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INTRODUCTION: Pancreatic lesions have varied morphology and presentation making their diagnosis challenging. The lesions may be asymptomatic incidentalomas on abdominal imaging for other conditions, symptomatic producing specific hormone effects or causing local effects. CASE: We report a 35-year-old woman with recurrent abdominal pain confirmed gastroesophageal reflux disease. Initial CT imaging reported findings of a pancreatic pseudocysts. A careful review of the imaging showed cystic dilatation of the main pancreatic duct mimicking a main pancreatic duct intra-ductal papillary mucinous neoplasm. At surgery, a small nodule palpated in the pancreatic head with sacculation in the body and tail. A histopathological review showed a pancreatic neuroendocrine tumour blocking the main pancreatic duct at the neck causing downstream dilatation and sacculation. This case highlights the difficulty of diagnosing small asymptomatic pancreatic tumours especially with limited range of imaging modalities while increasing awareness of these conditions to improve our ability to manage them effectively.

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Caroli's disease is also known as Congenital intrahepatic bile duct dilatation, and previously known as a congenital intrahepatic bile duct cyst; it is characterized by single or multiple intrahepatic cystic dilatations. In this article, we report a case of Caroli's disease (CT size 21.2 × 16.9 × 19.8 cm). Preoperative abdominal ultrasound and enhanced CT were misdiagnosed as biliary cystadenoma or hepatic echinococcosis, and finally diagnosed as Caroli's disease by postoperative histopathological examinations. Most of the disease is single or multiple cystic dilatation of small bile duct. Giant Caroli disease, cystic dilations with diameter >20 cm is very rarely seen in the clinic. The lack of experience of diagnosing giant cystic dilatation makes it difficult to make accurate diagnosis. Therefore, we analyze the causes of imaging misdiagnosis through this case report, and summarize the imaging diagnostic skills of the disease combined with relevant imaging diagnosis experience. The purpose of this study is to deepen the understanding of giant Caroli disease among imaging doctors so as to reduce the misdiagnosis of the disease in the future.

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BACKGROUND: With the widespread use of abdominal ultrasonography (US), incidental detection of common bile duct (CBD) dilatation is common in pediatric populations. This study investigated the causes and clinical significance of CBD dilatation in children without biliary symptoms, jaundice, or causative lesions in US. METHODS: We retrospectively reviewed pediatric patients with CBD dilatation from July 2013 to June 2023. All cases were detected via abdominal US. We analyzed the patients' clinical manifestations, laboratory data, diagnosis, underlying diseases, and clinical course. RESULTS: In a total of 687 patients enrolled, 338 met inclusion criteria (90 in hepatobiliary, 248 in CBD dilatation group). Of 128 patients with incidental CBD dilatation who underwent regular US examinations, 91 (71.1%) experienced resolution during follow-up. The proportion of patients with intrahepatic duct dilatation was significantly higher in the non-resolution group (p = 0.038). General health examination group had significant smaller CBD diameter compared to the gastrointestinal and infection groups. Correlation analysis found starting point of resolution decline at 3.24 mm (all-inclusive) and 2.51 mm (infant group) CBD diameter. CONCLUSIONS: Most children with incidental CBD dilatation did not have abnormal hepatobiliary function or other sonographic abnormalities. They usually remained asymptomatic and experienced uneventful clinical courses.

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BACKGROUND: Focal parenchymal atrophy and main pancreatic duct (MPD) dilatation have been identified as early signs of pancreatic ductal adenocarcinoma. However, limited evidence exists regarding their temporal progression due to previous study limitations with restricted case numbers. OBJECTIVE: To ascertain a more precise frequency assessment of suspicious pancreatic ductal adenocarcinoma findings as well as delineate the temporal progression of them. METHODS: A multicenter retrospective study was conducted on patients diagnosed with pancreatic ductal adenocarcinoma between 2015 and 2021. We included patients who had undergone at least one computed tomography (CT) scan ≥6 months before diagnosing pancreatic ductal adenocarcinoma. The temporal progression of suspicious pancreatic ductal adenocarcinoma findings on CT was investigated. RESULTS: Out of 1832 patients diagnosed with pancreatic ductal adenocarcinoma, 320 had a previous CT before their diagnosis. Suspicious pancreatic ductal adenocarcinoma findings were detected in 153 cases (47.8%), with focal parenchymal atrophy (26.6%) being the most common followed by MPD dilatation (11.3%). Focal parenchymal atrophy was the earliest detectable sign among all suspicious findings and became visible on average 2.7 years before diagnosis, and the next most common, MPD dilatation, 1.1 years before diagnosis. Other findings, such as retention cysts, were less frequent and appeared around 1 year before diagnosis. Focal parenchymal atrophy followed by MPD dilatation was observed in 10 patients but not in reverse order. Focal parenchymal atrophy was more frequently detected in the pancreatic body/tail. No significant relationship was found between the pathological pancreatic ductal adenocarcinoma differentiation or tumor stage and the time course of the CT findings. All cases of focal parenchymal atrophy progressed just prior to diagnosis, and the atrophic area was occupied by tumor at diagnosis. Main pancreatic duct dilatation continued to progress until diagnosis. CONCLUSION: This large-scale study revealed that the temporal progression of focal parenchymal atrophy is the earliest detectable sign indicating pancreatic ductal adenocarcinoma. These results provide crucial insights for early pancreatic ductal adenocarcinoma detection.

