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Biliary strictures caused by inflammation or fibrosis lead to jaundice and cholangitis which often make it difficult to distinguish malignant strictures. In cases when malignancy cannot be excluded, surgery is often performed. The concept of immunoglobulin G4 (IgG4)-related sclerosing cholangitis (SC) as a benign biliary stricture was recently proposed. The high prevalence of the disease in Asian countries has resulted in multiple diagnostic and treatment guidelines; however, there is need to formulate a standardized diagnostic strategy among various countries considering the utility, invasiveness, and cost-effectiveness. We evaluated accuracies of various diagnostic modalities for biliary strictures comparing pathology in the Delphi meetings which were held in Rochester, MN. The diagnostic utility for each modality was graded according to the experts, including gastroenterologists, endoscopists, radiologists, and pathologists from the United States and Japan. Diagnostic utility of 10 modalities, including serum IgG4 level, noninvasive imaging, endoscopic ultrasound, endoscopic retrograde cholangiopancreatography-related diagnostic procedures were advocated and the reasons were specified. Serum IgG4 level, noninvasive imaging, diagnostic endoscopic ultrasound and intraductal ultrasonography under endoscopic retrograde cholangiopancreatography were recognized as useful modalities for the diagnosis. The information in this article will aid in the diagnosis of biliary strictures particularly for distinguishing IgG4-SC from cholangiocarcinoma and/or primary SC.

Recurrent acute pancreatitis is characterized by frequent episodes of inflammation in the pancreas. The most common causes are alcohol abuse and gallstones but approximately 30% of cases are defined as idiopathic because initial evaluation fails to detect the etiology. In these patients, extensive laboratory and imaging evaluations usually lead to the uncovering of an occult biliary duct stone or sphincter of Oddi dysfunction as the main reason for frequent pancreatitis. We report a 42-year-old female with a longstanding history of acute recurrent pancreatitis in the setting of pancreas divisum due to its rarity. We focus on appropriate investigations by application of specialized laboratory and radiological modalities because determining the etiology is a crucial step in patient management.

Highlights • Bouveret’s syndrome is a rare entity which takes part of the gallstone ileus entities. • There are no standardized recommendations for workup and management, and there is a high chance of misdiagnosis. • In front of its vague presentation, various differential diagnosis should be taken into consideration. • The diagnosis is usually confirmed by CT scan, showing the fistula and Rigler’s Triad. • Surgery is most of the times the definitive treatment., Introduction This report is a case of proximal gastric outlet obstruction, misdiagnosed after esophagogastroduodenoscopy as invasive pancreatic malignancy. We report this case because of its rarity and to encourage physicians to take this etiology into consideration as part of differential diagnosis. Presentation of case We present a case of 88 year-old lady presenting for 3 days history of post-prandial coffee ground vomiting associated with obstipation, misdiagnosed as pancreatic malignancy with duodenal invasion after outpatient gastroscopy. The proper diagnosis was confirmed by CT Scan which revealed the presence of large calculi with aerobilia and gastric stasis. Surgery done to extract two impacted large gallstone of 2 × 2 × 1 and 6 × 3.5 × 3 cm followed by cholecystectomy, closure of fistulae, gastrojejunsotomy and vagotomy. Discussion Bouveret Syndrome is referred to as rare presentation of gastric outlet obstruction by passage of large gallstone through a fistula between the gallbladder and proximal gastrointestinal tract. It has a vague presentation, and can be misdiagnosed due to non-specific symptoms. The diagnosis is usually confirmed by a CT scan and it is treated surgically most of the time. Conclusion Bouveret syndrome is a rare entity, and should be taken into consideration as a differential diagnosis in front of proximal gastric outlet obstruction.

Highlights • Hydatid disease is a parasitic infestation by a tapeworm of the genus Echinococcosis of which 50%–75% of hydatid cysts occur in the liver. • In approximately one-fourth of the cases, hepatic hydatid cyst ruptures into the biliary tree producing obstructive jaundice. • Moreover, primary hydatid cysts of the biliary tract have been reported in English literature. • This is an interesting and intriguing case of obstructive jaundice caused by hydatid cyst in extrahepatic ducts 13 years after liver hydatid endocystectomy. • ERCP represent an important management strategy for patients with hydatid cysts in extra-hepatic ducts even primary or complications of liver hydatid cyst., Introduction Hydatid disease affects most commonly the liver, and complications with the rupture into the biliary tree develop in approximately one-fourth of the cases. Moreover, primary hydatid cysts of the biliary tract have been reported. Presentation of case We report an extremely rare case of obstructive jaundice caused by hydatid cyst in extrahepatic ducts 13 years after liver hydatid endocystectomy treated by Endoscopic retrograde cholangiopancreatography (ERCP). A 28-year-old male patient who had undergone surgical treatment – removal of liver hydatid cyst 13 years earlier, presented with signs of obstructed jaundice, confirmed with blood tests results and magnetic resonance cholangiopancreatography (MRCP). Actually, there were no pathological changes detected in the hepatic parenchyma, but the intrahepatic and extrahepatic bile ducts were dilated. ERCP was performed and the entire hydatid material was evacuated and washed out into the gastrointestinal tract. In addition, after laparoscopic cholecystectomy, hydatid cysts were also confirmed in the gallbladder. Discussion Generally, the obstructive jaundice caused by hydatid cyst in the extrahepatic ducts can also be caused by the rupture of the liver hydatid cyst in the biliary tract, or by primary hydatid cyst in the biliary tract. The ERCP plays a key role in the diagnosis and the treatment of this pathology. Conclusion The ERCP, has now become an important diagnostic and therapeutic procedure in management of primary extrahepatic hydatid cysts and of complicated liver hydatid cysts.

