Remnant Bile Duct Research Articles

Clonal analysis of metachronous double biliary tract cancers.

The molecular mechanisms underpinning the development of metachronous tumors in the remnant bile duct following surgical resection of primary biliary tract carcinomas (BTCs) are unknown. This study aimed to elucidate these mechanisms by evaluating the clinicopathologic features of BTCs, the alterations to 31 BTC-related genes on targeted sequencing, and the aberrant expression of p53, p16, SMAD4, ARID1A and β-catenin on immunohistochemistry. Twelve consecutive patients who underwent resection of metachronous BTCs following primary BTC resection with negative bile duct margins were enrolled. Among the 12 metachronous tumors, six exhibited anterograde growth in the lower portion and six exhibited retrograde growth in the upper portion of the biliary tree. Surgical resection of metachronous BTCs resulted in recurrence-free survival in seven, local recurrence in five, and death in two patients. Nine achieved 5-year overall survival after primary surgery. Molecular analyses revealed that recurrently altered genes were: TP53, SMAD4, CDKN2A, ELF3, ARID1A, GNAS, NF1, STK11, RNF43, KMT2D and ERBB3. Each of these was altered in at least three cases. A comparison of the molecular features between 12 paired primary and metachronous BTCs indicated that 10 (83%) metachronous tumors developed in clonal association with corresponding primary tumors either successionally or phylogenically. The remaining two (17%) developed distinctly. The successional tumors consisted of direct or evolved primary tumor clones that spread along the bile duct. The phylogenic tumors consisted of genetically unstable clones and conferred a poor prognosis. Metachronous tumors distinct from their primaries harbored fewer mutations than successional and phylogenic tumors. In conclusion, over 80% of metachronous BTCs that develop following primary BTC resection are probably molecularly associated with their primaries in either a successional or a phylogenetic manner. Comparison between the molecular features of a metachronous tumor and those of a preceding tumor may provide effective therapeutic clues for the treatment of metachronous BTC. © 2024 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of The Pathological Society of Great Britain and Ireland.

Single-operator cholangioscopy monitoring of a remaining bile duct after congenital choledochal cyst surgery: a case report with an innovative approach.

Video 1Single-operator cholangioscopy monitoring of a remaining bile duct after congenital choledochal cyst surgery: a case report with an innovative approach. After removing remaining pancreatic stones in the remnant bile duct, we inserted a Spyglass choledochoscope. Mucosa was regular and monochromatic. We performed biopsies with a Spybite miniforceps in the remnant cyst and its junction.

Laparoscopic definitive surgery for congenital biliary dilatation with aggressive hilar bile ductoplasty and complete resection of the intrapancreatic bile duct in pediatric patients is safe and effective, comparable to open surgery.

After surgery for congenital biliary dilatation (CBD), hilar and hepatic bile duct stenosis and intrapancreatic bile duct remnants can cause many postoperative complications. We investigated the efficacy of laparoscopic surgery with aggressive bile ductoplasty and complete excision of the intrapancreatic bile duct in CBD patients compared to open surgery. Pediatric patients who underwent surgery for CBD at our institution between 2006 and 2020 were divided into two groups: laparoscopic surgery (Lap group) and open surgery (Op group). Surgical outcomes were compared between the two groups. We enrolled 160 patients (85 in the Lap group and 75 in the Op group). Hilar bile ductoplasty was performed in 52 (61%) patients from the Lap group and 40 (53%) patients from the Op group. The operative time was significantly longer, the amount of blood loss was significantly lower, and the length of hospital stay was significantly shorter in the Lap group than in the Op group. Postoperative early and late complications occurred at a similarly low rate in both groups. When the surgical outcomes were compared between the group who underwent laparoscopic bile ductoplasty and the group that did not, postoperative early and late complications occurred similarly in both groups. Laparoscopic surgery for CBD with aggressive hilar bile ductoplasty and complete excision of the intrapancreatic bile duct is safe and effective, comparable to open surgery. However, it is necessary to verify whether long-term occurrence of hepatolithiasis similar to open definitive surgery can be obtained in the future.

