Biliary Ductal System Research Articles

Rare variant of pancreaticobiliary maljunction associated with pancreas divisum in a child diagnosed and treated by endoscopic retrograde cholangiopancreatography: A case report

BACKGROUNDPancreaticobiliary maljunction (PBM) is an uncommon congenital anomaly of the pancreatic and biliary ductal system, defined as a union of the pancreatic and biliary ducts located outside the duodenal wall. According to the Komi classification of PBM, the common bile duct (CBD) directly fuses with the ventral pancreatic duct in all types. Pancreas divisum (PD) occurs when the ventral and dorsal ducts of the embryonic pancreas fail to fuse during the second month of fetal development. The coexistence of PBM and PD is an infrequent condition. Here, we report an unusual variant of PBM associated with PD in a pediatric patient, in whom an anomalous communication existed between the CBD and dorsal pancreatic duct.CASE SUMMARYA boy aged 4 years and 2 mo was hospitalized for abdominal pain with nausea and jaundice for 5 d. Abdominal ultrasound showed cholecystitis with cholestasis in the gallbladder, dilated middle-upper CBD, and a strong echo in the lower CBD, indicating biliary stones. The diagnosis was extrahepatic biliary obstruction caused by biliary stones, which is an indication for endoscopic retrograde cholangiopancreatography (ERCP). ERCP was performed to remove biliary stones. During the ERCP, we found a rare communication between the CBD and dorsal pancreatic duct. After clearing the CBD with a balloon, an 8.5 Fr 4-cm pigtail plastic pancreatic stent was placed in the biliary duct through the major papilla. Six months later, his biliary stent was removed after he had no symptoms and normal laboratory tests. In the following 4-year period, the child grew up normally with no more attacks of abdominal pain.CONCLUSIONWe consider that ERCP is effective and safe in pediatric patients with PBM combined with PD, and can be the initial therapy to manage such cases, especially when it is combined with aberrant communication between the CBD and dorsal pancreatic duct.

Magnetic resonance imaging of fibropolycystic liver disease: the spectrum of ductal plate malformations.

Fibropolycystic liver diseases, also known as ductal plate malformations, are a group of associated congenital disorders resulting from abnormal development of the biliary ductal system. These disorders include congenital hepatic fibrosis, biliary hamartomas, polycystic liver disease, choledochal cysts and Caroli disease. Recently, it has been thought to include biliary atresia in this group of diseases, because ductal plate malformations could be implicated in the pathogenesis of this disease. Concomitant associated renal anomalies can also be present, such as autosomal recessive polycystic kidney disease (ARPKD), medullary sponge kidney and nephronophthisis. These disorders can be clinically silent or can cause abnormalities such as cholangitis, portal hypertension, gastrointestinal bleeding and infections. The different types of ductal plate malformations show typical findings at magnetic resonance (MR) imaging. A clear knowledge of the embryology and pathogenesis of the ductal plate plays a pivotal role to understand the characteristic imaging appearances of these complex diseases. Awareness of these MR imaging findings is central to the detecting and differentiating between various fibropolycystic liver diseases and is important to direct appropriate clinical management and prevent misdiagnosis.

Descriptive Anatomy of Hepatic and Portal Veins with Special Reference to Biliary Duct System in Broiler Chickens (Gallus gallus domesticus): A Recent Illustration

ABSTRACT Chickens have a great participation in meat and egg production. The anatomical scientific data of poultry is important to support the recent researches either for illustrations in academic studies or clinically in diagnosis and treatment of some poultry nutritional diseases. The current investigation was performed on twenty broiler chickens of both sexes. The chickens were anaesthetized, slaughtered then the venous system was flushed with a normal saline to anatomically investigate the distribution of hepatic portal veins both intra and extrahepatic, as well as the hepatic venous and biliary duct systems. The fowl had two hepatic portal veins draining the gastrointestinal tract with its associated organs as spleen and pancreas. The left hepatic portal vein was small, restricted to a limited portion of left hepatic lobe and had been constituted by five main venous tributaries draining the proventriculus, gizzard and pylorus, while the right hepatic portal vein was the largest, receiving the proventriculosplenic, gastropancreaticoduodenal and common mesenteric veins then piercing the right hepatic lobe to be distributed in both hepatic segments through right and left divisions. The fowl has two hepatic portal veins differed in size and distribution. A characteristic imaginary trapezoid shape was formed by some tributaries draining the caudoventral part of the gizzard. There are three ileocecal veins; cranial, caudal and the ileocecal tributary of the cranial mesenteric vein. The wall of gall bladder and the common hepatoenteric duct was characterized by the presence of well-defined longitudinal folds or striations.