Highlights • Lemmel syndrome is a rare condition that leads to cholangitis and/or pancreatitis due to intraduodenal diverticulum. • Surgery is considered for the treatment of severe or repeated symptoms in patients with this condition. • We herein report a successfully diagnosed and treated case of Lemmel syndrome with pancreaticobiliary maljunction., Background Lemmel syndrome is a rare condition that leads to cholangitis and/or pancreatitis due to intraduodenal diverticulum. Surgery is considered for the treatment of severe or repeated symptoms in patients with this condition. Case presentation An 81-year-old woman was admitted to our hospital, complaining of general fatigue, BT 38.8 degree, and right hypochondoralgia. Her hepatobiliary enzyme levels were elevated, and enhanced abdominal computed tomography revealed dilation of the common bile duct and intraduodenal diverticulum. After restarting oral intake, her symptoms were exacerbated. Endoscopic retrograde cholangio-pancreatography (ERCP) revealed pancreaticobiliary maljunction and parapapillary diverticulum. Under a diagnosis of Lemmel syndrome with pancreaticobiliary maljunction complicated by acute pancreatitis and cholangitis, we performed extrahepatic bile duct resection with cholecystectomy and papilloplasty. Her postoperative course was uneventful, and the patient was discharged 20 days after surgery. She remains well at 5 months after surgery. Conclusion We herein report a successfully diagnosed and treated case of Lemmel syndrome with pancreaticobiliary maljunction.

Introduction Many studies looked at outcomes and risk factors in laparoscopic cholecystectomies in general, including a few studies on risk factors and scoring systems in predicting conversion to open surgery. Little data has been produced on high-risk patients undergoing cholecystectomy. Identifying risk factors in this group could help stratify decision making regarding best management strategies. The aim of this study was to investigate outcomes of laparoscopic cholecystectomies in patients with ASA 3 and 4. Methods Data was collected and collated from a prospectively maintained database of all laparoscopic cholecystectomies performed by 13 general surgeons in a single unit. Case notes were reviewed for all patients with ASA 3 and 4 between 2013 and 2017. Data analysis was performed using R studio v 3.4. Results 244 cases were reviewed. Common bile duct was dilated in 52 cases (21.31%). Gall bladder wall was thick in 102 (41.8%) of the patients. Surgery was elective in 203 (83.2%) of the patients. ERCP was performed in 41 (16.9%) of the patients prior to surgery. 150 patients (62.2%) stayed for 1 day while 36 (14.9%) stayed for 2 days and the remaining 55 (22.9%) stayed for 3 days or more. Complications occurred in 37 (15.16%) of the patients while 23 (9.43%) of the patients were readmitted. 7 patients (2.87%) returned to theatre and 8 (3.28%) stayed in ITU post-op. Two patients died (0.82%). Conclusion Laparoscopic cholecystectomies in higher risk populations are safe. Alternative methods such as cholecystostomy and ERCP may be of benefit in these patients., Highlights • Gallstone disease represents a significant volume of elective and emergency work in the United Kingdom. • The primary aim of the study was to explore the factors that lead to complications in high risk patients (ASA 3-4). • Histological gallbladder thickness and emergency surgery were the factors most strongly associated with negative outcomes. • Age greater than 65 as an independent variable does not lead to an increase in negative outcomes. • Cholecystectomy is a relatively safe procedure in what is typically considered higher risk patients.

Introduction Adenoma and intra-adenoma carcinoma of the gallbladder are relatively rare diseases, and the World Health Organization classification reports a frequency of 0.3% for gallbladder adenomas. Precise preoperative diagnosis of gallbladder cancer, especially in the early stages, is challenging. Herein, we report a case of pyloric adenomatous carcinoma of the gallbladder, diagnosed by laparoscopic cholecystectomy and pathology, along with a literature review. This case was reported in accordance with the SCARE 2020 Guideline (Ref). Presentation of case A 62-year-old woman was diagnosed with a 4-mm polypoid lesion in the gallbladder during a medical examination. The patient was followed-up by ultrasonography (US) once a year and was referred to our department because of an increase in size. Carcinoembryonic antigen and carbohydrate antigen 19-9 levels were within normal limits. Abdominal ultrasonography revealed a pedunculated polypoid lesion in the body of the gallbladder measuring 8 mm. Computed tomography demonstrated that the whole tumor was enhanced in the early phase without significant lymph node enlargement. Magnetic resonance cholangiopancreatography demonstrated a type Ip polypoid lesion located in the body of the gallbladder without pancreaticobiliary junctional abnormalities. Endoscopic ultrasound detected a superficial nodular-type Ip polypoid lesion in the gallbladder body with a parenchyma-like internal echogenic pattern. Discussion Based on these findings, the patient was diagnosed with gallbladder adenoma, and laparoscopic cholecystectomy was performed. Histopathological examination revealed the tumor was a papillary growth of atypical high columnar epithelial cells. The final diagnosis was pyloric adenoma with high-grade dysplasia and intra-adenoma carcinoma. The patient is currently undergoing outpatient follow-up without recurrence for 1 year. Conclusion Early gallbladder carcinoma with adenoma should be considered in patients with small gallbladder polypoid lesions. Considering the surgical stress of cholecystectomy and the malignant potential of gallbladder cancer, preceding surgery would be acceptable., Highlights • Incidental gallbladder cancer is rare and preoperative assessment of GBPL is important. • Considering the malignant potential of GBC, preceding surgery would be acceptable.