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Neuroendocrine tumors (NETs) are a rare subset of malignancies in the biliary tract that may have an aggressive and initially asymptomatic course. A 93-year-old female presented with four days of abdominal pain with associated nausea, jaundice, and brown-colored urine. A CT scan revealed a soft-tissue lesion measuring 1.9 x 1.5 x 1.9 cm within the distal-most aspect of the common bile duct and papilla with marked bile duct dilatation, pancreatic duct dilatation, and multiple hepatic lesions of varying sizes. The biliary stricture was palliated with a stent via endoscopic retrograde cholangiopancreatography. Biopsies taken from the biliary mass were consistent with a well-differentiated NET: World Health Organization, Grade 3. The patient was minimally symptomatic after stenting and was discharged home. She ultimately decided not to pursue further treatment and passed away two months after the initial presentation. Currently, surgical excision is considered the main and only curative treatment for localized NETs, although chemotherapy and radiation therapy may be suitable. Early detection and treatment of these rare NETs in the biliary tree can potentially result in curative treatment.

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Biliary duct dilatation is a common incidental finding in practice, but it is unlikely to indicate biliary obstruction in the absence of clinical symptoms or elevated levels on liver function tests (LFTs). However, the clinical presentation may be nonspecific, and LFTs may either be unavailable or difficult to interpret. The goal of this AJR Expert Panel Narrative Review is to highlight a series of topics fundamental to the management of biliary duct dilatation, providing consensus recommendations in a question-and-answer format. We start by covering a basic approach to interpreting LFT results, the strengths and weaknesses of the biliary imaging modalities, and how and where to measure the extrahepatic bile duct. Next, we define the criteria for biliary duct dilatation, including patients with prior cholecystectomy and advanced age, and discuss when and whether biliary duct dilatation can be attributed to papillary stenosis or sphincter of Oddi dysfunction. Subsequently, we discuss two conditions in which the duct is pathologically dilated but not obstructed: congenital cystic dilatation (i.e., choledochal cyst) and intraductal papillary neoplasm of the bile duct. Finally, we provide guidance regarding when to recommend obtaining additional imaging or testing, such as endoscopic ultrasound or ERCP, and include a discussion of future directions in biliary imaging.

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@#Background and Objective. Choledochal cysts (CC) are rare congenital, cystic dilations of the biliary tree occurring predominantly in Asian populations and in females. Patients are usually children presenting with any of the following: abdominal pain, palpable abdominal mass, and jaundice. Its congenital nature hints at a potential genetic cause. A possible causal gene is TP53, a tumor suppressor with a germline variant called rs201753350 (c.91G>A) that changed from a G allele to an A allele, decreasing the cell proliferation suppressing activity of its functional protein. Currently, there is no information on the TP53 rs201753350 germline variant available for the Filipino population. This study determined the prevalence of rs201753350 and the association between the functional G allele, the rs201753350 germline variant A allele, and the occurrence of CCs in Filipino pediatric patients in a tertiary government hospital. Methods. Genomic DNA was extracted from blood samples of pediatric patients clinically diagnosed with CC. Controls were DNA samples collected from a previous study. The samples underwent PCR, electrophoresis, and sequencing. Results. A total of 109 participants (22 cases and 87 controls) were included in the study. The A allele (22.94%) occurs at a lower frequency than the G allele (77.06%) among both cases and controls. More individuals have a homozygous G/G genotype (54.13%) than a heterozygous A/G genotype (45.87%) while the homozygous A/A genotype was not observed. The estimated risk of choledochal cyst occurrence is significantly lower in individuals with the A allele (PR: 0.08, 95% CI: 0.01 – 0.55) and the A/G genotype (PR: 0.06, 95% CI: 0.01 – 0.40). Conclusion. There is no significant evidence to suggest an association between the TP53 rs201753350 germline variant and the occurrence of choledochal cysts in Filipinos. It is recommended that other mutations within and beyond the TP53 gene be investigated for possible associations with choledochal cyst occurrence.