ERRATAS: Descriptive Anatomy of Hepatic and Portal Veins with Special Reference to Biliary Duct System in Broiler Chickens (Gallus gallus domesticus): A Recent Illustration

ERRATAS: Descriptive Anatomy of Hepatic and Portal Veins with Special Reference to Biliary Duct System in Broiler Chickens (Gallus gallus domesticus): A Recent Illustration

The Inhibition Kinetics and Potential Anti-Migration Activity of NQO1 Inhibitory Coumarins on Cholangiocarcinoma Cells.

Altered expression of a cytosolic flavoenzyme NAD(P)H:quinone oxidoreductase-1 (NQO1) has been seen in many human tumors. Its remarkable overexpression in cholangiocarcinoma (CCA; an aggressive malignancy of the biliary duct system) was associated with poor prognosis and short survival of the patients. Inhibition of NQO1 has been proposed as a potential strategy to improve the efficacy of anticancer drugs in various cancers including CCA. This study investigated novel NQO1 inhibitors and verified the mechanisms of their enzyme inhibition. Among the different chemical classes of natural NQO1 inhibitors are coumarins, flavonoids, and triterpenoids. Coumarins are a group of particularly potent NQO1 inhibitors. The mechanisms and kinetics of enzyme inhibition of coumarin, aesculetin, umbelliferone, and scopoletin using the cell lysates as a source of NQO1 enzyme best fit with an uncompetitive inhibition model. Among the NOQ1 inhibitors tested in KKU-100 CCA cells, scopoletin and umbelliferone had the strongest inhibitory effect on this enzyme, while aesculetin and coumarin barely affected intracellular NQO1. All coumarins were further tested for cytotoxicity and anti-migration activity. At modest cytotoxic doses, scopoletin and umbelliferone greatly inhibited the migration of KKU-100 cells, whereas coumarin and aesculetin barely reduced cell migration. The anti-migration effect of scopoletin was associated with decreased ratio of matrix metalloproteinase 9/tissue inhibitors of metalloproteinases 1 (MMP9/TIMP1) mRNA. These findings suggest that natural compounds with potent inhibitory effect on intracellular NQO1 have useful anti-migration effects on CCA cells. In order to prove that the potent NQO1 inhibitor, scopoletin, is clinically useful in the enhancement of CCA treatment, additional in vivo studies to elucidate the mechanism of these effects are needed.

Cholangioscopy in the digital era.

Cholangioscopy allows direct visualization and subsequent therapeutic maneuvers of the biliary ductal system. With advances in endoscopic and imaging technology, cholangioscopy has become an important modality for the diagnosis of indeterminate biliary strictures and an essential therapeutic tool for difficult to remove biliary stones. Enhanced imaging and operability of the latest generation cholangioscopes have further expanded their clinical applications to include ductal tumor ablation, gallbladder drainage, access to difficult to reach branches of the biliary tree, and biliary foreign body manipulation. In this review, we discuss the technical evolution of cholangioscopy into the digital era and review the clinical evidence supporting its use in the diagnosis and therapy of biliary tract disease.

