Clinical implications of pediatric biliary intraepithelial neoplasia diagnosed from a choledochal cyst specimen.

BACKGROUND: Biliary intraepithelial neoplasia (BilIN), a noninvasive precursor of cholangiocarcinoma, can manifest malignant transformation. Since cholangiocarcinoma (CCA) may progress due to chronic inflammation in the bile ducts and gallbladder, choledochal cysts are considered a precursor to CCA. However, BilIN has rarely been reported in children, to date. METHODS: We reviewed medical records of patients (< 18 years of age, n = 329) who underwent choledochal cyst excision at Asan Medical Center from 2008 to 2022. BilIN was diagnosed in 15 patients. Subsequent analyses were performed of the demographics, surgical procedures, clinical course, and outcomes in these patients. Subgroup analysis and multivariate logistic regression test were performed to identify factors influencing BilIN occurrence. RESULTS: The mean age of the patients included in our study was 40.1 ± 47.6 months. In 15 patients, BilIN of various grades was diagnosed. Todani type I was prevalent in 80% of the patients. The median age at surgery was 17 months. During a mean follow-up of 63.3 ± 94.0 months, no adverse events such as stone formation in the remnant intrapancreatic common bile duct and intrahepatic duct or cholangiocarcinoma were observed, indicating a favorable outcome until now. CONCLUSIONS: The potential progression of choledochal cysts to BilIN in children was demonstrated. These results could underscore the importance of early and comprehensive excision of choledochal cysts, including resection margins for associated lesions and more thorough postoperative surveillance in patients with or at risk of BilIN.

An observational study on the prevalence of choledochal cyst with pancreatitis: Geographical implications and management.

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.

Choledochal anomalies in adults: A 20-year single-center retrospective cohort experience in the Middle East.

BACKGROUND AND STUDY AIMS: Choledochal cysts are rare congenital cystic dilatations of the bile ducts that occur in fewer than 1% of individuals. The disease is common in East Asia, and most of the literature concerns those populations, but some data about Western populations have been published recently. Long-term reports about the disease in Middle Eastern populations, however, are currently lacking. We report a single-center 20-year experience in diagnosing and managing choledochal anomalies. PATIENTS AND METHODS: Participants were adult patients in whom choledochal cysts were diagnosed over a 20-year (2000-2019) period at a single tertiary academic care center. Clinical data, including radiologic imaging findings, were retrieved from the patients' medical records. To describe the baseline characteristics of the population, we calculated descriptive statistics. RESULTS: Choledochal anomalies were diagnosed in 19 adult patients, whose median age was 30 years (interquartile range [IQR], 23-67 years). Of the choledochal cysts 13 (68.4%) were classified as Todani type I, 4 (21.1%) as Todani type IV, and 3 (15.8%) as Todani type V (Caroli's disease). No patient had underlying chronic liver disease, and liver synthetic function was preserved in all. Eighteen patients (94.7%) underwent surgery: cyst excision with Roux-en-Y hepaticojejunostomy in 17 and liver transplantation in 1. All 18 survived surgery, and the median postoperative hospital stay was 11 days (IQR, 5-34 days). All 18 were alive 90 days after surgery, and the median follow-up period was 40 months (IQR, 12-140 months). Seven patients (36.8%) developed postoperative surgical complications; 2 patients required rehospitalization, and 1 required reoperation. CONCLUSION: This description of adults with choledochal cysts is the latest long-term report about this disease in the Middle East. In our 20-year experience, the disease characteristics in our patients were moderately consistent with those described previously.

Risk factors and impact on bile leakage in patients with choledochal cysts: a retrospective case-control analysis.

Although bile leakage is a major postoperative complication after hepatobiliary surgery, the associated risk factors for pediatric patients remain poorly defined. Here, we intend to identify the perioperative risk factors for bile leakage in pediatric patients with choledochal cysts following Roux-en-Y hepaticojejunostomy. A multicenter case-control study investigating the risk factors for bile leakage was conducted among 1179 eligible pediatric patients with choledochal cysts following Roux-en-Y hepaticojejunostomy between January 2009 and December 2019. There were 267 cases with bile leakage, and approximately four control patients were identified for each case. Multivariable logistic regression was performed to identify the risk factors, including perioperative variables. According to univariable analysis, bile leakage was associated with severe cholangitis (p = 0.012), low albumin levels (p = 0.010), anemia (p = 0.002) and laparoscopic surgery (p = 0.004). Multivariable analysis showed that a low level of preoperative albumin (ALB) (odds ratio [OR] = 1.11; 95% CI 1.02-1.19; p = 0.016), worse symptoms (severe cholangitis) (OR = 1.16; 95% CI 1.01-1.26; p < 0.001), and a previous hepatobiliary procedure (OR = 1.32; 95% CI 1.09-1.63; p = 0.036) were independent factors that were associated with bile leakage. This study identified potential risk factors for bile leakage in patients following Roux-en-Y hepaticojejunostomy that should be targeted for interventions to reduce the occurrence of the condition.