A case with massive hemobilia long-term after internal drainage surgery for congenital biliary dilation

BackgroundCurrently, there is an unwavering consensus that the standard surgery for congenital biliary dilation (CBD) is extrahepatic bile duct resection and choledochojejunostomy. However, decades prior, choledochocyst–gastrointestinal anastomosis without extrahepatic bile duct resection (internal drainage surgery, IDS) was preferred for CBD because of its simplicity. Currently, there is almost no chance of a surgeon encountering a patient who has undergone old-fashioned IDS, which has been completely obsolete due to the risk of carcinogenesis from the remaining bile duct. Moreover, the pathological condition long after IDS is unclear. Herein, we report a case of life-threatening bile duct bleeding as well as carcinoma of the bile duct 62 years after IDS in a patient with CBD.Case presentationAn 82-year-old Japanese woman with hemorrhagic shock due to gastrointestinal bleeding was transferred to our hospital. She had a medical history of unspecified surgery for CBD at the age of 20. Based on imaging findings and an understanding of the historical transition of the surgical procedure for CBD, the cause of gastrointestinal bleeding was determined to be rupture of the pseudoaneurysm of the dilated bile duct that remained after IDS. Hemostasis was successfully performed by transcatheter arterial embolization (TAE) in an emergency setting. Then, elective surgery for extrahepatic bile duct resection and choledochojejunostomy was performed to prevent rebleeding. Pathological examination revealed severely and chronically inflamed mucosa of the bile duct. Additionally, cholangiocarcinoma (Tis, N0, M0, pStage 0) was incidentally revealed.ConclusionIt has been indicated that not only carcinogenesis, but also a risk of life-threatening bleeding exists due to long-lasting chronic inflammation to the remnant bile duct after IDS for CBD. Additionally, both knowledge of which CBD operation was performed, and an accurate clinical history are important for the diagnosis of hemobilia.

Long-term outcome of 18 years single-center study of surgical treatment of choledochal cyst patients

There are not many reports of postoperative long-term malignant risk or postoperative sequelae after surgery on choledochal cyst (CC). The purpose of this study was to report the long-term results of operated CC. The patients who underwent surgical treatments for CC between 2003 and 2020 at the Seoul National University Hospital were enrolled. Clinicopathologic factors and pre/postoperative CT or MRI were reviewed. Of the 153 patients, the female was 85%, and the mean age was 41 years old. The most common symptom was abdominal pain. According to the Todani classification, the most common type was 1c (36.6%). APBDU was 85.6% and C-P type, P-C type APBDU were observed at 43.8%, 41.8% respectively. Fourteen patients (9.2%) had biliary tract cancer including 8 cholangiocarcinomas and 8 gallbladder cancer. Two patients had both cancers. A comparison of patients with and without malignancy showed that age, operative time, EBL were significantly higher in the malignant group, while the diameter of the cyst is significantly smaller in the malignant group. There was no significant difference in APBDU type and Todani classification type between the two groups. The incidence of long-term complication was 14.3% and the median time interval was 30 months. The two most common complication was cholangitis or stricture (5.9%). There were 2 cases (1.3%) of new cancer near the intrapancreatic remnant bile duct. The 9.2% of resected CCs had combined malignancy on biliary tracts. Long-term complications such as cholangitis, anastomosis stricture, and new cancer may occur. Therefore continuous surveillance is required.