Introduction Lemmel's syndrome is a rare pathology that presents jaundice or dilation of common biliary tract, secondary to duodenal diverticula. Due to infrequency, a high suspicion has to be considered to avoid omitting this diagnosis, mainly in patients who present direct hyperbilirubinemia without choledocholithiasis. Presentation of case A 76-year-old female admitted to the emergency department with cholangitis, secondary to an ampullary diverticulum (papilla was located inside the diverticulum). As a consequence, the endoscopic retrograde cholangiopancreatography failed and a biliodigestive derivation (choledochal duodenum anastomosis) was performed. Discussion and conclusions This case is an example of an unusual clinical and anatomical presentation of duodenal diverticulum. This unusual presentation is an example of the importance of not overlooking a diagnosis, which can lead to more severe complications such as cholangitis. It is also important to consider that cases like this are risk factors for failed endoscopic management and surgical possibilities procedures should also be considered in the management., Highlights • Unusual clinical and anatomical presentation of duodenal diverticulosis • Risk factors for failed endoscopic retrograde cholangiopancreatography • Uncommon cholangitis cause, and direct hyperbilirubinemia without choledocholithiasis

Laparoscopic cholecystectomy can be complicated by a post- operative biloma. Bile leak from the duct of Luschka is reported to be the second most frequent cause, reported in 0.15%-2% of the patients. This case report aims to underline the significance of this anatomic variation and how the management of the aforementioned complication can be facilitated by MRI- MRCP. A 78 year old male patient underwent an elective laparoscopic cholecystectomy and was found to have a post-operative biloma. An MRCP was carried out to visualize the bile tree and bile leak was identified to be originated from a duct of Luschka. The patient was referred for an ERCP, sphingterotomy and placement of biliary stent to release the pressure in the bile ducts. In the next few days the bile leak was controlled and eventually ceased. The patient was discharged free of symptoms and no sign of bile leak was to be found on his follow up imaging. In comparison with other imaging modalities picturing the bile tree, MRCP fits the ideal profile to be used as a first line choice for clinicians, as it offers detailed anatomical images with high contrast between bile and adjacent tissues, without using any contrast agent or radiation. Keywords: Postoperative biloma, Duct of Luschka, Bile tree variations, MRCP

Highlights • Gallbladder mucocele extremely rare etiology of GOO. • Atypical symptoms made diagnosis difficult. • Surgical intervention is suggested., Introduction Gallstone(s) impacted at the distal small bowel causing intestinal obstruction as a result of cholecystoenteric fistula is a well-known, albeit uncommon surgical condition. The rare Bouveret’s Syndrome, which refers to the proximal impaction of gallstone(s) in the duodenum or pylorus resulting in the gastric outlet obstruction (GOO), has also been described in the literature. However, gallbladder mucocele with extrinsic compression of the duodenum and/or pylorus causing GOO is a separate entity that is extremely rare. Presentation of case A patient who presented with loss of appetite and weight, with intermittent vomiting over a course of eight months was found to have GOO secondary to extrinsic duodenal compression from a large gallbladder mucocele. Surgical intervention in the form of cholecystectomy was performed, and the patient’s symptoms resolved post-operatively. Discussion We describe an extremely rare case of GOO, which can be easily corrected with cholecystectomy. Removal of the gallbladder removes the root cause of the issue, and should be first line therapy unless the patient is unfit for surgery. Conclusion This extremely rare cause of GOO should be recognized, and treated promptly with surgery if possible.