Role of a pre-operative radiological scoring system in determining resectability for potentially resectable hilar cholangiocarcinoma

AimsCholangiocarcinoma is a rare cancer arising from the biliary tree. Case series indicate that 25–40% of all borderline resectable primary tumours are potentially resectable. The Memorial Sloane Kettering System (MSKCC) stratifies patients for resectability by longitudinal and radial extension of the hilar tumour. The Bismuth-Corlette system describes the longitudinal extension of the tumour within the biliary duct system. We sought to validate and, if possible, augment these two scores within an independent validation cohort. MethodsPatients diagnosed with hilar cholangiocarcinoma between January 2009 and December 2016 were analysed from a prospectively held database. Patients with distal cholangiocarcinoma, peripheral cholangiocarcinoma and gallbladder cancer were excluded. Comparison of surgical findings to pre-operative radiological imaging was undertaken at the time of surgery. ResultsThe validation cohort was formed of 198 patients, of which, 55 (27.8%) patients underwent resection. Logistic regression analyses identified that BC score, MSKCC score, age at diagnosis and left artery involvement were all significant independent predictor's univariately. BC score explained 28% of the variability in resectability compared to 26% explained by MSKCC. In combination, the model consisting of BC score, age at diagnosis and left artery involvement explained 39% of variability in resectability compared to the 34% explained same model including MSKCC score instead of BC score. ConclusionIn this cohort an augmented BC score, incorporating left hepatic artery involvement, is more discriminative in predicting resectability than the current MSKCC system.

Laparoscopic left hepatectomy for Klatskin tumour

Introduction: Surgical management of hilar cholangiocarcinoma remains the only treatment option with curative intent and potential influence on overall survival. Methods: In our case report, we describe a complete laparoscopic resection of Klatskin's tumour which consists of left hepatectomy with intracorporeal biliary reconstruction with Roux-en-Y hepaticojejunostomy. Results: A 28-year-old woman presented with obstructive jaundice without other symptoms. Her CEA, Ca-125, AFP and Ca 19-9 values were normal. CT, ERCP and MRCP showed a Bismuth type III.b. neoplastic lesion, without vascular involvement. Diagnostic laparoscopy was performed with ultrasonography. Cholecystectomy with resection of the extrahepatic biliary ductal system and transection of prestenotic right biliary branch was done. Left branches of the hepatic artery and portal vein were clipped and hepatoduodenal lymphadenectomy were performed. Segment II-IV were removed without caudate lobectomy and left hepatic vein was clipped. After removal the specimen through a minilaparotomy and construction a Roux-en-Y loop bilioenteric anastomosis was performed using total laparoscopic procedure. The operation time was 255 minutes with intraoperative blood loss of 200 ml. The pathologic result showed a GII., pT2b, pN0 primary hilar cholangiocarcinoma with all resected margins and nodes free of tumour (0/4), with perineural but no vascular invasion. The postoperative hospital stay was uneventful and the patient was discharged on day 14. The patient had adjuvant gemcitabine, 5-FU combination chemotherapy. The patient is well at 12-month visit with normal results of liver function test and MRCP. Conclusion: Laparoscopic resection is a feasible surgical approach in selected patients with hilar cholangiocarcinoma.

Cellular and stromal elements organization in the liver of grass carp, Ctenopharyngodon idella (Cypriniformes: Cyprinidae)

Fish liver is considered as a key organ that controls various life functions. The cellular and stromal elements of liver of eighteen specimens of adult grass carp were investigated by light- and electron- microscopy and enzyme histochemistry. The liver was formed of two lobes with a long process extended from the right lobe. Serial paraffin section of the liver identified different kinds of vascular- biliary structures as follows: 1) pancreatic–venous–biliary–arteriolar tracts (P-VBAT); 2) venous-biliary-arteriolar tracts, (VBAT); 3) pancreatic–venous–biliary tracts (P-VBT); 4) venous-biliary tracts (VBT); 5) venous–arteriolar tracts (VAT); 6) isolated veins named as venous tracts (VT); 7) isolated bile ducts, named as biliary tracts (BT); 8) biliary-arteriolar tracts (BAT); 9) pancreatic–biliary tracts (P-BT); 10) pancreatic– venous tracts (P-VT). Macrophages aggregates were associated with VBT and P-BT. The hepatic parenchyma was consisted of many populations of cells. Histochemically, the hepatocytes were strongly reacted with PAS, and Best's carmine. Moreover, strong staining patterns for acid phosphatase, ATPase, and alkaline phosphatase were demonstrated in hepatocytes. The hepatic satellate (Ito) cells were observed in the space of Disse and between hepatocytes. Rodlet cells and eosinophilic granular/ mast cells were encountered in the liver of grass carp. The sinusoids were lined by fenestrated endothelial cells and Kupffer cells. Moreover, dendritic-like cells were demonstrated in the sinusoids and perisinusoidal connective tissue. The biliary duct system was constituted of bile canaliculi, ductules, and bile ducts. Telocytes with their characteristics telopodes were located around bile ducts. The current findings are offering fundamental data on histology of grass carp liver.