Long-term Morbidity of Choledochal Malformations in Children.

OBJECTIVE: The aim of the study was to assess long-term morbidity in children operated for choledochal malformation (CM) by relating clinical complications to liver histopathology, follow-up imaging, liver stiffness, and biochemistry. METHODS: A single-center retrospective follow-up study including all CM patients (n = 55, 71% girls) treated during 1976 to 2018 was performed. Mann-Whitney U test and Spearman rank correlation were used for statistical analyses. RESULTS: During median follow-up of 5.8 (interquartile range, 2.5-12) years, 1 patient was lost to follow-up whereas all survived. Intraoperative liver biopsies showed fibrosis in 32%, and patients with Metavir stage ≥2 were younger at surgery (0.36 [0.11-1.9] vs 3.8 [0.72-10.5] years, P = 0.024) than those without fibrosis. Overall, 21% had long-term complications including cholangitis in 9 (>2 episodes in 5) patients, anastomotic stricture in 2 referred patients and adhesive volvulus or hepatocellular carcinoma in 1 each. Anastomotic strictures were successfully managed nonoperatively and hepatocellular carcinoma with thermoablation. In postoperative magnetic resonance cholangiography (MRCP) performed 6.4 (3.6-16) years after hepaticojejunostomy, diameters of both main intrahepatic ducts had decreased significantly to 3.0 (2.5-3.5) mm (P = 0.0001) but a distal cyst stump was remaining in 30% with a length of 6.0 (4.0-20) mm that associated with operation age (r = 0.71, P = 0.015) and fusiform CM type. Follow-up ultrasound revealed mild dilation of intrahepatic bile ducts in 6.3% and mildly to moderately elevated liver biochemistry in 23%, and liver stiffness (>7 kPa) in 22%. CONCLUSIONS: Whilst cholangitis was the most common postoperative problem, individual patients experienced other more significant complications and one quarter of patients showed evidence of underlying liver dysfunction.

Guía de práctica clínica en dilatación congénita de las vías biliares en la edad pediátrica / Clinical practice guidelines in congenital dilation of biliary tract in pediatric ages

Las dilataciones congénitas de los conductos biliares, son poco frecuentes y se pueden descubrir en cualquier etapa de la vida, incluido el período prenatal. Es una afección que puede ser asintomáticas, ocasionar síntomas escasos o producir graves complicaciones, razones por las cuales, la actitud actual del especialista se enfoca hacia el diagnóstico temprano y el tratamiento correcto de las diferentes variantes de dilatación congénita de la vía biliar antes que aparezcan las complicaciones mencionadas. El objetivo de publicar esta guía responde a la necesidad de contar con herramientas actualizadas en el manejo de estas afecciones que permitan sistematizar la conducta y mejorar la calidad de la asistencia médica, la docencia y las investigaciones y está aprobada por la Sociedad Cubana de Cirugía Pediátrica. Se realizó una extensa revisión de la literatura científica basada en la mejor evidencia publicada mundialmente sobre el tema. Se presentan el concepto de esta afección, su epidemiología, clasificación, etiología, cuadro clínico, formas de presentación, diagnóstico y conducta a seguir con los diferentes tipos, así como las medidas posoperatorias y de seguimiento del paciente. Si todos los procedimientos se realizan de forma correcta y en el momento oportuno, el pronóstico es favorable en la mayoría de los casos(AU)

Congenital dilation in the biliary duct is not common and can be found in any stage of life, including the prenatal period. It is a condition that can be asymptomatic, causing scarse symptoms or producing severe complications; therefore, the current performance of specialists must be focused towards the early diagnosis and the accurate treatment of the different types of congenital dilation of the biliary tract before the above mentioned complications appear. The aim of publishing this guidelines has to do with the need of having updated tools for the management of these conditions which allow to systematize the behaviours and to improve the quality of medical care, teaching and researches, and also because it is approved by the Cuban Society of Pediatric Surgery. It was made an extensive review of the scientific literature based in the best evidences published worldwide on the topic. It is presented the concept of this condition, its epidemiology, classification, etiology, clinical picture, ways of presentation, diagnosis and behaviour to follow with the different types of it, as well as the patient's post-operative and follow-up measures. If all the procedures are made in a proper way and in the opportune moment, the prognosis is favorable in most of the cases(AU)

Choledochal malformations in adults in the Netherlands: Results from a nationwide retrospective cohort study.