Introduction and importance Pancreaticobiliary maljunction (PBM) is a rare congenital anomaly that is frequently associated with carcinoma of the biliary tract. However, there is still no clear evidence that PBM is associated with pancreatic tumors. Here we describe a case of gallbladder cancer and intraductal papillary mucinous neoplasm (IPMN) that is associated with PBM. Case presentation A 72-year-old man underwent a cholecystectomy with hepatectomy (S4a + S5) and regional lymph node dissection for gallbladder adenocarcinoma invading the front lobe branch of the hepatic artery. A pylorus-preserving pancreaticodudenectomy was also performed for pancreatic IPMN. Clinical discussion Presence of mucin type 6 (MUC6) -positive pyloric gland metaplasia in both the dilated pancreatic duct and the gallbladder background mucosa suggests that pancreatic IPMN and gallbladder cancer may have a common phenotypic origin. Additionally, analysis of 41 reported cases of pancreatic cancer associated with PBM revealed that in all metachronous multiple cancer cases, biliary tract cancer preceded the pancreatic cancer with congenital biliary dilatation accompanied by PBM. The analysis also revealed an increased proportion of pancreatic cancer cases with PBM in patients who had not undergone a flow diversion procedure located in pancreatic head. Conclusion We show an interesting relationship between pancreatic/gallbladder cancer and PBM. More comprehensive evaluations of the whole pancreaticobiliary system in follow-up of patients with PBM is required to understand the full extent of this relationship., Highlights • Pyloric gland metaplasia seen both in the pancreatic duct and in gallbladder suggests that IPMN and gallbladder cancer may have a common origin. • The analysis of pancreatic cancer with PBM cases revealed that in all metachronous cases, biliary tract cancer preceded the pancreatic cancer. • This observation suggests that PBM patients should be monitored for synchronous or metachronous cancer of the whole pancreaticobiliary system.

The intussusception of the small bowel is rarely encountered in adult patients and is frequently associated with a lead point that is often malignant. In a 69-year-old female patient with an episode of gastrointestinal (GI) bleeding, computed tomography (CT) showed a duodenal-jejunal intussusception caused by an intraluminal mass. Open polypectomy and reduction of intussusception were performed and the diagnosis of gangliocytic paraganglioma was made at pathological evaluation. It would be important to consider neoplasms like gangliocytic paraganglioma in the setting of adult small bowel intussusception.

Background & Aims Low-phospholipid-associated cholelithiasis (LPAC) syndrome, a rare genetic form of intrahepatic cholelithiasis in adults, is still poorly understood. We report the results of the largest-ever case-control study of patients with LPAC syndrome aiming to assess the prevalence, clinical features, and comorbidities of the disease. Methods We included all LPAC cases diagnosed between 2001 and 2016 in 11 French centres. Controls consisted of all patients who underwent a cholecystectomy for common gallstone disease in a single non-academic centre over 1 year. A logistic regression analysis was used to identify the clinical features associated with LPAC syndrome across several patient strata with increasing levels of diagnostic confidence. The ratio between the incident cases of LPAC syndrome and the total number of cholecystectomies for gallstones was used to assess the relative prevalence of the disease. Results In this study, 308 cases and 206 controls were included. LPAC syndrome accounted for 0.5–1.9% of all patients admitted with symptomatic gallstone disease. Age at first symptoms, Graphical abstract, Highlights • Low-phospholipid-associated cholelithiasis (LPAC) syndrome affects approximately 1% of adults with symptomatic cholelithiasis. • Normal weight, common bile duct stones, and lack of cholecystitis are clinical features significantly associated with this syndrome. • ABCB4 variants in patients with LPAC may be associated with an increased personal or family risk of hepato-biliary cancer.

Background The biliary bronchial fistula is rare and difficult to treat. Here we report a 49-year-old woman diagnosed with biliary bronchial fistula due to cough with yellow-green sputum. Case presentation this is a typical case of the biliary bronchial fistula with typical symptoms. The position of the abscess cavity below the diaphragm could not be catheter drainage. After anti-infection treatment, yellow-green sputum was reduced. Follow-up showed a good prognosis. Conclusion biliary bronchial fistula is rare in the clinic, combined with chest and abdomen infection.

Author(s): Freise, Jonathan; Mena, Jorge; Wen, Kwun Wah; Stoller, Marshall; Ho, Sunita; Corvera, Carlos | Abstract: IntroductionHepatolithiasis (intrahepatic stones) is rare in adolescent patients and requires complex management strategies to prevent recurrent infections and progression to hepatic fibrosis. Surgical management is often required. In cases of unclear etiology, further work-up is indicated to provide insight into future management. In this report we describe an extensive stone analysis.Presentation of caseA 20-year-old Caucasian female presented with known hepatolithiasis and multiple prior recurrent bouts of abdominal pain requiring hospitalization. Magnetic resonance cholangiopancreatography (MRCP) demonstrated an abnormal left-sided hepatic biliary ductal system dilatation. She was treated surgically with a formal left hepatectomy and preservation of the caudate lobe. The right ductal system had no stones or evidence of inflammation, and her bile and stones cultures were negative for organism growth. An extensive analysis demonstrated stone composition primarily of cholesterol.DiscussionAdolescent presentations of hepatolithiasis are rare and considerations in the differential diagnosis include primary sclerosing cholangitis, bile acid transporter defects, and other known genetic diseases. This case is unique because only the left half of the intrahepatic ductal system had evidence of stone disease and the bile was sterile. A detailed stone analysis demonstrating cholesterol supersaturation provides additional context though the etiology remains unclear in this case and will require lifelong follow-up.ConclusionEarly-onset hepatolithiasis is rare and requires expert management, and in some cases definitive surgical management with life-long follow-up. Extensive stone analysis and genetic testing can be performed to help identify disease etiology in unique cases.