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Mirizzi syndrome (MS) describes a rare complication of cholelithiasis resulting from extrinsic compression of the common hepatic duct by impacted gallstones in the cystic duct or Hartmann's pouch. MS is most commonly seen in adults and is more prevalent in the female population. Due to the pathophysiology of MS being similar to other causes of cholecystitis and biliary obstruction, the symptomatology is rather nonspecific. While ultrasound and magnetic resonance cholangiopancreatography are commonly used for diagnosis, treatment of this condition typically involves cholecystectomy. Identifying MS versus other more common causes of obstructive jaundice is paramount in limiting complications. In this report, we describe a case of MS diagnosed in a 32-year-old male who presented with nonspecific abdominal pain and other signs of obstructive jaundice. The goal of this study is to show how identifying a rare underlying cause of a common presentation can lead to improved patient outcomes.

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Over the past two decades, there have been an increasing number of reports describing a sixth type of choledochal cyst (cystic duct cyst) in adults that was not included in Todani's classification. This sixth entity has not yet been systematically reviewed in the literature. We therefore explored this condition in adults from the perspective of the clinical presentation, diagnosis and treatment through a systematic review of the evidence. The final analysis included 33 reported cases, with 55% of them reported in Asia. The mean age was 39.3 years old, with a female-to-male ratio of 1.5:1. Magnetic resonance cholangiopancreatography was accurate in establishing the diagnosis in 69% of cases. Where reported, standard laparoscopic/open cholecystectomy was performed in about 74% of patients, while around 25% of them needed extensive surgery. Associated malignancy was reported in 6.1% of cases, while 28% of patients had co-existent gallstone-related disease. No significant post-operative morbidity or mortality was reported. In this era of emergency laparoscopic cholecystectomy, surgeons should be aware of this rare condition, with the particular understanding that it is associated with gallstone-related disease in a significant number of reported cases.

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OBJECTIVE: To conduct a long-term retrospective evaluation of the safety and effectiveness of sialographic balloon dilatation in Stensen's duct stenosis (SDS). STUDY DESIGN: Retrospective cohort. SETTING: Single-institution academic tertiary referral center. METHODS: All SDS balloon dilatations (SSBDs) performed from 2011 to 2017 were monitored. Pain relief was evaluated by a numeric rating scale at 3-year follow-up at least. Long-term glandular swelling frequency patterns, quality of life (QoL), and drug consumption were retrospectively assessed. Procedure-related complications were recorded. RESULTS: Twenty-one SSBD procedures were recorded (mean ± SD age, 55 ± 12 years), all performed under local anesthesia. SSBD led to complete dilatation in 7 patients (33%), residual stenosis in 8 (38%), and no dilatation (failure) in 6 (29%). Retrospective analysis of clinical outcomes was possible for 17 patients, 71% of whom presented with long-term pain relief, at a mean relief of 3.2 points on the numeric rating scale (P < .001). Long after SSBD, patients presented with a mean decrease of 15.4 glandular swellings per month (P < .001). Medical consumption was reduced to 18% of patients taking some drugs because of SDS after SSBD vs 71% before. SSBD showed an impact on QoL in >80% of patients, with mean improvements of 26% and 25% in the percentage point reduction of physical and mental QoL, respectively (P < .001). No complications were noted except temporary discomfort due to the procedure. CONCLUSION: Despite the advent of sialendoscopy-guided techniques, SSBD should be considered for SDS treatment, as it is a safe procedure and provides sustained pain relief.

