully treated by a combination of modern minimal invasive techniques. Despite operated by experienced surgeons, the post-op complications are still a concerned problem due to difficult laparoscopic techniques, injuries of hepatic artery, infection and risk of malignancy. We suggested that minimal-invasive procedures should be considered first with the aim of relieving symptoms, biliary drainage and preparing for the reoperation. [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., (© 2023. Japanese Society of Gastroenterology.)

Conclusions: 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.

Purpose: The purpose of this study is to review our experience in patients who underwent re-operation for delayed biliary complications after choledochal cyst (CDC) excision., Methods: All the patients who underwent re-operation. for delayed biliary complications after CDC excision between August 2007 and July 2020 were included in this retrospective study. The outcomes of these patients were compared with those who underwent primary surgery (CDC excision) at our institution., Results: Of the total 40 patients with delayed biliary complications, 25 (62.5%) were female. Thirty-seven (92.5%) patients had a history of cholangitis. The median interval between CDC excision and the reoperation was 70 (4-216) months. The median duration of symptoms before reoperation was 12 (2.5-84) months. Re-do hepaticojejunostomy and direct hepaticojejunostomy were performed in 34 and in 6 patients respectively. Median operative time and blood losses were 219 min and 150 ml respectively. The median postoperative stay was 9 days. Postoperative complications developed in 10 (25%) patients. There was no operative mortality. Over a median follow-up of 71 months, a satisfactory outcome was achieved in 86% of patients. Restricture and intrahepatic stones developed in three and two patients respectively. Incidence of type IV cyst, cholangitis before operation, and operative blood loss were significantly more in the re-operative group. Clinical outcomes like the incidence of recurrent cholangitis, re-stricture, and postoperative hospital stay were comparable between the two groups., Conclusion: Surgery affords excellent results for majority of the patients with delayed biliary complications after CDC excision. Type IV cysts are more commonly associated with the development of delayed biliary complications., Competing Interests: Declaration of competing interest None declared., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Purpose: Complicated choledochal cysts (CDC) have a variable presentation, and their management differs from an uncomplicated CDC. They are infrequently reported. We present our 15 years of experience in the management of complicated CDC., Methodology: We reviewed the data of patients with CDCs managed at a tertiary level center from 2005 to 2020 from a prospectively maintained database., Results: Of 215 patients with CDC, 123 patients presented with complicated CDC. The median age of complicated CDC was 31 years with a female preponderance (62.6%). The most common type of CDC associated with complications was type I (69.1%), followed by type IVA (29.3%). The Complicated CDC was presented as cholangitis with or without cystolithiasis (n = 45), cystolithiasis and hepatolithiasis(n = 44), malignancy(n = 10), complications associated with incomplete cyst excision (n = 10), acute pancreatitis (n = 8), chronic pancreatitis(n = 8), portal hypertension (n = 6), spontaneous rupture (n = 4), gastric outlet obstruction (n = 1). These patients were managed as a one-stage approach (52.03%) and a two-stage approach (47.96%). On univariate and multivariate analysis, increasing age, prolonged duration of symptoms, and presence of abnormal pancreaticobiliary ductal junction (APBDJ) were significantly associated with complicated CDC., Conclusion: The management of complicated CDC varied depending on the associated pathology, many of them required a staged approach. Increasing age, prolonged duration of symptoms, and presence of APBDJ were significantly associated with complicated CDC., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Hepatobiliary scintigraphy is a minimally invasive imaging method that evaluates bile flow dynamics. At our hospital, it has been performed for postoperative evaluation of patients with choledochal cysts (CC). This study evaluated the usefulness of biliary scintigraphy for predicting late complications in patients with CCs., Methods: The study included pediatric patients with CC who underwent surgery at Chiba University Hospital from 1978 to 2020, followed by postoperative biliary scintigraphy and subsequent radiologic evaluation. The patients were divided into two groups according to the presence or absence of "biliary cholestasis" on biliary scintigraphy., Results: The study included 108 patients, with a median age at surgery of 2 years and 11 months. The median follow-up period was 5203 days, with 11 hepatolithiasis cases and 8 cholangitis cases. No patients had cholangiocarcinoma. Twelve patients were considered to have "cholestasis" following biliary scintigraphy evaluation. There was no significant difference in the occurrence of hepatolithiasis between the cholestasis and non-cholestasis groups (p = 0.47), but cholangitis was significantly more common in the cholestasis group (p = 0.016)., Conclusion: Biliary cholestasis on postoperative hepatobiliary scintigraphy was a risk factor for cholangitis in patients with CCs. These particular patients should be monitored carefully., (© 2022. The Author(s).)