Highlights • Duplication of the gallbladder is a rare congenital biliary anomaly. • We report a previously undescribed variation, in which the gallbladder is attached over a wide area to the right hepatic duct. • Surgical management may be more complicated when the duplicate gallbladder is connected separately and more proximally in the biliary tree., Introduction Duplication of the gallbladder is a rare congenital biliary anomaly and may present with similar pathology to that seen in a single gallbladder. We present a previously unreported case of a symptomatic duplex gallbladder arising directly from a long segment of the right hepatic duct. Presentation A 23 years old female was referred to our team with right upper quadrant pain suggestive of biliary colic. Ultrasound, contrast enhanced CT and magnetic resonance cholangiopancreatography revealed a normal gallbladder and a separate cystic lesion containing multiple gallstones and communicating with the right main hepatic duct. Surgical management involved cholecystectomy, resection of the cystic lesion from the right hepatic duct and reconstruction with hepaticojejunostomy. The patient made a good recovery from surgery, reporting complete resolution of symptoms. Histology of the cystic lesion confirmed duplicate gallbladder with features of severe chronic cholecystitis. Discussion Symptomatic duplicate gallbladders warrant cholecystectomy and in more straightforward variations, in which a shared, single cystic duct is encountered, a laparoscopic approach is feasible. Surgical management may be more complicated for anomalies in which the duplicate gallbladder is connected separately and more proximally in the biliary tree. Conclusion Trabecular duplicate gallbladder, in which a second gallbladder originates from the right or left hepatic duct is extremely rare. We report a previously undescribed variation, in which the gallbladder is attached over a wide area to the right hepatic duct and outline the successful surgical management undertaken.

Highlights • Double cancers of the bile duct are often associated with pancreaticobiliary maljunction. • Double cancers of the bile duct without pancreaticobiliary maljunction are very rare. • Double cancers of the bile duct may have a terribly poor prognosis., Background Double cancers of the biliary tract system are rare. Most of these cancers are synchronous double cancers of the gall bladder and bile duct, associated with pancreaticobiliary maljunction (PBM). Synchronous double cancers of the extrahepatic bile duct without PBM are especially rare, and only 4 cases have been reported. Case presentation A 78-year-old woman was admitted to our hospital for examination of hyperbilirubinemia and liver dysfunction. Contrast-enhanced abdominal computed tomography, Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography revealed 2 stenotic regions in the common bile duct: at its junction with the cystic duct and in the distal bile duct. No findings suggested PBM, such as a markedly long common channel. The diagnosis based on endoscopic brush cytology from both stricture portions was adenocarcinoma. The patient had a pylorus-preserving pancreaticoduodenectomy with regional lymph node resection. Macroscopically, there were 2 stenotic regions at the cystic duct junction and in the distal bile duct. Microscopically, the tumor at the junction of the cystic duct was a well-to-moderately differentiated adenocarcinoma. On the other hand, the tumor of the distal bile duct was a poorly differentiated adenocarcinoma. There was no evidence of communication between these 2 cancers. Conclusion Double cancers of the extrahepatic bile duct without PBM are very rare. Therefore, an accurate diagnosis prior to surgery is necessary. Furthermore, this rare condition seems to be associated with a poor prognosis.

Highlights • Portal vein arterialization may be a bridging treatment for retransplantation. • This case demonstrates feasible of portal vein arterialization in sick patient. • Portal hypertension usually occurs after portal vein arterialization. • Calibration of fistula after recovery of liver allograft should be considered., Objective Hepatic artery thrombosis (HAT) is one of the most serious complications of liver transplantation that can potentially lead to loss of the allograft. Retransplantation is the only option when revascularization can’t be performed but the donor may be not available in the short period of time. We report the technique of using portal vein arterialization (PVA) for bridging before retransplantation. There are few reports in living donor setting. Case description The recipient of the liver was a 59 year old male who received an extended right lobe graft from his son. Post operative day 41, HAT was diagnosed from angiogram and liver function got rapidly worse. We decided to re-anastomose the hepatic artery but this was not possible due to a thrombosis in the distal right hepatic artery. So PVA by anastomosis of the common hepatic artery to splenic vein was performed. During the early postoperative period liver function gradually improved. Unfortunately, he died from massive GI hemorrhage one month later. Discussion PVA has previously been reported as being useful when revascularization was not successful. The surgical technique is not complicated and can be performed in sick patient. Liver graft may be salvaged with oxygenated portal flow and recover afterwards. However, portal hypertension after PVA seem to be an inevitable complication. Conclusions PVA may be a bridging treatment for retransplantation in patients whom hepatic artery reconstruction is impossible after HAT. Regards to the high morbidity after procedure, retransplantation should be performed as definite treatment as soon as possible.