Analysis of caudate lobe biliary anatomy and its implications in living donor liver transplantation - a single centre prospective study.

Biliary complications are a significant cause of morbidity after living donor liver transplant (LDLT). Bile leak may occur from bile duct (anastomotic site in recipient and repaired bile duct stump in donor), cystic duct stump, cut surface pedicles or from divided caudate ducts. The first three sites are amenable to post-operative endoscopic stenting as they are in continuation with biliary ductal system. However, leaks from divided isolated caudate ducts can be stubborn. To minimize caudate duct bile leaks, it is important to understand the anatomy of hilum with attention to the caudate lobe biliary drainage. This single-centre prospective study of 500 consecutive LDLTs between December 2011 and December 2016 aims to define the biliary anatomy of the caudate lobe in liver donors based on intraoperative cholangiograms (IOCs) with special attention to crossover caudate ducts and to study their implications in LDLT. Caudate ducts were identified in 468 of the 500 IOCs. Incidence of left-to-right crossover drainage was 61.37% and right to left was 21.45%. Incidence of bile leak in donors was 0.8% and in recipients was 2.2%. Proper intraoperative identification and closure of divided isolated caudate ducts can prevent bile leak in donors as well as recipients.

Tu1375 - Differential Requirement of IL-4Rα for Tuft and Goblet Cell Hyperplasia in the Intrapancreatic Biliary Duct System after Pancreatitis

Tu1375 - Differential Requirement of IL-4Rα for Tuft and Goblet Cell Hyperplasia in the Intrapancreatic Biliary Duct System after Pancreatitis

Surgical oriented anatomical study of variations in extra-hepatic biliary ductal system & its related vessels with its clinical significance

Surgical oriented anatomical study of variations in extra-hepatic biliary ductal system & its related vessels with its clinical significance

Temporary Biliary Stent Placement for Benign Stricture with Laparoscopic-Assisted Endoscopic Stent Retrieval in a Patient Status Post Roux-en-Y Gastric Bypass: A Multidisciplinary Approach to a Complex Condition

AbstractBenign biliary strictures are multifactorial in etiology and can be difficult to treat, causing significant morbidity and mortality. In patients with prior upper gastrointestinal surgery, such as Roux-en-Y gastric bypass, options for intervention are limited by the inability of an endoscope to reach the biliary ductal system due to altered anatomy. In patients with an inaccessible ductal system by endoscopic retrograde cholangiopancreatography (ERCP), percutaneous bile duct stent placement for benign strictures has traditionally been reserved for cases of failed cholangioplasty without additional surgical options. Our case details a novel multidisciplinary management approach for the treatment of a refractory biliary stricture in a patient with Roux-en-Y gastric bypass anatomy with remnant gastrectomy. Following interventional radiology-guided retrievable biliary stent placement and subsequent remodeling, laparoscopy-assisted ERCP with stent retrieval was performed through the combined efforts of minimally invasive surgery and gastroenterology.