Postoperative surveillance of bile duct cysts through modified “Roux en Y” hepaticojejunostomy by transgastric endoscopic access

Presenter: André de Moricz MD | Irmandade Santa Casa de Misericórdia de São Paulo Background: Choledochal cyst (CC) is a congenital dilatation of extra or intrahepatic bile ducts and is usually associated with pancreaticobiliary malformation, risk of choledocholithiasis, and biliary tract cancer. The standard treatment for CC consists of complete cystic resection with biliodigestive anastomosis; the hepaticojejunostomy is the preferred type of reconstruction due to lower complication rates. Methods: A survey of all choledochal cyst cases operated by the Pancreas group of our service was carried out from July 1984 to March 2020, and 22 patients who underwent surgical treatment were identified. Based on the need for subsequent access to biliodigestive anastomosis for intrahepatic lithiasis treatment, biliary stricture, and further cancer surveillance on remnant intrahepatic cysts, a modified “Roux-en-Y” was proposed hepaticojejunostomy providing surveillance of remnant choledochal cyst and treatment for postoperative hepaticojejunostomy complications by less invasive strategy. Results: Considering the possibility of late complications such as stenosis or incomplete resection of the cyst, a surveillance strategy must be considered. All the current procedures depend on advanced radiology, generate discomfort to the patient in ostomy cases, or rely on more invasive and repeated biliary surgical procedures. The new technique proposes a jejunal segment of approximately 10,0 to 15,0 cm kept far from the anastomosis allowing its placement, without tension, in the anterior gastric wall below the small curvature between the body the stomach antrum, which enables access to the bile ducts through Endoscopic Retrograde Cholangiopancreatography (ERCP) guided by Endoscopic Ultrasound (EUS) and therefore less invasive access than the ones described in the literature. Conclusion: The modified “Roux en Y” hepaticojejunostomy is a new and straightforward technique that allows surveillance of patients submitted to the biliodigestive anastomosis of any kind and are useful in patients submitted to choledochal cyst resection once it enables vigilance of the remnant bile duct and makes interventions possible when needed.

Hemobilia after bile duct resection: perforation of pseudoaneurysm into intra-pancreatic remnant bile duct: a case report

BackgroundHemobilia occurs mainly due to iatrogenic factors such as impairment of the right hepatic or cystic artery, and/or common bile duct in hepatobiliary-pancreatic surgery. However, little or no cases with hemobilia from the intra-pancreatic remnant bile duct after bile duct resection (BDR) has been reported. Here, we report a case of massive hemobilia due to the perforation of psuedoaneurysm of the gastroduodenal artery (GDA) to the intra-pancreatic remnant bile duct after hepatectomy with BDR.Case presentationA 68-year-old male underwent extended right hepatectomy with BDR for gallbladder carcinoma. He presented with upper gastrointestinal bleeding 2 months after the initial surgery. Upper endoscopy identified a blood clot from the ampulla of Vater and simultaneous endoscopic balloon tamponade contributed to temporary hemostasis. Abdominal CT and angiography revealed a perforation of the psuedoaneurysm of the GDA to the intra-pancreatic remnant bile duct resulting in massive hemobilia. Subsequent selective embolization of the pseudoaneurysm with micro-coils could achieve complete hemostasis. He survived without any recurrence of cancer and bleeding.ConclusionHemobilia could occur in a patient with BDR due to perforation of the pseudoaneurysm derived from the GDA to the intra-pancreatic remnant bile duct. Endoscopic balloon tamponade was useful for a temporal hemostasis and a subsequent radiologic interventional approach.

Cystic duct patch closure of remnant bile duct in living donor hepatectomy when primary closure is difficult: An easy solution.

Backgrounds/AimsIn living donor hepatectomy, hepatic duct division is a crucial step and often a technical challenge, with the aim of obtaining a good hepatic duct for anastomosis in the recipient and an adequate stump in the donor for closure. Very rarely, after duct division, the remaining stump may not be adequate for primary closure. In such a difficult situation, the options would be either to close stump transversely or a Roux-en-Y Hepaticojejunostomy.MethodsWe describe a novel surgical technique of “Cystic duct patch repair”, utilizing the available local tissues for closure of bile duct wall.ResultsTwo year follow up of this technique showed satisfactory results with no evidence of stricture and normal liver functions.ConclusionsIn living donor hepatectomy, “Cystic duct patch closure” may be used if the post closure cholangiogram is not satisfactory. Although the best method is prevention by ensuring a stump for closure, very rarely this error can occur and can be sorted by cystic duct patch repair.