Highlights • Total laparoscopic living donor right hepatectomy is rarely reported worldwide. • Right liver transection plane used to be determined through ischemic demarcation and IOUS. • The site of bile duct division was determined according to MRCP or intraoperative cholangiography. • ICG fluorescence can real-timely visualize the surgical margin and biliary branches of right lobe. • ICG fluorescence navigation makes the procedure simplified, safer and more accurate., Introduction Total laparoscopic living donor right hepatectomy (TLDRH) is sporadically reported worldwide. Liver transection margin used to be determined by ischemic demarcation or intraoperative ultrasonography. To identify the site of bile duct division relied on preoperative MRCP and intraoperative cholangiography, which is experience demanding. Presentation of case A 34-year-old man volunteered for living donation to his brother who suffered decompensated HBV-related cirrhosis. Right lobe donation without MHV fulfilled the volumetric criteria. After hilum dissection, ICG was injected into the right portal branch. Right lobe was transected tracing the real-time fluorescence-enhanced borderline and the course of MHV. The right bile duct was transected above the bifurcation that was fluorescently visualized within the parenchyma. The liver graft was retrieved from a pre-made suprapubic incision after simple vascular clamping. The warm ischemia time was 6 min. The recipient procedure was successful with back-table graft venoplasty using cryopreserved iliac artery allografts. The donor recovered uneventfully and was discharged from hospital on POD 7. Discussion The operative time, blood loss and postoperative course of donor is comparable to those undergoing ordinary laparoscopic right hepatectomy in our institute. ICG fluorescence can real-timely visualize the surgical margin and biliary branches of right lobe, which helps preserve every last bit of functional liver volume for the donor and avoid the complicated traditional intraoperative cholangiography. Conclusion TLDRH proves to be achievable in surgical teams confortable with both laparoscopic hepatectomy and LDLT. ICG fluorescence navigation could make the procedure simplified, safer and more accurate. More practice and technical modification are necessary.

Introduction Horseshoe kidneys are the most common fusion defect of the kidneys, which amounts to about 0.25% of the population. They are usually asymptomatic and are often identified incidentally. The horseshoe kidney can push the second and third part of the duodenum anteriorly, leading to an altered CBD course. Choledocholithiasis is seen in approximately 10‐15% of patients with cholelithiasis. Presently, the most preferred approach for managing CBD stones is ERCP. However, in ERCP failure cases, Laparoscopic CBD exploration is the primary treatment modality, with or without T-tube use, with all the advantages of minimally invasive surgery. Case presentation and discussion A 65-year-old female presented with complaints of pain in the right hypochondriac region for three months associated with nausea, jaundice, and loss of appetite and weight. Her USG abdomen showed cholelithiasis with dilated CBD with horseshoe kidney with severe hydronephrosis of the left kidney. They are usually asymptomatic and are often identified incidentally. In this patient, it was believed that the horseshoe kidney had pushed the second and third part of the duodenum anteriorly, leading to an altered CBD course leading to ERCP failure. MRCP confirmed cholelithiasis with choledocholithiasis with dilated CBD of 11.3 mm with horseshoe kidney. ERCP was attempted but was unsuccessful due to non-visualization of the papilla due to overcrowding of duodenal folds. For patients with ERCP failure, laparoscopic CBD exploration is mandatory. For this patient, the CBD was cannulated with a guidewire, if needed, for repeat ERCP and was closed with T-tube in situ. Conclusion There are no particular preoperative indicators that can predict the failure of ERCP. However, in ERCP failure cases, laparoscopic CBD exploration (with or without T-tube use) is the primary treatment modality., Highlights • Horseshoe kidney causing altered CBD course leading to ERCP failure • Laparoscopic CBD exploration in patients with ERCP failure • Cannulation of CBD with guidewire for easier identification of papilla in case of repeat ERCP • One step laparoscopic cholecystectomy with laparoscopic CBD exploration vs. ERCP • Laparoscopic CBD exploration with T-tube insertion

Highlights • Pancreatic schwannoma is rare and preoperative diagnosis is difficult due to the lack of specific symptoms, radiological characteristics and tumor markers. • Incidental detection of pancreatic schwannoma is predicted to increase due to the widespread use of CT and MRI. It is important to take into account this tumor in the differential diagnosis of pancreatic tumors. • We should be aware that FDG-PET/CT shows abnormal accumulation in a benign pancreatic schwannoma., Introduction Schwannomas are neoplasms originating from Schwann cells in nerve sheath, but pancreatic schwannomas are rare. Presentation of case A 59-year-old female incidentally pointed out a 2-cm-sized solid mass in the pancreas head by computed tomography (CT) which was performed for other reasons. Magnetic resonance imaging (MRI) showed a mass with hypointensity on T1-weighted images and a heterogenous hyperintensity on T2-weighted images. Magnetic resonance cholangiopancreatography (MRCP) showed no abnormal findings in the main pancreatic duct. The mass of the pancreas head showed a significantly increased uptake on fluorin-18 fluorodeoxyglucose positron emission tomography CT (FDG-PET/CT). Based on the radiological findings, a malignant potential tumor was preliminarily considered. Subtotal stomach-preserving pancreatoduodenectomy (SSSPD) was performed to make a definite diagnosis. The resected specimen showed a well-encapsulated circumscribed mass, measuring 2.0 cm, in the pancreas head. Histopathological examination revealed proliferation of spindle cells showing interlacing and palisading patterns in the tumor. The proliferating cells showed no atypia and few mitoses. Immunohistochemically, the spindle cells were positive for S-100 protein. The Ki67 labeling index was approximately 2%. Based on these findings, a diagnosis of benign schwannoma of the pancreas was made. Conclusion Although pancreatic schwannoma is a rare neoplasm, it is important to take into account this tumor in the differential diagnosis of pancreatic tumors. In addition, we should be aware that FDG-PET/CT shows abnormal accumulation in a benign pancreatic schwannoma.