Sixty year old female with hypertension and crampy abdominal pain episodes. Admitted to hospital (September-2020) by obstructive jaundice. MRCP: biliary dilation due to Todani Ic (fusiform) choledocal cyst (CC), distal sludge. ERCP: normal mucosa prominent papilla; biliary dilation compatible with CC; choledocholithiasis; 8-mm CHD filling defect. Sphincterotomy, removal of stones/sludge, brush-cytology of the filling defect (pathology: atypias). US: dilation resolution (CBD: 6.5 mm).

Background: Recently, single definitive hepaticojejunostomy has been proposed to replace two-stage procedures, including initial urgent biliary drainage and final biliary reconstruction, with a reduced hospitalization time and corresponding overall hospitalization cost. We aimed to investigate the safety and efficacy of the initial definitive operation compared with the two-stage approach., Methods: The medical records of patients with perforated CDCs managed between 2010 and 2017 were retrospectively reviewed. The qualified samples were divided according to a single definitive operation or two-stage approach. We conducted a comparison of the clinical characteristics, including surgical parameters, length of stay, and short- and intermediate-term complications., Results: A total of 117 patients with perforated CDCs were reviewed, with 48 cases of single-stage management and 69 cases of two-stage management. No differences in the baseline characteristics between the two groups were found, such as age, sex distribution, ultrasound presentation, or laboratory findings. The initial definitive operation was associated with a lower total duration of drainage, including abdominal drainage and biliary drainage (p < 0.001), and rate of unplanned readmission (OR, 0.056; 95% CI 0.007-0.435; p < 0.001) than those who underwent two-stage management. Furthermore, the initial definitive operation significantly reduced the postoperative hospital stay (p < 0.001), and the overall hospitalization cost was accordingly reduced compared with two-stage management (p < 0.001)., Conclusions: The initial single definitive operation was safe and effective for most of the patients with perforated CDCs when expertise was available. Therefore, a single definitive operation should be considered the treatment of choice for perforated CDCs., (© 2020. Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: To compare the clinical and radiologic findings between perforated and non-perforated choledochal cysts in children., Materials and Methods: Fourteen patients (mean age ± standard deviation, 1.7 ± 1.2 years) with perforated choledochal cysts (perforated group) and 204 patients (3.6 ± 3.8 years) with non-perforated choledochal cysts (non-perforated group) were included between 2000 and 2019. All patients underwent choledochal cyst excision after ultrasound, CT, or MR cholangiopancreatography. Relevant data including demographics, clinical symptoms, laboratory findings, imaging findings, and outcomes were analyzed. Statistical differences were compared using the Mann-Whitney U test and Fisher's exact test., Results: Choledochal cyst perforation occurred only in children under the age of 4 years. Acute symptoms, including fever ( p < 0.001), were more common in the perforated group than in the non-perforated group. High levels of white blood cells ( p = 0.004), C-reactive protein ( p < 0.001), and serum amylase ( p = 0.002), and low levels of albumin ( p < 0.001) were significantly associated with the perforated group. All 14 patients with perforated choledochal cysts had ascites, whereas only 16% (33/204) of patients in the non-perforated group had ascites ( p < 0.001). In the subgroup of patients who had ascites, a large amount of ascites ( p = 0.001), increase in the amount of ascites in a short time ( p < 0.001), complex ascites ( p < 0.001), and perihepatic pseudocysts ( p < 0.001) were more common in the perforated group than in the non-perforated group., Conclusion: Children with perforated choledochal cysts have characteristic clinical and radiologic findings compared to those with non-perforated choledochal cysts. In young children with choledochal cysts, perforation should be differentiated in cases with acute symptoms, laboratory abnormalities, and characteristic ascites findings., Competing Interests: So-Young Yoo who is on the editorial board of the Korean Journal of Radiology was not involved in the editorial evaluation or decision to publish this article. All remaining authors have declared no conflicts of interest., (Copyright © 2022 The Korean Society of Radiology.)