BACKGROUND AND AIMS: Patients with a choledochal malformation, formerly described as cysts, are at increased risk of developing a cholangiocarcinoma and resection is recommended. Given the low incidence of choledochal malformation (CM) in Western countries, the incidence in these countries is unclear. Our aim was to assess the incidence of malignancy in CM patients and to assess postoperative outcome. METHODS: In a nationwide, retrospective study, all adult patients who underwent surgery for CM between 1990 and 2016 were included. Patients were identified through the Dutch Pathology Registry and local patient records and were analysed to determine the incidence of malignancy, as well as postoperative mortality and morbidity. RESULTS: A total of 123 patients with a CM were included in the study (Todani Type I, n = 71; Type II, n = 10; Type III, n = 3; Type IV, n = 27; unknown, n = 12). Median age was 40 years (range 18-70) and 81% were female. The majority of patients (99/123) underwent extrahepatic bile duct resection, with additional liver parenchyma resections in eight patients, only exploration in two, and a local cyst resection in eight patients. Postoperative 30-day mortality was 2% (2/123) and limited to patients who underwent liver resection. Severe morbidity occurred in 24%. In 14 of the 123 patients (11%), a malignancy was found in the resected specimen. One patient developed a periampullary malignancy 7 years later. CONCLUSIONS: In a large Western series of CM patients, 11% were found to have a malignancy. This justifies resection in these patients, despite the risk of morbidity (24%) and mortality (2%).

Choledochal cysts: Similarities and differences between Asian and Western countries.

Choledochal cysts (CCs) are rare bile duct dilatations, intra-and/or extrahepatic, and have higher prevalence in the Asian population compared to Western populations. Most of the current literature on CC disease originates from Asia where these entities are most prevalent. They are thought to arise from an anomalous pancreaticobiliary junction, which are congenital anomalies between pancreatic and bile ducts. Some similarities in presentation between Eastern and Western patients exist such as female predominance, however, contemporary studies suggest that Asian patients may be more symptomatic on presentation. Even though CC disease presents with an increased malignant risk reported to be more than 10% after the second decade of life in Asian patients, this risk may be overstated in Western populations. Despite this difference in cancer risk, management guidelines for all patients with CC are based predominantly on observations reported from Asia where it is recommended that all CCs should be excised out of concern for the presence or development of biliary tract cancer.

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.

Pediatric choledochal cysts: diagnosis and current management.

Choledochal cysts are rare congenital disorders first described by Vater and Ezler in 1723. Their exact etiology remains incompletely understood; however, an anomalous pancreaticobiliary union (APBDU) and subsequent reflux of biliary contents into the biliary tree are thought to play a role. Accordingly, APBDU-associated choledochal cyst patients are significantly more likely to have evidence of hepatitis, cholangitis or pancreatitis and pathologically confirmed inflammation. In 1977, Todani and colleagues modified the original Alonso-Lej classification to include five types of CC. Type I and IV are the most common and most likely to be associated with malignancy. The majority of choledochal cysts are diagnosed in childhood. Clinical presentation varies and most often consists of nonspecific abdominal pain. Diagnosis is typically accomplished using multimodality imaging techniques including computed tomography, magnetic resonance imaging, ultrasound and MRCP. The use of diagnostic PTC and ERCP in CC has been largely replaced by MRCP. Appropriate management consists of prompt, complete cyst excision followed by restoration of biliary enteric continuity when necessary. Minimally invasive CC resection in the pediatric population has demonstrated acceptable outcomes. Prognosis is generally excellent; however, malignancy risk remains higher than the general population even after complete surgical excision.

Acute pancreatitis complicating choledochal cysts in children.