Highlights • Small SPN of the pancreas is sometimes difficult to diagnose preoperatively and mimicking pancreatic cancer. • The clinical presentation of SPNs is nonspecific, but acute pancreatitis is rare in the reported literature. • We should be aware that SPN of the pancreas can become a cause of acute pancreatitis., Introduction A solid pseudopapillary neoplasm (SPN) of the pancreas is rare neoplasm that occurs predominantly in young women. The clinical presentation of SPNs is nonspecific, but acute pancreatitis is rare in the reported literature. Presentation of case A 36-year-old man was referred to our hospital because of upper abdominal pain and elevation of serum amylase. A computed tomography (CT) scan showed swelling of the pancreas body and a poorly enhanced and indistinct mass in the pancreas body. He was diagnosed with acute pancreatitis. The symptom was improved with conservative treatment, but acute pancreatitis recurred twice during a period of 2 months. Magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERCP) showed stenosis of the MPD adjacent to the mass. Distal pancreatectomy was performed because the mass in the pancreas body seemed to cause repeated acute pancreatitis and malignant pancreatic cancer could not be excluded. Immunohistochemically, a diagnosis of SPN of the pancreas was made from the resected specimen. Discussion To the best our knowledge, only 6 cases have been reported in the literature concerning the SPN presenting with acute pancreatitis. Conclusion We report a man with a small SPN of the pancreas presenting with acute pancreatitis and mimicking pancreatic cancer. We should be aware that this rare pancreatic tumor can become a cause of acute pancreatitis.

Highlights • Amputation neuroma (AN) arising from a remnant cystic duct after cholecystectomy is rare. • It is difficult to distinguish AN and malignant tumor because radiological findings of ANs mimic findings of malignancy. • Intraoperative diagnosis is necessary to select an appropriate surgical procedure., Introduction Amputation neuroma is difficult to diagnose preoperatively. Amputation neuroma arising from a remnant cystic duct after cholecystectomy is rare. Herein, we present a case of amputation neuroma derived from a remnant cystic duct along with a review of the literature. Presentaion of the case A 60-year-old woman visited our hospital due to a tumor located in the hepatoduodenal ligament. A gallbladder adenoma was resected by open cholecystectomy 30 years prior. Endoscopic ultrasonography demonstrated branched intraductal papillary mucinous neoplasm of the pancreas and a tumor with a low-echoic pattern in the extrahepatic biliary system. Enhanced computed tomography revealed a 6-mm tumor in the artery phase. Surrounding lymph nodes were not swollen. Magnetic resonance cholangiopancreatography showed that the tumor presented with slightly high intensity on T2 weighted imaging. Operative findings revealed that the whitish nodule was moderately attached to surrounding tissues. The remnant cystic duct and the tumor could not be separated; however, no direct invasion toward common bile duct was observed. Rapid intraoperative pathological examination demonstrated that the tumor was a neuroma. The peration time was 251 min and blood loss was 80 ml. The patient was discharged nine days after surgery with no postoperative complications. Conclusion It is difficult to distinguish amputation neuroma from malignant tumors because radiological findings of a neuroma mimic findings of malignancy. Intraoperative diagnosis is necessary to select an appropriate surgical procedure due to the difficulty of preoperative diagnosis.

Highlights • Aberrant subvesical bile duct poses risk of bile duct injury during cholecystectomy. • Intraoperative fluorescent cholangiography (IFC) could allow us to identify a fine minute bile duct. • IFC is one of a promising technique to improve the safety of cholecystectomy further., Introduction Aberrant subvesical bile ducts are rare structural anomaly located in the peri-hepatic gallbladder fossa. This duct poses the risk for intraoperative bile duct injury resulting in clinically relevant bile leakage. Presentation of case Aberrant subvesical bile duct was detected by preoperative magnetic resonance cholangiopancreatography in a 52-year old woman with gallbladder polypoid tumor harboring the risk to be gallbladder cancer. During open cholecystectomy with full thickness dissection, the aberrant duct was identified by intraoperative fluorescent cholangiography (IFC), and dissected safely. Discussion Aberrant subvesical bile ducts are mostly found unexpectedly as intra and/or postoperative bile leakage, and remain an important cause of bile duct injuries after laparoscopic cholecystectomy. IFC, which offers real-time imaging of biliary anatomy, has a potential to overcome these problems. Conclusion We performed cholecystectomy by using IFC to identify the aberrant subvesical bile duct. To the best of our knowledge, this is the first report showing the fluorescence image of an aberrant subvesical bile duct in a state of nature.