Purposes: Congenital biliary dilatation (CBD), defined as pancreaticobiliary maljunction (PBM) with biliary dilatation, is a high risk factor for biliary tract cancer (BTC). KRAS and p53 mutations reportedly affect this process, but the mechanisms are unclear, as is the likelihood of BTC later in life in children with CBD. We investigated potential carcinogenetic pathways in children with CBD compared with adults., Methods: The subjects of this study were nine children with CBD and 13 adults with PBM (10 dilated, 3 non-dilated) without BTC who underwent extrahepatic bile duct resections, as well as four control patients who underwent pancreaticoduodenectomy for non-biliary cancer. We evaluated expressions of Ki-67, KRAS, p53, histone deacetylase (HDAC) and activation-induced cytidine deaminase (AID) in the biliary tract epithelium immunohistochemically., Results: The Ki-67 labeling index (LI) and expressions of KRAS, p53, HDAC, and AID in the gallbladder epithelium were significantly higher or tended to be higher in both the children with CBD and the adults with PBM than in the controls., Conclusions: BTC may develop later in children with CBD and in adults with PBM, via HDAC and AID expression and through epigenetic and genetic regulation., (© 2021. Springer Nature Singapore Pte Ltd.)

Background: Triple gallbladder is a rare congenital anomaly of the biliary tract that can be associated with heterotopic tissue. Gallbladder triplication results from the failure of rudimentary bile ducts to regress during embryological development, and can be difficult to distinguish from Todani type II choledochal cysts and biliary duplication cysts., Case Presentation: A 2-year-old patient presented to our institution with intermittent abdominal pain for 1 year. She had elevated transaminases with imaging concerning for a choledochal cyst. After assessment with magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography, she was diagnosed with a gallbladder multiplication and a common bile duct stricture. She underwent laparoscopic cholecystectomy, which confirmed the diagnosis of triple gallbladder. One of the three gallbladders demonstrated heterotopic gastric mucosa on final pathology, including at the cystic duct margin. Follow up testing with a technetium 99 m scan demonstrated a subtle focus of increased activity in the right upper abdomen at the expected location of the common bile duct, concerning for the presence of residual gastric mucosa. The patient remains well without abdominal pain., Conclusions: We describe the first case of heterotopic gastric mucosa in a triple gallbladder in a young patient presenting with chronic abdominal pain. We also demonstrate the safety and feasibility of laparoscopic cholecystectomy in young children with triple gallbladder. Finally, we propose an interdisciplinary approach to the management of common bile duct strictures in the setting of ectopic acid secretion, involving a combination of medical management, endoscopic intervention, and possible salvage laparoscopic Roux-en-Y hepaticojejunostomy., (© 2022. The Author(s).)

A young adult male presented with biliary colic and intermittent jaundice for 1 year. Abdomen findings were unremarkable. Routine investigations revealed a raised total bilirubin. On abdominal ultrasonography, common bile duct (CBD) dilatation with multiple stones was noted. On further imaging with magnetic resonance cholangiopancreatography, type I choledochal cyst (CDC) was suspected. A laparoscopic approach was planned. Intraoperatively, dilatation of cystic duct was noted which constitute type VI CDC. Partial malrotation of the gut and accessory right hepatic artery were also noted as incidental finding. Laparoscopic cholecystectomy with CBD exploration and removal of stones, biliary stent placement, cystic duct cyst excision and primary repair of CBD was done. Postoperatively, the patient improved symptomatically with a fall in bilirubin to normal range. We are describing the laparoscopic management of a rare case of type IV CDC which was diagnosed intraoperatively., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Competing Interests: Competing interests: None declared.