AIM: To analyse the characteristics of patients with choledochal cysts presenting with acute pancreatitis. METHODS: Multicenter retrospective review of all paediatric patients (<18 years) with choledochal cysts managed over a 14-year period (2001-2014) at two tertiary paediatric surgical centres. Patient data were analysed for demographics, presentation, radiological classification of cyst type (Todani), operative interventions, complications and long-term follow-up. RESULTS: A total of 49 patients with choledochal cysts were identified with 15 (31%) being Type I fusiform, 18 (37%) Type I cystic and 16 (32%) Type IV-A. Seventeen (35%) patients presented with acute pancreatitis, one having had an ante-natally diagnosed choledochal cyst. Patients presenting with pancreatitis were older when compared to the non-pancreatitis group (5.1 vs. 1.2 years, P = 0.005). Nine out of 16 (53%) patients with Type IV-A cysts presented with pancreatitis compared to five (33%) of Type I fusiform and three (17%) of Type I cystic. There was however no statistically significant association between Todani types and the development of pancreatitis (Type I fusiform, P = 1.0; Type I cystic, P = 0.063; Type IV-A, P = 0.053). The rate of complications was similar in both groups. CONCLUSION: Pancreatitis was a common presentation in children with a choledochal cyst, however, there was no clear statistically significant association with Todani types and pancreatitis.

Cholangiocarcinoma: Current Knowledge and New Developments.

Cholangiocarcinoma (CCA) is the second most common primary malignancy. Although it is more common in Asia, its incidence in Europe and North America has significantly increased in recent decades. The prognosis of CCA is dismal. Surgery is the only potentially curative treatment, but the majority of patients present with advanced stage disease, and recurrence after resection is common. Over the last two decades, our understanding of the molecular biology of this malignancy has increased tremendously, diagnostic techniques have evolved, and novel therapeutic approaches have been established. This review discusses the changing epidemiologic trends and provides an overview of newly identified etiologic risk factors for CCA. Furthermore, the molecular pathogenesis is discussed as well as the influence of etiology and biliary location on the mutational landscape of CCA. This review provides an overview of the diagnostic evaluation of CCA and its staging systems. Finally, new therapeutic options are critically reviewed, and future therapeutic strategies discussed.

Pancreaticobiliary Maljunctions in European Patients with Bile Duct Cysts: Results of the Multicenter Study of the French Surgical Association (AFC).

BACKGROUND: Pancreaticobiliary maljunctions (PBMs) are congenital anomalies of the junction between pancreatic and bile ducts, frequently associated with bile duct cyst (BDC). BDC is congenital biliary tree diseases that are characterized by distinctive dilatation types of the extra- and/or intrahepatic bile ducts. Todani's types I and IVa, in which dilatation involves principally the main bile duct, are the most frequent. PBM induces pancreatic juice reflux into the biliary tract that is supposed to be one of the main factors of biliary cancer degeneration, although the diagnostic criteria of PBM that can be either morphological and/or functional are not well defined especially in Western series. OBJECTIVE: The aim of this study was to assess the relative prevalence of PBM in BDC in a large European multicenter study, to analyze the characteristics of PBM and try to propose diagnostic criteria of PBMs based on morphological and/or functional criteria and define the positive, negative predictive values, sensibility and specificity of either criteria. RESULTS: From 1975 to 2012, 263 patients with BDC were analyzed. Among them, 190 (72.2 %) were considered to present PBM. Types I and IVa had a similar rate of PBM association. According to the "AFC classification," 57.2 % had a C-P type, 34.5 % a P-C type and 8.3 % a complex type ("anse-de-seau"). The median length of the common channel in patients with PBM was 15.8 ± 6.8 mm (range 5-40 mm). The median intrabiliary amylase and lipase levels were 65,249 and 172,104 UI/L, respectively. For the diagnostic of PBM, a common channel length of more than 8 mm and an intrabiliary amylase level superior to 8000 UI/L were associated with a predictive positive value and a specificity of more than 90 %. Synchronous biliary cancer had an incidence of 8.7 % in all patients with BDC and PBM 11.1 % in adults. Compared to type IV, the type I BDC was associated with statistically more cancer patients in the presence of PBM. CONCLUSIONS: Characteristics of PBM associated with BDC in Western population are quite close to reported Eastern series. The results suggest considering both the intrabiliary value of amylase >8000 UI/L and a length of a common channel >8 mm as appropriate values for positive diagnosis of PBM.

Sphincter of Oddi Dysfunction and the Formation of Adult Choledochal Cyst Following Cholecystectomy: A Retrospective Cohort Study.