Highlights • Laparoscopic subtotal cholecystectomy was successfully performed for a patient. • The rates of bile duct injuries is high after laparoscopic cholecystectomy. • Removal of the stone is the main purpose of treatment. • Laparoscopic subtotal cholecystectomy is useful for patients with Mirizzi syndrome., Introduction Mirizzi syndrome is a rare complication of gallstone disease. The purpose of this report is to describe the utility of laparoscopic subtotal cholecystectomy for Mirizzi syndrome. Presentation of case A 53-year-old female presented with dark urine and right upper quadrant pain. Blood tests revealed elevated liver and biliary enzyme levels. Magnetic resonance cholangiopancreatography showed a narrowed common hepatic duct compressed by a large gallstone, consistent with Mirizzi syndrome. Semi-urgent laparoscopic cholecystectomy was planned. At operation, circumferential dissection of the gallbladder neck was difficult. The fundus of the gallbladder was opened and a 2 cm stone extracted. The gallbladder neck was sutured and a drain placed. The postoperative clinical course was uneventful. Discussion After laparoscopic cholecystectomy in patients with Mirizzi syndrome, complication rates, including bile duct injuries, is high. In patients with Mirizzi syndrome, removal of the responsible stone is the main purpose of treatment. Conclusion Laparoscopic subtotal cholecystectomy is a useful technique for patients with Mirizzi syndrome to avoid bile duct injury.

Highlights • Choledochal cyst is a rare malformation of the common bile duct causing jaundice in the pediatric group. • Diagnosis can easily be made using abdominal ultrasonography, hence a high degree of suspicion is needed among clinicians. • Surgery, open or laparoscopic, remains the core management depending upon the availability of the resources and expertise., Introduction Choledochal cyst is a rare common bile duct malformation that usually presents in the pediatric group with abdominal pain, distension, and jaundice. The pathophysiology remains unclear for the cause, and surgery aims to restore biliary enteric drainage. Case presentation We present a six-year-old female who presented with gradual abdominal distention associated with jaundice. Abdominal ultrasound was suggestive of choledochal cyst, and CT-scan confirmed the diagnosis. She was operated on successfully and fared well. Conclusion Choledochal cysts are a rare entity of common bile duct malformations and should be considered as a differential diagnosis in the pediatric age group. Diagnosis can be easily made by non-invasive and in-expensive radiologic modalities like ultrasonography in resource-limited settings.

Annular pancreas (AP) in adults is a rare embryologic abnormality detected after development of complications or as incidental finding. Diagnosis and treatment strategies for symptomatic adult AP remain controversial. We describe two different presentation of AP: a woman with a specific upper abdominal pain treated with medical therapy and a man with upper gastroenteric obstructive symptoms which underwent surgical duodeno-jejunal by-pass. English language literature about annular pancreas etiology, diagnosis and treatment was reviewed. No specific guidelines and protocols exist about management of AP, therefore, treatment and operative approaches must be individualized. In consideration of the possible post-operative complications, surgical treatment should be reserved in case of failure of conservative medical therapies.

Highlights • Intraductal tubulopapillary neoplasms (ITPNs) is a rare malignant lesion. • The patient was diagnosed as causing acute pancreatitis by ITPN. • There are only 5 cases reported about ITPNs presenting as acute pancreatitis. • It is important to diagnose that the cause of acute pancreatitis is ITPN., Introduction The 2010 World Health Organization classification of intraductal neoplasms of the pancreas includes intraductal tubulopapillary neoplasms (ITPNs) and intraductal papillary mucinous neoplasms, the latter being a rare and new concept. ITPN sometimes cause acute pancreatitis; therefore, distinguishing ITPN from idiopathic acute pancreatitis is important but challenging. Presentation of case We present the case of a 72-year-old male who had recurrent pancreatitis for the past 2 years, his diagnosis was idiopathic acute pancreatitis. He was admitted to our hospital with severe acute pancreatitis and cholangitis due to intrapancreatic bile duct stenosis. After the treatment of cholangitis, contrast-enhanced computed tomography revealed a tumor at the pancreatic head. Endoscopic retrograde cholangiopancreatography (ERCP) showed stenosis of the main pancreatic duct and distal bile duct, and adenocarcinoma was detected using brush cytology of the bile duct stricture and pancreatic juice. The patient was diagnosed with invasive ductal carcinoma and pancreaticoduodenectomy was performed. Histopathological findings revealed dilation of the pancreatic duct, and proliferation of columnar cells and cuboid epithelial cells in the main pancreatic duct of the pancreatic head. Mucus production was poor, and immunostaining results revealed ITPN. The patient is alive and do not exhibit signs of recurrence for 12 months. Discussion ITPNs can cause acute pancreatitis, which can be challenging to preoperatively diagnose. ITPNs presenting as acute pancreatitis are rare, with reported only 5 cases. Conclusion It is important to be keep in mind that there is a possibility of ITPN after diagnosis of idiopathic acute pancreatitis.