Intraductal Papillary Neoplasm of the Bile Duct Asynchronously Recurred at the Remnant Bile Duct 3 Years after Operation for Hilar Cholangiocarcinoma

Intraductal Papillary Neoplasm of the Bile Duct Asynchronously Recurred at the Remnant Bile Duct 3 Years after Operation for Hilar Cholangiocarcinoma

Post Cholecystectomy Stump Calculus Cholecystitis with Completion Cholecystectomy –A Rare Case Report

Cholecystectomy is the most common method of choice for treating symptomatic gall stones. It can either be performed laproscopically or by open cholecystectomy. Partial cholecystectomy is usually done whenever there is difficulty in performing total cholecystectomy due to various reasons such as acute attack with adhesions, fibrosis, in view of preventing bile duct and vascular injuries, variations in anatomy which lead to bile duct remnant which can cause post cholecystectomy syndrome. After a partial or subtotal cholecystectomy, symptoms may recur from pathology in the gallbladder remnant. When this occurs, a completion cholecystectomy is required as treatment of choice. We here report a case of a patient with stump cholecystitis in a gallbladder remnant to demonstrate open completion cholecystectomy.

Laparoscopic transcystic common bile duct exploration: surgical indications and procedure strategies

LTCBDE combined with or without modified techniques is safe and efficacious for the management of gallstones and concomitant, even large, common bile duct (CBD) stones. To describe the surgical indications and procedure strategies of laparoscopic transcystic common bile duct exploration (LTCBDE), a retrospective analysis of 205 patients with concomitant gallstones and CBD stones treated using LTCBDE between June 2008 and June 2015 was performed. Clinical data on disease characteristics, methods for cystic duct incision and CBD stone extraction (with or without laser lithotripsy), and surgical outcomes were collected and reviewed. CBD stones were successfully cleared in all patients. No patient was converted to choledochotomy or laparotomy. The cystic duct diameter ranged 3-8mm, and 85 patients with cystic duct diameter ≥ 5mm. The mean time for CBD stone extraction was 25.3min, with the operative time ranged from 63 to 170min. Lithotripsy was used in 74 (36.1%) patients among which 26 patients with cystic duct diameter ≥ 5mm. Estimated blood loss during surgery was 10-120ml per patient, and no intra-operative blood transfusions were needed. The mean postoperative hospital stay was 5.1 (range 3-7) days, and postoperative complications developed in seven patients. No bile duct injury, stricture, remnant, recurrent stones, or other adverse events were observed during the mean follow-up of 8 months. Based on preoperative MRCP and intra-operative IOC findings about cystic duct diameter, the diameter of CBD, CBD stone size, we summarized and proposed the surgical indications and suitable techniques and strategies during LTCBDE.

Long-Term Follow-Up of Patients with Primary Sclerosing Cholangitis Undergoing Liver Transplantation with Duct-to-Duct Biliary

Background Reconstruction of biliary drainage after liver transplantation (LTx) in patients with primary sclerosing cholangitis (PSC) has been a matter of controversy. Over the recent years, the traditional method of Roux-en-Y hepaticojejunostomy (RY) has been challenged by duct-to-duct (DD) biliary reconstruction. The argument against DD reconstruction has been for potential increased risk of development of cholangiocarcinoma (CCA) in the bile duct remnant. Methods This is a retrospective review of biliary complications, graft and patient survival after LTx in PSC patients based on type of biliary reconstruction DD vs. RY. Results A total of 120 LTx for PSC were performed between 2005 and 2016. Twenty two patients were excluded because they received partial grafts. DD was done in 39 patients and 59 patients were reconstructed with RY. One-, 5-, and 10-year survival was similar between the two groups. Bile leak and biliary stricture was not significantly different between the 2 groups. Nine patients in DD group (23%), and 13 patients in RY group developed biliary strictures and all managed endoscopically or percutaneously. There was one case of anastomotic leak in each group. There was no case of CCA observed in these patients over the long period of follow-up. Conclusion DD biliary reconstruction at the time of LTx in selected PSC patients is both effective and safe, with comparable outcomes with those with RY reconstruction and NO increased risk for development of CCA in the bile duct remnant.