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Introducción: La perforación espontánea de los conductos biliares es una rara enfermedad caracterizada por una disrupción no traumática de la vía biliar en pacientes aparentemente sanos. Se trata de una grave situación potencialmente letal, pero diagnosticada y tratada correctamente tiene un pronóstico excelente. Objetivo: Caracterizar los principales elementos clínico-quirúrgicos expresados en una serie de 5 pacientes operados en un servicio de referencia nacional. Presentación de casos: Se presenta la experiencia con una serie de casos en 16 años en una sola institución. La afección se observó en niñas recién nacidas y lactantes con una edad media de 4 meses, y se presentó desde la clínica como una colestasis acompañada de distensión abdominal, ascitis biliar, acolia, y signos de irritación peritoneal. El 80 % de los casos se intervinieron en el hospital "William Soler", y en un caso se ejecutó el procedimiento después de una laparotomía por una posible apendicitis aguda, en otro hospital. El diagnóstico se basó en el cuadro clínico descrito, la ecografía abdominal, la paracentesis con medición del índice bilirrubina líquido ascítico/bilirrubina sérica, y la colangiografía intraoperatoria. La cirugía definitiva se realizó inmediatamente, y consistió en: lavado peritoneal, colangiografía diagnóstica, reparación hepaticoyeyunostomía en Y de Roux y colocación de drenaje. Conclusiones: El tratamiento realizado resulta eficaz y seguro en todos los casos, con una excelente evolución, sin complicaciones importantes y con una total supervivencia posoperatoria. La colangiografía intraoperatoria permitió identificar el sitio de la perforación y diagnosticar malformaciones asociadas como dilataciones biliares congénitas y anomalías de la unión bilio-pancreática.

Introduction: Spontaneous bile duct perforation is a rare condition characterized by non-traumatic disruption of the bile duct in apparently healthy patients. It is a serious potentially lethal situation, but correctly diagnosed and treated its prognosis is excellent. Objetive: To characterize the main clinical-surgical elements expressed in a series of 5 patients operated in a national referral service. Case presentation: The experience with a series of cases during a period of 16 years in a single institution is presented. The condition was observed mainly in newborn girls and infants with an average age of 4 months, and presented clinically as cholestasis accompanied by abdominal distension, biliary ascites, acholia, and signs of peritoneal irritation. 80% of the cases were operated primarily in the "William Soler" hospital, and in one case the procedure was performed after a laparotomy for a possible acute appendicitis, in another hospital. The diagnosis was based on the clinical picture described, abdominal ultrasound, paracentesis with measurement of the ascitic liquid bilirubin/serum bilirubin index, and intraoperative cholangiography. Definitive surgery was performed immediately and consisted of: peritoneal lavage, diagnostic cholangiography, Roux-en-Y liver and jejunostomy repair and drainage placement. Conclusions: The treatment performed was effective and safe in all cases, with an excellent evolution, no major complications and total postoperative survival. Intraoperative cholangiography made it possible to identify the site of perforation and to diagnose associated malformations such as congenital biliary dilatations and anomalies of the biliary-pancreatic junction.

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A 56-year-old man was referred to our hospital for fever and back pain. Contrast-enhanced computed tomography, magnetic resonance cholangiopancreatography (MRCP) and endoscopic ultrasound revealed marked dilatation of the main pancreatic duct from head to tail and surrounding cysts with no evidence of pancreatic masses or obstructive causes. Endoscopic retrograde pancreatography (ERP) from the major duodenal papilla showed a short and narrow ventral pancreatic duct and ERP from the minor duodenal papilla revealed a dilated dorsal pancreatic duct with a narrow branch which connected to the ventral pancreatic duct. Intra-ductal ultrasound showed no nodules in the main pancreatic duct, and pancreatic cytology was negative. Peroral pancreatoscopy showed that the main pancreatic duct was covered with normal epithelium without tumors. Biopsy from the pancreatic duct showed no neoplastic changes. He was diagnosed with incomplete pancreas divisum accompanied by dilatation of dorsal pancreatic duct. Sphincterotomy of the minor duodenal papilla was performed. Seven months later, MRCP re-examination showed that the main pancreatic duct dilatation was relieved. Review of the literature identified 10 cases of pancreas divisum accompanied by dilatation of main pancreatic duct to date. To our knowledge, this is the first reported case of pancreas divisum with pancreatic duct dilatation without Santorinicele.

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Caroli's disease is a rare congenital bile duct malformation characterized by intrahepatic bile duct dilatation. This kind of situation is seldom encountered in clinical work. We report such a case who presented to our emergency department with recurrent fever as initial symptom. According to the clinical manifestation and imaging examination, a 13-year-old boy was diagnosed with suppurative cholangitis and sepsis caused by Caroli's disease. The symptoms were got relieved after antibiotic therapy upgraded from cephalosporins to carbapenems. After 5 months of follow-up, he did not have fever, abdominal pain or any other discomfort. We believe the present report is of medical significance since it serves as a reminder that Caroli's disease may have atypical presentations and be masked by non-specific clinical findings. The report hopes to enlighten our pediatric colleagues by providing more knowledge on such rare congenital disease.