Choledochal cysts, congenital segmental dilations of the common bile duct, have been reported in few cats, and histologic characterization is lacking. A 20-mo-old spayed female domestic shorthair cat was presented because of vomiting and weight loss. There was progressive elevation of liver enzyme activity (ALT > ALP, GGT) and hyperbilirubinemia. Diagnostic imaging identified focal cystic dilation of the common bile duct, dilation and tortuosity of adjacent hepatic ducts, and a prominent duodenal papilla. A choledochal cyst was suspected, and the animal was euthanized. On postmortem examination, there was a 2-cm, firm, thickened, cystic dilation of the common bile duct, patent with adjacent ducts. Histologically, the cyst wall was expanded by fibroblasts, collagen, and lymphoplasmacytic inflammation. Adjacent bile ducts were markedly dilated and tortuous, with lymphoplasmacytic inflammation and papillary mucosal hyperplasia that extended to the major duodenal papilla. There was chronic neutrophilic cholangitis, suggesting bacterial infection and/or disturbed bile drainage, extrahepatic obstruction, and lymphoplasmacytic pancreatitis with ductular metaplasia. Prominent lymphoid follicles within biliary ducts and duodenum suggested chronic antigenic stimulation. Choledochal cysts can be associated with chronic neutrophilic cholangitis, extrahepatic obstruction, choledochitis, duodenal papillitis, and pancreatitis, and should be a differential for increased hepatic enzymes and hyperbilirubinemia in young cats.

Background: Gallstones are uncommon in infants and children and Asian children are thought to have very low risk. Diagnoses have increased in recent years with the widespread use of ultrasonography (USG). This study aimed to review our experience with risk factors, complications, and treatment of pediatric gallstones in low-incidence populations., Methods: We retrospectively reviewed patients younger than 18 years old diagnosed with gallstones using USG between November 2006 and December 2012 in a tertiary referral hospital in Taiwan. Demographic information including age and sex, follow-up period, USG findings, predisposing factors, complications, treatment approaches and outcomes were recorded., Results: Ninety-eight children with gallstones diagnosed with USG were enrolled and reviewed in our study. Females comprised 55% of patients, with no specific gender tendency. No risk factor could be identified in 30.8% of patients. The most common risk factors were cephalosporin (CS) use, presence of a choledochal cyst (CC), and spherocytosis. CS use was not associated with a higher dissolution rate. The presence of type IVa CC implied a high rate of gallstone recurrence after Roux-en-Y hepaticojejunostomy. Complications were seen in 22.4% of patients, but only two needed emergency stone removal. Expectant management was performed in 61% of patients and 62.5% of them achieved spontaneous resolution; the stone dissolution rate was not lower than in the group treated with ursodeoxycholic acid (UDCA). Stone size was significantly decreased after UDCA use if resolution did not occur., Conclusions: Pediatric gallstones showed high resolution rate, and the clinical course was largely benign. CS use was the most common risk factor and did not predict a higher dissolution rate. Type IVa CC was also an important risk factor associated with a high recurrence rate. Conservative treatment and oral UDCA may be reasonable strategies in most patients, unless complications are present., Competing Interests: Declaration of competing interest The authors declare that they have no conflict of interest., (Copyright © 2020. Published by Elsevier B.V.)

Background: Choledochal cysts are divided into 5 types. Physicians believe that Caroli disease (which refers to type V biliary cysts) is a special type of biliary cyst caused by a mutation in the PKHD1 gene and is associated with autosomal recessive polycystic kidney disease (ARPKD). There is currently no clear association between other types of choledochal cysts and polycystic kidney disease., Case Presentation: We report a 65-year-old male patient with jaundice, decreased appetite, and itchy skin. His biochemistry test results indicated obstructive jaundice disease. Cross-sectional imaging showed a type IVA choledochal cyst accompanied by autosomal dominant polycystic kidney disease (ADPKD). Due to economic difficulties, the patient achieved percutaneous transhepatic cholangial drainage (PTCD) instead of surgery., Conclusion: To our knowledge, this is the second case report of the coexistence of type IVA choledochal cysts and ADPKD. We conclude that it is vital to be aware that the above condition is a possibility. This case report will aid earlier diagnosis and management and possibly prevent further damage to liver and kidney function.