Clinical characteristics of 88 cases of acute pancreatitis in children of different age

Objective To investigate the clinical features of acute pancreatitis (AP) in children of different age. Methods From January 2010 to December 2015, pediatric inpatients with AP were searched by pediatric inpatient medical records retrieval system. According to age, patients were divided into three groups: infant and toddler group (age 0 years to three years), preschool group (age >three years to six years) and school-aged group (age >six years to 16 years). The clinical features, etiology and laboratory findings of different age groups were retrospectively analyzed. One-way analysis of variation or rank sum test was performed for measurement data analysis. Chi-square test or Fisher exact probability test was used for the count data analysis. Results A total of 88 pediatric patients (male 38, female 50) were enrolled, and the mean age was (8.8±4.4) years. Infant and toddler group, preschool group and school-aged group were 16, 14, 58 cases, respectively. The average days of hospitalization of infant and toddler group, preschool group and school-aged group were (8.4±1.9), (9.4±2.6) and (7.5±2.5) days, respectively, and the difference was statistically significant (F=3.649, P=0.030). About 93.2% (82/88) pediatric patients had abdominal pain. The incidence rate of epigastric distention of school-aged group (34.5%, 20/58) was higher than those of infant and toddler group (2/16) and preschool-aged group (1/14). The rate of nausea/vomiting of infant and toddler group was significantly higher than those of preschool group and school-aged group (12/16, 4/14 and 46.6% (27/58), respectively), and the differences were both statistically significant (χ2=6.250 and 6.805, both P 0.05). The percentage of obese children of school-aged group was significantly higher than those of infant and toddler group and preschool group (31.0%, 18/58; 1/16 and 1/14, respectively), and the difference was statistically significant (χ2=6.689, P=0.035). In addition, the level of serum total cholesterol of school-aged group was higher than that of infant and toddler group and preschool group ((5.1±0.9), (3.9±0.6) and (4.8±0.8) mmol/L, respectively), and the difference was statistically significant(F=13.855, P<0.01). The positive rates of abdominal ultrasound of infant and toddler group, preschool group and school-aged group were 5/16, 4/14 and 21.1%(12/57), respectively, and there was no statistically significant difference (χ2=0.889, P=0.706). The positive rates of magnetic resonance cholangiopancreatography (MRCP) of infant and toddler group and school-aged group were 4/4 and 5/6, respectively, and which were both higher than those of pancreases computed tomography (CT) examination (4/14 and 51.2%, 22/43) and those of abdominal ultrasound (5/16 and 21.1%, 12/57), and the differences were significant (χ2=6.655 and 15.207, both P<0.05). Conclusions Obese children is more in school-aged children with AP. Children of this age should pay more attention to life style. Nausea or vomiting symptom is more obvious in the infant and toddler children with AP and the condition is more severe. If children of this age with unexplainable vomiting, AP should be considered. Biliary factors are more common in the infant and toddler children with AP. When children are suspected with abnormal structure of pancreatic and biliary ductal system, MRCP should be conducted. Key words: Acute pancreatitis; Clinical characteristics; Etiologies; Children

Endoscopic Retrograde Cholangiopancreatography

Endoscopic retrograde cholangiopancreatography (ERCP) is a specialized endoscopic procedure to view the biliary and pancreatic ducts fluoroscopically. First introduced in the 1970s as a diagnostic tool, ERCP has since evolved primarily into a therapeutic modality and is today regarded as the premier tool for performing therapeutic interventions involving the biliary and pancreatic ductal systems. Relatively complex, ERCP requires advanced training. Although generally considered safe, it does carry a risk of significant complications, including pancreatitis; thus, the most important factor to mitigate complications is to ensure the presence of an appropriate indication for the procedure. This review addresses the indications (e.g., disorders of the major duodenal papilla, biliary diseases, and pancreatic diseases), contraindications, protocol, and complications of ERCP, as well as an overview of the tools available for ERCP procedures. Figures show ampullary adenomas, choledocholithiasis, Mirizzi syndrome, benign common bile duct stricture, primary sclerosing cholangitis, malignant biliary stricture, bile leak, type I and III choledochal cysts, chronic pancreatitis, pancreatic ductal disruption, classic fish-mouth appearance of the papilla in a patient with main duct intraductal papillary mucinous neoplasm, pancreas divisum, side-viewing duodenoscope used for ERCP, and selected tools of ERCP. Tables list the Rome III criteria, risk-stratifying patients for choledocholithiasis, diagnostic criteria for acute cholangitis, choledochal cyst classification, and the Cambridge classification system for chronic pancreatitis. This review contains 15 highly rendered figures, 5 tables, and 77 references.