To determine the causes underlying the formation of adult choledochal cyst.Anomalous pancreaticobiliary junction is the most widely accepted theory regarding the etiology of choledochal cyst. However, choledochal cysts have been found in patients in the absence of this anomaly. Because the number of adult patients with choledochal cyst is increasing, it is important to address this controversy.Bile amylase levels in the cysts of 27 patients (8 males and 19 females) who had undergone cholecystectomy were retrospectively evaluated.The average age of the 27 patients was 45.8 ± 10.1 years and the majority (85.2%) were diagnosed with Todani type I cysts. None of the patients had dilatation of the common bile duct prior to surgery. There were 6 (22.2%) patients with anomalous pancreaticobiliary junction. However, amylase levels did not significantly differ between patients with and without this anomaly (P = 0.251). According to bile amylase levels, pancreatobiliary reflux was present in 21 (77.8%) patients. The mean amylase level significantly differed in patients with pancreatobiliary reflux (23,462 ± 11,510 IU/L) and those without (235 ± 103 IU/L) (P < 0.001). In patients with pancreatobiliary reflux, only 4 patients had anomalous pancreaticobiliary junction. That is, the majority of patients (17/21, 81%) having pancreatobiliary reflux did not have an anomalous junction of the pancreatic and biliary ducts.Since the only explanation for pancreatobiliary reflux in patients with a normal pancreaticobiliary junction is sphincter of Oddi dysfunction, we proposed that the formation of adult choledochal cyst is mainly due to sphincter of Oddi dysfunction.

Controversies in Choledochal Malformations: A Survey among Dutch Pediatric Surgeons.

BACKGROUND: Choledochal malformation (CM) is a rare medical condition of which 80% are diagnosed in pediatric patients. There are several important controversies regarding diagnostic workup, management, and follow-up in these pediatric patients. To assess preferences and practices of Dutch pediatric surgeons regarding the diagnostic procedures, management, and follow-up of children with CM we conducted an electronic survey. METHODS: A questionnaire was sent to all the pediatric surgeons working in the academic centers and the only community hospital with a pediatric surgery service. The questionnaire included, items regarding incidence, diagnostic workup, interval between diagnosis and surgery, surgical techniques, and follow-up. We also assessed whether personal exposure influenced the preferences and practices. RESULTS: Overall 22 out of the 31 (71%) Dutch pediatric surgeons returned the questionnaire. Total 15 out of 22 (68%) encountered CM up to 2 times/y, whereas 7 out of 22 (32%) encountered it more than 2 times/y. Indications for surgery were significantly different between surgeons who encountered CM > 2 time/y versus those who did not: 6/6 (100%) of surgeons encountering CM > 2 times/y considered the presence of an asymptomatic CM an indication for surgery versus 5/14 (36%) of the pediatric surgeons who encountered a CM up to 2 times/y (p = 0.01). Overall 12 out of the 22 (55%) respondents preferred surgery between 6 months and 2 years of age. The amount of exposure did not differ in preferred age at surgery or surgical technique. In the symptomatic child 10/22 (45%) of respondents preferred surgery within 3 months. Overall 7/22 (32%) favored laparoscopic resection. Hepaticojejunostomy with Roux-en-Y reconstruction was the preferred reconstruction for all the respondents. One-third stated that they never performed a parenchyma resection. Follow-up was limited to 10 years in almost half of the respondents. CONCLUSION: Dutch pediatric surgeons demonstrate a wide variety of opinions regarding diagnostic workup, treatment, and follow-up of CM. While most surgeons encounter CM up to 2 times/y, there is an association between exposure and several of the outcome parameters. Some of the answers are not in line with the expert opinion. This demonstrates that there is a need for evidence-based (inter)national guidelines regarding the diagnostic approach, management, and follow-up.

What is the incidence of biliary carcinoma in choledochal cysts, when do they develop, and how should it affect management?

The incidence of cancer in choledochal cysts (CCs) in adults was calculated to determine the timing and need for surgery. In 78 publications (1996-2010), 434 of 5780 reported CCs patients had cancer. Cholangiocarcinoma (70.4 %) and gallbladder cancer (23.5 %) were the most common malignancies. Only nine malignancies were reported before age 18 (0.42 %). In contrast, the incidence of malignancy in adults was 11.4 %. The median age for diagnosis of cancer was 42 years, and the incidence increased with each decade.

The risk of carcinogenesis in congenital choledochal cyst patients: an analysis of 214 cases.