Choledochal cysts are dilations of the biliary tree that cause a variety of clinical symptoms and can lead to several types of complications. Choledochal cysts are most commonly diagnosed in childhood and frequently present with abdominal pain, jaundice and, in infants, an abdominal mass. Although the most concerning complication is malignant transformation of the cyst epithelium, other complications such as stone formation, acute pancreatitis and stricture can also occur and lead to patient morbidity. Treatment is aimed at not only relieving patient symptoms, but also decreasing a long-term cancer risk. We present a case of a child presenting with abdominal pain and vomiting secondary to a type IVa choledochal cyst complicated by acute pancreatitis, a common bile duct stricture and cystolithiasis., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

We report the case of a 62-year-old woman who was admitted for epigastralgia and oral intolerance for 15 days, associating cholestasis in blood tests. A magnetic resonance cholangiography (MRC) revealed the presence of a lobulation continuous with the cystic duct that was consistent with a Todani VI bile duct cyst. The biliary cyst Todani VI is a rare entity within bile duct malformations. The first well-documented case was reported back in 1983, and its inclusion as sixth type in the classification was suggested in 1991. It is an uncommon cause of abdominal pain that we must keep in mind, and the importance of early diagnosis and treatment lies in the condition's oncogenic potential.

An isolated dilatation of the cystic duct (type VI choledochal cyst (CDC)) is extremely rare with only 21 cases reported in the world literature until now. There is only one case of in situ gall bladder cancer (GBC) reported in association with type VI CDC in the literature. Here we are reporting a case of type VI CDC with papillary GBC., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

A 3 year old girl presented initially with acute severe hemorrhagic pancreatitis diagnosed by clinical features including a positive Cullen's sign and raised serum amylase. A contrast enhanced computed tomography (CECT) scan corroborated the same and also reported a type I choledochal cyst. Magnetic resonance cholangiopancreatography (MRCP) 2 months later reported a type II choledochal cyst and a persistent pseudo pancreatic cyst. Intraoperative features and close perusal of the CECT and MRCP films showed the presence of mixed type I and II choledochal cyst. The case is reported for the presence of Cullen's sign, CECT and MRCP features of a mixed type I and II choledochal cyst and successful surgical outcome. It appears to be only the fifth case of mixed type I and II choledochal cyst in world literature in the pediatric age group, following the 4 previous cases in children reported from Japan., (Copyright © 2019 Elsevier Inc. All rights reserved.)

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., (© 2019 The Authors. BJS Open published by John Wiley & Sons Ltd on behalf of BJS Society Ltd.)

This case report describes an extremely rare association between gallbladder agenesis and choledochal cyst (CC). A 9-year-old girl presented with recurrent abdominal pain in the right upper quadrant. Radiological studies revealed a CC type IVa and an agenesis of gallbladder and cystic duct. Due to the possibility of biliary neoplasm, the patient underwent cyst resection and hepaticoduodenostomy. Histopathological findings showed inflamed fibrous tissue covered by biliary epithelium with no evidence of malignancy., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Choledochal cysts (CDCs) and biliary atresia (BA) are rare pediatric hepatobiliary anomalies that require surgical intervention due to increased risk of malignancy and liver failure, respectively. The underlying disease and operative procedures place patients at risk for long-term complications, which may continue to affect them into adulthood. Lack of a transitional care model in the health-care system potentiates the challenges they will face following aging out of their pediatric providers' care. We sought to elucidate the long-term complications and challenges patients with CDCs and BA face, review the current literature regarding transitioning care, and propose guidelines aiding adult providers in continued care and surveillance of these patients. A literature review was performed to assess short-term and long-term complications after surgery and the current standards for transitioning care in patients with a history of CDCs and BA. While transitional programs exist for patients with other gastrointestinal diseases, there are few that focus on CDCs or BA. Generally, authors encourage medical record transmission from pediatric to adult providers, ensuring accuracy of information and compliance with treatment plans. Patients with CDCs are at risk for developing biliary malignancies, cholangitis, and anastomotic strictures after resection. Patients with BA develop progressive liver failure, necessitating transplantation. There are no consensus guidelines regarding timing of follow up for these patients. Based on the best available evidence, we propose a schema for long-term surveillance., (© 2018 Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.)