Intrahepatic Biliary Intraductal Papillary Mucinous Neoplasm With Intermittent Jaundice: Case Report

Intraductal papillary mucinous neoplasms (IPMNs) represent a relatively new clinical entity. IPMNs have malignant potential which exhibit a broad histological spectrum, ranging from adenoma to invasive carcinoma. Although IPMNs are commonly seen within pancreas, they may develop anywhere within the biliary ductal system and produce mucin which may cause intermittent jaundice. This report details the case of 50-year-old woman presenting with intermittent jaundice who was diagnosed as hepatic IPMN.

Surgical outcome of choledochal cysts in adults: a prospective cohort study

Background Choledochal cysts (CCs) are cystic dilatation of the biliary ductal system. Adult cases comprise around 20% of all cases. Purpose The aim of this study was to integrate all possible technical methods to prevent complications arising from residual choledochal tissue by presenting our experience in adult patients. Patients and methods A prospective cohort study of 24 adult patients, who underwent surgery for CC, over a 4-year period from March 2013 to February 2017 at the Gastroenterology Surgical Unit, Faculty of Medicine, Alexandria University, Egypt, was carried out. Cysts were classified according to the Todani classification. Biliary anatomy was defined by intraoperative cholangiography. Results The present study included six males and 18 females. Their ages ranged from 18 to 43 years (mean 26.4 years). Pain was the most common symptom at presentation (20 patients, 83.3%). Eighteen patients (75%) had type I cysts. All patients underwent excision of the extrahepatic bile duct cyst. The mean follow-up period was 34.6 months. Conclusion The present study showed satisfactory medium-term results following surgical resection of adult CC. Our approach was effective, to a great extent, in preventing complications of residual cysts. Excision of the extrahepatic bile duct should be guided by intraoperative cholangiography and distal clips to avoid pancreatic duct injury.

Dramatic Effect of Rituximab in Immunoglobulin G4-Related Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a cholestatic liver disease characterized by fibrotic obliteration of the biliary ductal system. PSC is presumed to be an autoimmune disorder but there is no specific pharmacological therapy. Ursodiol (UDCA) often improves alkaline phosphatase levels but it has not shown an effect on the natural history of PSC. An IgG4 variant of PSC has been identified which seems to respond to corticosteroids, in contrast to classic PSC, but little is known about the therapeutic options for IgG4 PSC. We report a patient who only responded to high doses of steroids, but upon infusions of rituximab exhibited a dramatic and sustained response. A 68-year-old female developed obstructive jaundice with MRCP revealing a stricture in the CBD. Suspecting cholangiocarcinoma, she had an exploratory laparotomy and resection of the bile duct stricture. Pathology of the resected bile duct showed no malignancy but abundant plasma cells; this led to stain for IgG4, which was markedly positive. She thus began prednisone (40 mg/d) which resolved the cholestasis and improved liver chemistries. However repeated attempts to taper steroids were followed by biochemical relapse. Repeat MRCP revealed no obstructive lesions, and she was forced to resume and remain on high-dose prednisone. Mycophenolate was tried as a potential steroid-sparing agent, resulting in some decline in liver enzymes, but ultimately had to be discontinued due to severe facial rash. UDCA administration had no significant effect. Due to the lack of response and considering the IgG4 plasma cells observed on the resected bile duct specimen, the decision was made to try plasma cell suppressive therapy. At the peak of the patient's liver enzymes, rituximab infusions were started biweekly (Figure 1). By September 2015, after only 5 infusions, the patient had near complete resolution of liver chemistries. Prednisone was gradually tapered off, and the patient's symptoms and liver enzymes remained within normal limits to this date.Figure 1The IgG4 variant of PSC is uncommon (5% or less of PSC) but a few reports suggest it responds to high-dose steroids. For patients with incomplete remission, steroid dependency, or steroid intolerance, there are no other options. Our patient demonstrates that in cases of histology-verified IgG4 PSC, the use of rituximab, a monoclonal antibody directed against B-lymphocytes, could be considered capable of inducing a sustained steroid-free remission.