Surgical technique and results of intrapancreatic bile duct resection for hilar malignancy (with video)

BackgroundHilar malignancy can occasionally be associated with high grade dysplasia (HGD) adjacent to invasive malignancy. For patients with HGD extending into the intrapancreatic bile duct, the authors adopted intrapancreatic bile duct resection (IP-BDR). The aims of this study were to compare the incidence of clinically relevant postoperative pancreatic fistula (CR-POPF), distal R0 resection and local recurrence within the distal bile duct remnant for patients undergoing extrahepatic bile duct resection without pancreaticoduodenectomy (with or without IP-BDR). MethodsPatients who presented with hilar malignancy and underwent extrahepatic bile duct resection without pancreaticoduodenectomy between January 2005 and December 2016 were identified and the outcomes retrospectively evaluated. ResultsOf 217 patients who met the inclusion criteria 62 (29%) patients underwent IP-BDR. There was a significant difference between patients undergoing standard resection vs. IP-BDR in terms of CR-POPF (5% (8/155) patients: vs 18% (11/62), p < 0.001). There were no significant differences between two groups of R0 status on distal margin (5% (8/155) patients: vs 10% (6/62), p = 0.359). No patient developed recurrence within the residual intrapancreatic bile duct. DiscussionThe incidence of CR-POPF after IP-BDR for hilar malignancies was 18%. IP-BDR was associated with CR-POF, but does not appear to alter survival or local recurrence rate.

Expanding Role of Radiofrequency Ablation in the Treatment of Intraductal Papillary Neoplasms of the Bile Duct (IPNB)

Introduction: Intraductal papillary neoplasms of the bile duct (IPNB) are relatively rare neoplasms with high malignancy potential. Total surgical resection has been the main curative treatment, but is many times limited by the location and extent of the lesion. Endoscopic retrograde cholangiopancreatography (ERCP) guided radiofrequency ablation (RFA) is a newly immerging therapy primarily used in palliation of pancreaticobiliary malignancies. We present a case using ERCP-guided RFA as an adjuvant curative treatment after incomplete surgical resection of cholangiocarcinoma arising from an IPNB. Case: 59 year old male with no significant past medical history presented forpainless jaundice. Initial bloodwork was consistent with obstructive jaundice, and MRI of abdomen demonstrated marked intrahepatic ductal dilation to the portal confluence. ERCP with Spyglass was performed. Cholangiography showed a single diffuse stenosis 20 mm in length in common hepatic duct, correlating with a frond-like/villous mass in the upper third of the bile duct seen with direct visualization and biopsied. Pathology returned as IPNB with high grade dysplasia (HGD). He underwent an exploratory laparotomy with bile duct resection, but negative margins could not be achieved. Surgical pathology demonstrated cholangiocarcinoma arising from an IPNB, without lymphovascular invasion. HGD was still present at the margin of the intra-pancreatic common bile duct (CBD) remnant. Repeat ERCP was performed one month later. ERCP-guided RFA was successfully performed on the intra-pancreatic CBD remnant, and repeat cholangioscopy demonstrating circumferential whitish mucosa indicative of ablated mucosa. The patient recovered well with initiation of chemotherapy and radiation planned in the future.Figure: IPNB seen on cholangiogram as 2cm stricture.Discussion: IPNB is a term encompassing a large spectrum of neoplasms affecting the biliary system, first categorized by WHO in 2010. Multiple histological subtypes exist, but in general carry a high risk of malignancy. Approximately 75% of IPNBs have features of invasive carcinoma, and pathology within a single IPNB lesion is usually heterogeneous warranting treatment. Ideally total surgical resection should be performed, but as this case demonstrates, may not always be possible. The role of ERCP-guided RFA for the treatment of pancreaticobiliary neoplasms is evolving. Here we demonstrate its utility as an adjuvant therapy in the curative treatment of an IPNB.Figure: Cholangioscopy of hepatic duct mass.Figure: Cholangioscopy after RFA.