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Objective: This study aimed to explore the etiology, clinical features, diagnosis, and treatment of spontaneous bile duct perforation (SBDP) in children. Methods: The clinical data of children with SBDP who were admitted to Wuhan Children's Hospital between January 2014 and January 2020 were retrospectively analyzed. Results: In all, 28 cases of children with SBDP (male, 28.6%; female, 71.4%; male-to-female ratio, 1:2.5; average age, 2.15 years) were analyzed. The most common symptoms were fever (85.7%), nausea and vomiting (78.6%), and abdominal distension (67.9%). Among the 28 patients, 26 (92.9%) had elevated hypersensitive C-reactive protein, 24 (85.7%) had an increased neutrophil percentage, and 22 (78.6%) had raised peripheral blood leukocyte counts. Moreover, 19 patients (67.9%) showed increased serum total bilirubin levels, and 5 (17.9%) showed an elevated conjugated bilirubin level. Abdominal CT examination revealed that the gallbladder wall of patients was thickened with edema, accompanied by gallbladder stenosis and gallbladder mucosa enhancement; furthermore, ascites was found in the abdominal cavity and lesser omental bursa. Twenty-two patients underwent abdominal paracentesis, and 20 (90.9%) of them were exposed to bile-based ascites. Among the 28 patients, four recovered with conservative treatment, whereas the others (85.7%) were surgically treated. Of the twenty-four patients undergoing surgery, the perforation site was found at the union of the hepatic and cystic ducts in 12 patients (50%), no perforation site was observed in 9 patients (37.5%), and a common hepatic duct was observed in 3 patients (12.5%). All 24 patients underwent stage I surgery, and temporary biliary drainage was performed because of severe abdominal inflammation. Cholangiography and enhanced CT revealed an abnormal location of the pancreatic duct joining the bile duct in 64.3% patients. Following surgery, 15 patients underwent hepaticojejunostomy. Subsequently, 3-month to 6-year follow-up (median, 30 months) indicated that the patients recovered well with no serious complications. Conclusion: SBDP in children may be associated with pancreaticobiliary malunion (PBM) and congenital weakness of the bile duct wall. However, the clinical manifestations of this condition lack specificity; this limitation can be assisted through diagnosis via abdominal CT and by performing abdominal paracentesis. Once SBDP diagnosis is confirmed, the patient should follow the principles of individualized treatment.

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BACKGROUNDS/PURPOSE: The purpose of this study was to identify risk factors and establish a treatment strategy for clinical hepatico-jejunostomy stenosis defined with intrahepatic bile duct dilatation after pancreaticoduodenectomy. METHODS: The 443 patients who underwent PD from April 2006 to December 2015 were analyzed. Clinical characteristics were compared between patients with and without clinical HJ stenosis, and risk factors for clinical HJ stenosis were analyzed. In addition, the treatment and clinical course of patients with clinical HJ stenosis were retrospectively reviewed. RESULTS: Clinical HJ stenosis defined with intrahepatic bile duct dilatation was identified in 40 patients (9.0%). Multivariate analysis revealed that the independent risk factor for clinical HJ stenosis was the hepatic duct at surgery ≤8 mm. Endoscopic HJ stenosis was identified in 36 patients, and 31 patients were treated successfully with double balloon endoscopic retrograde cholangiography; five patients required re-anastomosis (n = 3) and percutaneous transhepatic biliary drainage (n = 2). Complete obstruction of HJ was found in five patients, and treatment with DB-ERC was successful in only one patient. CONCLUSION: The independent risk factor for clinical HJ stenosis was hepatic duct diameter ≤8 mm. Most cases of endoscopic HJ stenosis were treated successfully with DB-ERC, except in patients with complete obstruction.