Previous research has confirmed that patients with choledochal cyst have an elevated risk of cholangiocarcinoma and gallbladder carcinoma. Current data suggest a risk of malignancy of 6 to 30% in adults with choledochal cyst. Malignancy has also occasionally been identified in children and adolescents. Multiple factors, including the age of the patient, cyst type, histological findings, and localization, have an impact on the prognosis. Information on long-term outcomes after cyst excision is limited. However, recent data suggest a lifelong elevated risk of up to 4% of cancer development following operation. This paper presents a review of the literature on cancer in patients with choledochal cyst before and after excision. A postoperative follow-up concept that consists of annual controls of CA19-9 and abdominal ultrasound is introduced., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Biliary tract shows a large number of anatomic variations and duplication of the bile duct is an extremely rare anomaly. It has been reported to be associated with other congenital conditions like Abnormal Pancreato Biliary Junction (APBJ), biliary atresia and choledochal cyst and may lead to complications like cholangitis, choledocholithiasis and malignancy. The clinical presentation may be with one of the above complications and the condition usually reveals itself only on imaging investigations, as a surprise to the radiologist and the surgeon. Its detection is important prior to any biliary tract surgery to prevent inadvertent bile duct injury. The authors report a case of a rare subtype of extrahepatic bile duct duplication with coexisting choledochal cyst.

Choledochal cysts are rare congenital anomalies of the biliary tree, which are more prevalent in female Asian population. Malignant transformation occurs in 10% to 30% of adults with choledochal cysts. A 39-year-old man presented with abdominal pain for 2 months, with irregular thickening of bile duct wall on ultrasound and enhanced CT. Staging with F-FDG PET/CT demonstrated lymph node and liver metastasis. F-FDG PET/CT may become a valuable imaging procedure in diagnosing and staging of carcinoma arising from choledochal cysts.

Background: In biliary atresia, the disease process of obliterative cholangiopathy may begin in the perinatal period; however, no chronological evidence exists on how the cholangiopathy progresses to biliary obliteration. This is the first acquired case with the final diagnosis of type III cystic biliary atresia with an extrahepatic biliary cyst which showed the progression of obliterative cholangiopathy in chronological order after birth., Case Presentation: An 81-day-old girl presented with acute abdominal distress due to bilious peritonitis caused by biliary cyst perforation, for which she underwent emergency biliary drainage. Postoperative images showed a dilated common bile duct and hepatic ducts bilaterally, with flow of the contrast medium to the duodenum through the dilated common bile duct. Biochemistry of the bile collected during and after the operation revealed elevated levels of pancreatic enzymes in the bile from the gallbladder. The patient was diagnosed as having a congenital choledochal cyst and underwent laparotomy at 120 days of age which revealed that she had pancreaticobiliary maljunction. The biliary cyst was resected at the narrow portion just above the junction with the main pancreatic duct. During dissection up to the hepatic hilum, we found that the hilar hepatic ducts were bilaterally replaced by fibrous tissue and were obstructed, leading to a diagnosis of type III a1, μ biliary atresia. The fibrous tissue was excised, and hepatic portoenterostomy was performed according to the Kasai procedure. The patient's postoperative course was uneventful and the jaundice resolved within 1 month. She has had normal liver function tests with no episode of cholangitis for 3 years after discharge., Conclusions: We demonstrated the process of acquired type III biliary atresia in a patient with cystic biliary atresia and biliary cyst perforation. To the best of our knowledge, this is the first case of acquired cystic biliary atresia showing chronological progression of the course of obliterative cholangiopathy, providing a better understanding of the development of type III biliary atresia as an acquired disease.