Two-Dimensional Ultrasonographic Prenatal Diagnosis of Choledochal Cyst: Our Experience and Literature Review

Choledochal cyst is an abnormality of the biliary ducts, characterized by a dilatation of a portion of the biliary ductal system (most commonly a portion or the whole of the extrahepatic bile duct system) that presents usually at ultrasonography as a cystic mass in the right upper quadrant abdomen. Ultrasonographic prenatal diagnosis may be challenging since the detection of any intraabdominal cyst raises the issue of differential diagnosis considering its nature as well as the organ of origin. In this paper, we report our experience with a case of a choledochal cyst strongly suspected during routine mid - trimester ultrasound scan, when a round liquid mass was found in the right upper quadrant of the abdomen; the cyst was independent of the other intra-abdominal organs but a connection was observed between the latter cyst and dilated bile ducts. The diagnosis was confirmed at birth and the infant underwent successful surgical correction of the anomaly. We review the current literature as well, discussing the current open challenges of diagnosing and managing these cases.

A Rare Case of Intraductal Papillary Mucinous Neoplasm of Biliary Duct in a Patient With Prostate Adenocarcinoma

A 80-year-old male was evaluated for elevated prostate specific antigen (PSA) level and biopsy of prostate gland revealed Adenocarcinoma. Patient underwent cross sectional tomography (CT) imaging of abdomen which showed 2.3 x 2.7 cm soft tissue mass centered at porta hepatis with diffuse dilatation of left intrahepatic biliary ductal system and mild prominence of right intrahepatic ductal system. On physical examination, head, eyes, ears, nose and throat were normal. There was no jaundice. Abdomen was soft, non-tender, non-distended, no masses or free fluid and normal bowel sounds. Laboratory evaluation showed normal liver enzymes, lipase and amylase. Carbohydrate Antigen (CA 19-9) was 35.2 units/ml. The patient underwent endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreaticography (ERCP). EUS showed 2 adjacent hilar masses involving common hepatic duct and left hepatic duct with protrusion of the tissue into the lumen of the duct and upstream ductal dilatation with parenchymal atrophy. ERCP revealed a large filling defect in the common hepatic duct extending into the left hepatic duct. A large amount of clot and soft tissue with a fish-egg appearance was retrieved. Two pigtail biliary stents were placed into the left hepatic duct upstream from soft tissue mass. The biopsy of the soft tissue revealed intraductal papillary neoplasm of the bile duct with diffuse high-grade dysplasia and intramucosal carcinoma. The patient underwent radical resection of common hepatic duct, left hepatic lobectomy with Roux-en Y hepaticojejunostomy to the right hepatic duct. Histopathological examination of the resected specimen revealed intraductal papillary neoplasm with diffuse high-grade dysplasia. The surgical margins and lymph nodes were negative for carcinoma. Post-operative course was complicated by intra-abdominal abscesses and pneumonia requiring intravenous antibiotics and CT guided drainage. Follow up CT scan, 2 months after the surgery was negative for any masses. Patient feels well and is asymptomatic 12 months since surgery. Intraductal Papillary Mucinous Neoplasms (IPMNs) are intraductal mucin-producing tumors arising from epithelial cells and have malignant potential. It usually arises from the pancreatic duct. It can lead to cyst formation or papillary proliferation and may have varying degrees of cellular atypia. The malignant potential rises with increasing size (> 3cm). Multiple genetic mutations have been described. IPMN of the biliary tract are rarely reported in literature. It is more often associated with higher levels of CA 19-9 and Carcinoembryonic antigen (CEA). Most IPMNs are benign and non invasive, however due to increasing size and malignant potential, surgical resection is the treatment of choice. Our patient's IPMN was 2.7 cm with high-grade dysplasia that was treated with surgical resection.