Recurrence Rate and Pattern of Perihilar Cholangiocarcinoma after Curative Intent Resection

The aim of this study was to investigate the rate and pattern of recurrence after curative intent resection of perihilar cholangiocarcinoma (PHC). Patients were included from 2 prospectively maintained databases. Recurrences were categorized by site. Time to recurrence and recurrence-free survival (RFS) were estimated using the Kaplan-Meier method. Cox proportional hazards modeling was used to identify independent poor prognostic factors. Between 1991 and 2012, 306 consecutive patients met inclusion criteria. Median overall survival was 40 months. A recurrence was diagnosed in 177 patients (58%). An initial local recurrence was found in 26% of patients: liver hilum (11%), hepaticojejunostomy (8%), liver resection margin (8%), or distal bile duct remnant (2%). An initial distant recurrence was observed in 40% of patients: retroperitoneal lymph nodes (14%), intrahepatic away from the resection margin (13%), peritoneum (12%), and lungs (8%). Only 18% of patients had an isolated initial local recurrence. The estimated overall recurrence rate was 76% at 8 years. After a recurrence-free period of 5 years, 28% of patients developed a recurrence in the next 3 years. Median RFS was 26 months. Independent prognostic factors for RFS were resection margin, lymph node status, and tumor differentiation. Only node-positive PHC precluded RFS beyond 7 years. Perihilar cholangiocarcinoma will recur in most patients (76%) after resection, emphasizing the need for better adjuvant strategies. The high recurrence rate of up to 8 years justifies prolonged surveillance. Only patients with an isolated initial local recurrence (18%) may have benefited from a more extensive resection or liver transplantation. Node-positive PHC appears incurable.

Is Pancreaticoduodenectomy Feasible for Recurrent Remnant Bile Duct Cancer Following Bile Duct Segmental Resection?

BackgroundWhen bile duct cancer recurs after surgery, treatment options are limited. This study examines the usefulness of reoperative pancreaticoduodenectomy for recurrent cancer after initial segmental bile duct resection. MethodsSix patients (5 males, 1 females; median age 65 years) who underwent pancreaticoduodenectomy for recurrent remnant bile duct cancer following segmental bile duct resection were included: 4 underwent surgery at Severance Hospital and 2 at Gangnam Severance Hospital from January 2000 to December 2013. Medical records data were retrospectively reviewed, including demographics, type of first and second surgery, radicality of resection, TNM stage, adjuvant treatments, complications, and survival. Kaplan-Meier curves were used to analyze survival. ResultsThe median interval between operations was 57 (range 7–95) months. Median operation time was 6.9 (range 5.2–12.8) h, blood loss was 400 (range 50–1170) mL, intensive care unit stay was 1 (range 1–2) day, and postoperative hospital stay was 33 (range 15–55) days. No patient died. Four had severe complications. The median survival after pancreaticoduodenectomy was 16 (range 5–89) months. Four patients had recurrence. T stage, N stage, and resection radicality influenced survival. ConclusionPancreaticoduodenectomy is reasonable for recurrent remnant bile duct cancer following segmental bile duct resection, particularly for patients with no distant metastasis, locally confined recurrence, and good general condition.

Repeated Introperative Cholangiography is Helpful for Donor Safety in the Procurement of Right Liver Graft with Supraportal Right Bile Duct Variants in Living-Donor Liver Transplantation

BackgroundDespite recent advances in preoperative diagnostic imaging and operative techniques, biliary variation of the donor still remains a challenge in the procurement of graft. The supraportal right bile duct (BD) variant including presentation as trifurcation is a potential trap for injuring the remnant bile duct of donor. MethodsBefore living/related-donor liver transplantation (LRLT), cholangiogram with magnetic resonance images of each donor was performed as a routine. After exploration of the donor before hilar dissection, intraoperative chloangiography (IOC) was routinely performed. Among the supraportal right bile duct variants, if the preoperative cholangiography showed a suspected trifurcation of the bile duct, we then performed 3 sessions of IOC during liver graft procurement, including prior to hilar dissection, before the division of bile ducts and after the division. We reviewed the cholangiogram and the postoperative laboratory data of a consecutive series of 25 donors of LRLT. ResultsThere was no division injury of the remnant bile duct of all of the donors. ConclusionsRepeated IOC is suggested as a routine for variants of supraportal right bile ducts especially trifurcation pattern in graft procurement to avoid the injury of donor remnant bile ducts.