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Background and Aims: Identifying factors associated with increased short-term risk of pancreatic cancer in the setting of acute pancreatitis (AP) can inform clinical care decisions and expedite cancer diagnosis. Methods: A retrospective cohort study of patients hospitalized for AP between 2007 and 2017 in an integrated health-care system in Southern California. AP cases were identified by diagnosis code with laboratory confirmation. Multivariable Cox proportional hazards regression model was used to assess risk of pancreatic cancer within 3 years of AP, adjusting for patient demographics, clinical parameters (body mass index, AP etiology, chronic pancreatitis, diabetes) and radiographic imaging features. Results: Among 9,490 patients hospitalized with AP, the mean (standard deviation) age was 55.8 (17.8) years, 55% were women, and 42% were Hispanic. Majority of AP cases were biliary (64%), 12% were alcohol-related, 5% were hypertriglyceridemia-induced, and 19% were other/unknown etiology. Ninety-five (1%) patients were diagnosed with pancreatic cancer within 3 years of AP (4.2 cases/1000 person-years). Risk factors for pancreatic cancer were age ≥65 years (hazard risk [HR]: 2.5, 95% confidence interval [CI]: 1.2-5.3), male sex (HR: 1.9, 95% CI: 1.2-2.8), Asian/Pacific Islander race (HR: 2.0, 95% CI: 1.1-3.6), and underweight body mass index (HR: 2.6, 95% CI: 1.1-6.5). In addition, other/unknown AP etiology (HR: 2.0, 95% CI: 1.3-3.1) and dilatation of the main pancreatic duct (HR: 6.6, 95% CI: 4.2-10.5) were independently associated with increased risk of pancreatic cancer. Conclusion: In addition to older age, the lack of well-established etiology, underweight body habitus, and main pancreatic duct dilatation were independently associated with increased short-term risk of pancreatic cancer among patients hospitalized for AP.

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Pancreatic ductal adenocarcinoma (PDAC) accounts for the majority of all pancreatic cancers and is highly lethal. Focal parenchymal atrophy (FPA) of the pancreas has been reported as a characteristic imaging finding of early PDAC. Here, we reviewed 76 patients with PDAC who underwent computed tomography (CT) between 6 months and 3 years before PDAC diagnosis, as well as 76 sex- and age-matched controls without PDAC on CT examinations separated by at least 5 years. FPA was observed corresponding to the location of the subsequent tumor on pre-diagnostic CT in 14/44 (31.8%) patients between 6 months and 1 year, 14/51 (27.5%) patients between 1 and 2 years, and 9/41 (22.0%) patients between 2 and 3 years before PDAC diagnosis. Overall, FPA was more frequently observed in patients with PDAC (26/76; 34.2%) on pre-diagnostic CT than that in controls (3/76; 3.9%) (p < 0.001). FPA was observed before the appearance of cut-off/dilatation of the main pancreatic duct, suggesting that FPA might be the earliest sign of PDAC. FPA was less frequently found in tumors in the pancreatic head (3/27; 11.1%) than in those in the body (14/30; 46.7%) or tail (9/19; 47.4%). FPA may predict the subsequent PDAC diagnosis, serving as an important imaging sign for the early diagnosis of pancreatic cancer.

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A choledochal cyst involving the cystic duct (type 6) is a rare disease. Dilatation of the common bile duct along with the involvement of cystic duct is extremely rare and only a few cases have been reported until now. A 38-year-old woman was evaluated for complaints of pain in the upper abdomen. On initial imaging with ultrasonography (USG) and magnetic resonance cholangiopancreatography (MRCP), she was diagnosed to have a type 1a choledochal cyst. Intraoperatively we found that it was not a simple cystic dilatation of the common bile duct (CBD) alone but the cystic duct was also dilated. Simple cholecystectomy with excision of the cyst and reconstruction with a Roux en Y hepaticojejunostomy was performed. The patient is doing well after six months of follow up. This type of choledochal cyst (CDC) with combined dilatation of cystic duct and CBD has not been defined in the Todani classification. Two studies published until now have given their own extension of the Todani classification as either type 1D or 6B. Our another case where a 23-year-old female with similar complaints was diagnosed with an isolated cystic duct dilatation (type 6A); here, we did a simple laparoscopic cholecystectomy. Thus, we need to know that these distinct type of choledochal cyst exists and has to be added to the classification. It is also important to classify type 6 into two types A and B as their management differs.

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Caroli's syndrome is a rare autosomal recessive congenital disorder of the biliary tree characterized by intrahepatic bile duct dilation and hepatic fibrosis. Very few cases have been encountered in routine day-to-day practice. The patients usually present with features of cholangitis such as pain abdomen and jaundice. They may also present with features of chronic liver disease and portal hypertension. Very rarely, they may develop cholangiocarcinoma and present with jaundice, weight loss, and abdominal mass or ascites. Here, we report one such case of a young female who presented to us with features of cholangitis with sepsis and encephalopathy, which was finally diagnosed as Caroli's syndrome. The aim of presenting this case is to learn that even patients with common symptoms of pain abdomen and jaundice may be harboring some rare congenital disease like Caroli's syndrome, as in our case.