BACKGROUND: Choledochal cysts are most common in Asian populations. In addition, the incidence of biliary tract malignancies from choledochal cysts is increasing, but the risk of carcinogenesis is still unclear. MATERIAL AND METHODS: Clinical data from 214 congenital choledochal cyst cases from 1968 to 2013 were retrospectively analyzed. RESULTS: Todani type I was more common (139, 65.0%) than type IVa (53, 24.8%) or type V (17, 7.9%) in these choledochal cyst patients. Biliary tract malignant tumors occurred in the gallbladder (2, 13.3%), common bile duct (10, 66.7%), and intrahepatic bile duct (3, 20%) in 15 patients (7.0%), including one patient in whom malignant transformation occurred in the intrahepatic bile duct in a type IVa patient 15 years after extrahepatic cyst resection. An age at symptom onset ≥ 60 years was a risk factor (p < 0.001), while an initial complete surgery was a protective factor for carcinogenesis (p = 0.001). CONCLUSIONS: Choledochal cysts should be removed once diagnosed because of an increased risk of malignant transformation with increasing age. Complete cyst removal is necessary for the first surgical treatment. Additional hepatectomy should be considered for type IVa choledochal cysts because cholangiocarcinoma can arise from the intrahepatic bile duct years after the extrahepatic cyst excision.

Increasing occurrence of choledochal malformations in children: a single-center 37-year experience from Finland.

OBJECTIVE: Few reports on choledochal malformations (CMs) in European populations exist. MATERIALS AND METHODS: The medical records of pediatric CM patients managed in our nationwide referral center for pediatric hepatobiliary surgery between 1976 and 2013 (n = 38; 71% females) were reviewed. RESULTS: Over follow-up time, the relative proportion of fusiform CMs increased significantly (p = 0.007) and the estimated total incidence rose from 1:128,000 to 1:38,000 (p = 0.017). Cystic CMs (42%) presented at younger age than fusiform CMs (47%) (0.8 vs. 4.6 years, p = 0.001). Two-thirds had abdominal pain and half were cholestatic at presentation. Pancreatitis had occurred in 16%. In addition to ultrasound, 71% underwent magnetic resonance cholangiopancreatography and 39% underwent endoscopic retrograde cholangiopancreatography. Median CM dilatation was 15 (10-28) mm and a 12 (9-13) mm long common pancreaticobiliary channel was confirmed in 61%, increasingly during recent years. Intrahepatic biliary tree was dilated in 19%, whereas main ducts were dilated in 50%. Apart from two operated in the 1970s and one with choledochocele, patients underwent resection of extrahepatic bile ducts with Roux-en-Y hepaticojejunostomy at age of 4.2 (0.8-9.2) years. Postoperative bile leakage and hemorrhage required reoperations in two. At last follow-up 4.8 (1.3-13.2) years postoperatively, none had dilated intrahepatic biliary ducts or elevated plasma bilirubin (5 [3-7] µmol/l). Single cholangitis episodes had occurred in two, whereas others were asymptomatic. CONCLUSION: CM incidence has increased significantly in Finland, being currently over threefold higher than previous estimates in the Western world would suggest. Removal of the extrahepatic biliary tree with hepaticojejunostomy for type I and IV CMs yielded excellent results.

Anomalous union of the pancreaticobiliary duct without choledochal cyst: is cholecystectomy alone sufficient?

PURPOSE: Anomalous union of the pancreaticobiliary duct (AUPBD) is frequently associated with choledochal cyst and biliary tract cancers. Management of AUPBD with choledochal cyst consists of extrahepatic bile duct excision and cholecystectomy. In cases of AUPBD without choledochal cyst, cholecystectomy alone is usually recommended.This study aimed to evaluate the occurrence of biliary tract cancer in AUPBD patients in order to assess the validity of the currently recommended operative management. METHODS: Of a total of 10,255 endoscopic retrograde cholangiopancreatography cases performed at two Korea University hospitals from 2001 to 2010, 55 (0.54 %) cases of AUPBD were identified. Patients with AUPBD were divided according to its subtype (P-C union and C-P union) and the presence of choledochal cyst for analysis. The occurrence of benign and malignant disease was evaluated and compared between the groups. RESULTS: Gallbladder stones were more frequently found in AUPBD patients without choledochal cyst (p=0.032). Biliary tract cancer occurred more frequently in P-C union (p=0.050),especially the common bile duct cancer (p=0.023). When analyzed according to the presence of choledochal cyst, biliary tract cancer occurred more frequently in AUPBD patients without choledochal cyst (p=0.005), with bile duct cancer being significantly more common (p=0.015). However, there was no difference in the presence of gallbladder cancer between the two groups (p=0.318). CONCLUSIONS: Since cancers of the biliary tract occur more frequently in the AUPBD group without choledochal cyst,cholecystectomy alone may not be protective of the future occurrence of bile duct cancers, and thus, vigilant surveillance is necessary in this population group.