Fumarase Deficiency Associated With Noninflammatory Biliary Atresia

Fumarase Deficiency Associated With Noninflammatory Biliary Atresia

Classifying extrahepatic bile duct metachronous carcinoma byde novoneoplasia site

Extrahepatic bile duct (EHBD) cancer may occur metachronously, and these cancers are resectable with a favorable prognosis. We aimed to identify the pattern of metachronous EHBD cancer. We classified the cases of metachronous EHBD cancer reported in the literature thus far and investigated two new cases of metachronous EHBD cancer. A 70-year-old female underwent R0 bile duct resection for a type 1 Klatskin tumor (pT1N0M0). A 70-year-old male patient underwent R0 bile duct resection for a middle bile duct cancer (pT2N1M0). Imaging studies of both patients taken at 14 and 24 mo after first surgery respectively revealed a metachronous cholangiocarcinoma that required pancreaticoduodenectomy (PD). Histopathology of the both tumors after PD revealed cholangiocarcinoma invading the pancreas (pT3N0M0). Both patients have been free from recurrence for 6 years and 16 mo respectively after the second surgery. Through a review of the literature on these cases, we classified the pattern of metachronous EHBD cancer according to the site of de novo neoplasia. The proximal remnant bile duct was most commonly involved. Metachronous EHBD cancer should be distinguished from an unresectable recurrent tumor. Classifying metachronous EHBD cancer may be helpful in identifying rare metachronous tumors.

Hepatobiliary and Pancreatic: Cystic bile duct remnant after surgery for a choledochal cyst

Choledochal cysts are congenital anomalies of the bile duct that result in dilatation of the extrahepatic bile duct, the intrahepatic bile ducts or both components of the biliary system. Although several forms of dilatation have been described including bile duct diverticula, the most common is diffuse dilatation of the extrahepatic bile duct. The majority of these patients also have an anomalous junction of the bile duct and main pancreatic duct creating a “long common channel”. At least 50% of patients are diagnosed before the age of 10 years because of abdominal pain, jaundice, pancreatitis or a palpable abdominal mass. However, 25% of patients present as adults, usually with abdominal pain, pancreatitis or abnormal liver function tests. The diagnosis is readily made on an ultrasound study but additional investigations can include computed tomography (CT) scans, magnetic resonance imaging (MRI) scans and endoscopic retrograde cholangiopancreatography. The treatment of choice is usually surgical excision of the cyst with a biliary reconstruction using a choledochojejunostomy. Complications of untreated choledochal cysts include biliary cirrhosis, portal hypertension and the development of cholangiocarcinoma. Complications after surgery can include a cystic bile duct remnant as illustrated below. The patient was a 48-year-old woman who was admitted to our hospital with upper abdominal pain. Her past history included excision of a choledochal cyst at the age of 8 years. She was investigated with an enhanced CT scan, MRI scan (T2 weighted) and magnetic resonance cholangiopancreatography (MRCP). The CT scan (Figure 1, left) and MRCP (Figure 1, right) show a cystic structure in the head of the pancreas with a filling-defect, 15 mm in diameter. Various tumor markers were within the reference range. At laparotomy, there was a dilated remnant of the bile duct in the head of the pancreas (Figure 2, left). This was resected down to the junction with the main pancreatic duct. The bile duct remnant contained a calculus (Figure 2, right) while histological examination of the wall of the sac only revealed chronic inflammatory tissue. The patient has been followed for 12 months since surgery and is currently painfree. The management of enlarged but asymptomatic bile duct remnants is less clear although there are case reports of the development of bile duct cancer.