Choledochal cyst is a rare congenital malformation which is usually found in young children and adolescents but can rarely present in the adult age group. It can present with several complications like cholangitis, pancreatitis, biliary cirrhosis, portal hypertension, liver abscess, malignancy and rarely spontaneous rupture (in 2% of cases) causing biliary peritonitis. A case of spontaneous rupture of choledochal cyst during second trimester of pregnancy is reported where the patient was managed by minimal surgery, peritoneal washout, placement of T-tube and planned for definitive treatment in the postpartum period. The pregnancy continued till 35 weeks with favourable fetomaternal outcome., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Aim: Cystic biliary atresia (CBA) can be easily misdiagnosed as choledochal cyst (CC). Some patients have already progressed to severe liver fibrosis and missed the optimal surgical time, when the differential diagnosis is made. We aim to determine the differentiation between CBA and CC, and to validate the value of aspartate aminotransferase-to-platelet ratio index (APRI) in the assessment of liver fibrosis and prediction of post-operative outcome for infants with biliary cystic malformations (BCMs)., Methods: Clinical data of children (categorised into CBA and CC groups) with BCMs were analysed retrospectively. Biochemical indicators together with B-ultrasound examinations and the degree of liver fibrosis were analysed, and those with statistical difference between the two groups were selected for diagnostic receiver operating characteristic curve analysis., Results: The parameter that showed the highest accuracy with a significant diagnostic performance for differentiating CBA from CC was cyst size. Liver assessment at operation was categorised into mild fibrosis and moderate-to-severe fibrosis. The APRI values were much lower in the mild fibrosis groups than in the moderate-to-severe fibrosis group (0.4 ± 0.2 vs. 1.4 ± 0.8, P < 0.001). A cut-off value of 0.96 (area under the curve 0.92, P < 0.001) showed a sensitivity of 81.3% and a specificity of 100% for moderate-to-severe fibrosis. Lower APRI value was correlated with short-term post-operative bilirubin clearance., Conclusion: There is still certain difficulty in the early identification of CBA and CC clinically. Liver fibrosis could occur as early as infantile period in both CBA and CC. In infants with BCMs, APRI can be used as a non-invasive method to detect liver fibrosis in early stages., (© 2017 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Background: Choledochal malformations comprise various congenital cystic dilatations of the extrahepatic and/or intrahepatic biliary tree. Choledochal malformation is generally considered a premalignant condition, but reliable data on the risk of malignancy and optimal surgical treatment are lacking. The objective of this systematic review was to assess the prevalence of malignancy in patients with choledochal malformation and to differentiate between subtypes. In addition, the risk of malignancy following cystic drainage versus complete cyst excision was assessed., Methods: A systematic review of PubMed and Embase databases was performed in accordance with the PRISMA statement. A meta-analysis of the risk of malignancy following cystic drainage versus complete cyst excision was undertaken in line with MOOSE guidelines. Prevalence of malignancy was defined as the rate of biliary cancer before resection, and malignant transformation as new-onset biliary cancer after surgery., Results: Eighteen observational studies were included, reporting a total of 2904 patients with a median age of 36 years. Of these, 312 in total developed a malignancy (10·7 per cent); the prevalence of malignancy was 7·3 per cent and the rate of malignant transformation was 3·4 per cent. Patients with types I and IV choledochal malformation had an increased risk of malignancy (P = 0·016). Patients who underwent cystic drainage had an increased risk of developing biliary malignancy compared with those who had complete cyst excision, with an odds ratio of 3·97 (95 per cent c.i. 2·40 to 6·55)., Conclusion: The risk of developing malignancy among patients with choledochal malformation was almost 11 per cent. The malignancy risk following cystic drainage surgery was four times higher than that after complete cyst excision. Complete surgical resection is recommended in patients with choledochal malformation., (© 2018 The Authors. BJS published by John Wiley & Sons Ltd on behalf of BJS Society Ltd.)

Rationale: Choledochal cysts are a congenital disorder of the common bile duct that can cause progressive biliary obstruction and biliary cirrhosis. They were classified by Todani into five types. Of these, type VI choledochal cysts are rarely reported in the literature., Patient Concerns: A 22-month-old girl presented with intermittent epigastralgia for approximately 10 days and fever for three days. Fasting and total parenteral nutrition were administered after admission. However, sudden onset of severe epigastric pain occurred. An abdominal sonogram showed turbid ascites and peritonitis was impressed., Diagnoses: An emergent exploratory laparotomy was performed, and perforation of the posterior wall of types I and VI choledochal cysts was observed., Interventions: Intraoperative cholangiography revealed concomitant types I and VI choledochal cysts with stricture of the distal common bile duct. Definite surgery for resection of the choledochal cysts and gallbladder was performed with Roux-en-Y choledochojejunostomy., Outcomes: The patient had no evidence of ascending cholangitis at three years after the operation., Lessons: Type VI choledochal cysts are rarely reported in the literature. To our knowledge, this is the first reported pediatric case of concomitant types I and VI choledochal cysts complicated with acute pancreatitis and spontaneous perforation.

Introduction: The aim of this study was to compare laparoscopic hepaticojejunostomy (LHJ) and open hepaticojejunostomy (OHJ) for choledochal cyst associated with hilar hepatic duct stenosis (HHDS)., Methods: Data collection was prospective for LHJ cases from 2009 and retrospective for OHJ cases from 2003 to 2008. Data were compared with respect to HHDS. HHDS was incised longitudinally as required during hilar hepatic ductoplasty., Results: Fifty-eight subjects were studied (LHJ: n = 27, 4 boys, 23 girls; OHJ: n = 31; 6 boys, 25 girls). HHDS was present in 10 LHJ cases and 10 OHJ cases. Todani classification of choledochal cyst in LHJ patients was type 1A (n = 16) and type 4A (n = 11), and in OHJ patients, it was type 1A (n = 19) and type 4A (n = 12). There were significant differences between LHJ and OHJ with regard to mean operative time (386 vs 341 min), mean blood loss (5.9 vs 18.4 mL), recommencement of enteral feeding (3.9 vs 6.4 days), and hospital stay (11.7 vs 15.5 days) (all P < 0.05). Hepatic ductoplasty was performed in 23 LHJ patients and in 21 OHJ patients. There were no intraoperative complications and no conversions to OHJ. There were no significant differences between LHJ and OHJ, regardless of the presence of HHDS, for postoperative complications, which included minor bile leakage, anastomotic stricture, and intestinal obstruction., Conclusion: LHJ is as effective as OHJ for the treatment of choledochal cysts irrespective of the presence of HHDS and the need for hepatic ductoplasty., (© 2017 Japan Society for Endoscopic Surgery, Asia Endosurgery Task Force and John Wiley & Sons Australia, Ltd.)

An 18-year-old man presented with spontaneous severe epigastric pain, progressing and radiating to back since 3 days. It was associated with epigastric tenderness, bilious vomiting and jaundice. He had been intermittently experiencing these symptoms for the last 1 year. No known comorbid. Ultrasound showed a poorly visualised heterogeneous focus at porta hepatis; considering poor visualisation, this might represent an enlarged calcified lymph node or cystic duct calculus causing extrinsic compression or a large sludge ball within the common bile duct (CBD), leading to dilatation of common hepatic duct and intrahepatic biliary system. Subsequent magnetic resonance cholangiopancreatography revealed a focal saccular dilatation of middle part of CBD, a type I-B choledochal cyst, large heterogeneous focus seen within it representing choledochocystolithiasis. Later, CT was performed for further characterisation of surrounding anatomy and pathology, which confused the appearance of choledochocystolithiasis for Mirizzi syndrome. Later, surgery and histopathology confirmed type I-B choledochocystolithiasis and chronic cholecystitis., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

The choledochocele is a cystic dilatation of the intraduodenal portion of the bile duct and corresponds to the type III biliary cysts in the Todani ´s classification. Eventhough the majority of patients remain asymptomatic they can be an atypical cause of abdominal pain or relapsing acute pancreatitis events. The risk of malignancy is lower than other choledochal cyst (<2,5%). The treatment is based on surgical or new endoscopic techniques of resection. In some of the cases an endoscopic sphincterotomy is the first approach.