Introduction: We describe a case of a duodenal mass causing malignant biliary obstruction and the use of LAMS cholecystoduodenostomy for gallbladder drainage revealing white bile. Case Description/Methods: An 83 year-old male developed orange colored urine and stool for 3 weeks. CA 19-9 was 8,381 U/mL and direct bilirubin was 43.7 mg/dL. CT Abdomen and Pelvis with contrast showed a dilated common bile duct to 1.6cm with narrowing at the ampulla, dilated intrahepatic ducts, and a duodenal mass. ERCP demonstrated a large duodenal ulcerated mass from D1 to D3 and replaced the medial aspect of the duodenal wall. Despite probing the mass with guidewire, bile duct access was unsuccessful. Biopsies showed poorly differentiated adenocarcinoma with mucinous and signet ring cell features (Figure 1A). EUS showed a 4x6cm obstructing polypoid mass in the duodenum (star) with invasion into the distal common bile duct (arrow) resulting in a dilated common bile duct (circle) (Figure 1B). Subsequent LAMS cholecystoduodenostomy was performed for drainage (arrow) (Figure 1C). White bile (clear bile) was noted upon deployment into the gallbladder (star) (Figure 1D). Discussion: White bile in malignant biliary obstruction occurs because the biliary epithelium secretes mucus into bile despite the obstruction, resulting in colorless fluid. White bile has been associated with worse survival in these patients.Figure 1.: (a) duodenal adenocarcinoma with mucinous and signet ring cell features (b) duodenal adenocarcinoma (star) invading the distal common bile duct (arrow) resulting in a dilated common bile duct (circle) (c) LAMS deployment (arrow) within the dilated gallbladder while creating the cholecystoduodenostomy (d) White bile (star) draining through the LAMS.

Introduction: We describe a case of IgG4 cholangiopathy diagnosed via biliary pinch biopsies. Case Description/Methods: 78 year old male presented with pruritis and jaundice and had MRCP showing a 6x6cm liver mass and a 3x3cm pancreatic mass. Two years prior he presented with similar symptoms and was given a provisional diagnosis of IgG4 disease based on an elevated serum IgG4 of 300, hypoechoic mass on EUS and distal CBD structuring on ERCP. EUS biopsies showed necrotic pancreatic parenchyma with fibrosis and inflammatory changes, IgG4 was negative. He was treated with 5 sequential ERCP’s and biliary stents over 14 months and responded to stenting and prednisone (for one month) which was noted as possible shrinkage of the pancreatic mass. During his current presentation, EUS showed a dilated bile duct with a thickened wall and FNA/FNB revealed tough bile duct tissue that resisted passage of the needle. Preliminary FNA cytology was non-diagnostic. Diffuse changes of the pancreas suggested chronic pancreatitis; FNB was performed with cytology of the pancreatic head and bile duct showing no tumor cells. ERCP showed a 3cm distal bile duct stricture, and stricturing of the common hepatic duct, right and left hepatic ducts, and intrahepatic bile ducts. Brush biopsies and pinch biopsies were obtained from distal bile duct strictures. Balloon dilation of right hepatic duct, common hepatic duct and distal bile duct was performed with subsequent bilateral stent placement in right and left hepatic ducts. Brush biopsies of right hepatic duct, common hepatic duct and common bile ducts showed no tumor cells. Biopsy of the common hepatic duct showed dense lymphoplasmacytic inflammation suggestive of IgG4-related disease with IgG4+ cells at 25/HPF (Figure 1). There was an IgG4/CD138 ratio of at least 50%. Distal bile duct biopsies showed fibromuscular tissue, negative for malignancy. Discussion: The patient was subsequently treated with rituximab. 3 months later, repeat ERCP for stent removal showed that strictures had resolved and the biliary tree was open and draining immediately after stent removal. The cholangiogram normalized. This case demonstrates ERCP and biliary biopsies as a novel and quantifiable method of diagnosing IgG4 disease when the gold standard EUS guided core biopsy does not provide the diagnosis. ERCP and biliary pinch biopsies can be effective for diagnosing not only IgG4 disease, but this technique can also be applied to the diagnosis of bile duct or pancreaticobiliary malignancies.Figure 1.: Lymphoplasmacytic inflammation surrounding bile ducts; IgG4 positive cells at 25/HPF.

INTRODUCTION: Primary sclerosing cholangitis (PSC) is a chronic fibro-inflammatory process affecting hepatobiliary system. Reported lifetime incidence of cholangiocarcinoma (CCA) in PSC is 20%. Endoscopic retrograde cholangiography (ERCP) with brush cytology has high specificity but variable sensitivity to detect CCA. Atypical cells are frequently noted on cytology. It is unclear if atypical cells suggest presence of, or increased risk of development of cholangiocarcinoma as compared to patients with normal brush biopsy. METHODS: This is an IRB approved retrospective study looking at adult patients with PSC who received index ERCP with brush cytology at our large tertiary care academic institution from 2005 to 2018. All endoscopies were performed by two experienced endoscopists with more than 20 years of experience. We included patients having normal cells or atypical cells on brush cytology in our study. Demographic and clinical data including CA 19-9, results of imaging, follow up time, presence or development of cholangiocarcinoma if any, were recorded. RESULTS: We identified 210 patients with PSC of which 165 patients without an existing diagnosis of cholangiocarcinoma received index ERCP with brush cytology. Mean age was 43 (18) years and 67% were males. Of the 165 patients, 130 (79%) had normal cells on cytology, 28 (17%) patients had atypical cells on cytology, 1 had low grade dysplasia, and 6 (3.6%) patients were diagnosed with high grade dysplasia/adenocarcinoma. Two (7.1%) of 28 patients with atypical cells were diagnosed with cholangiocarcinoma as compared to 5 (3.8%) of 130 patients with normal cytology (P = 0.61) at a mean follow up of 7.3 (5.5) years. Of the 2 patients with atypical cells on cytology who were diagnosed with cholangiocarcinoma, the first patient was diagnosed within 3 months; this patient had significantly elevated CA 19-9 of 768 ng/ml. The CA 19-9 of all other 27 patients was less than 100 ng/ml. The second patient developed cholangiocarcinoma at 16 years from index ERCP. Twenty-four patients had subsequent repeat ERCP with brush cytology, of which 22 (92%) had turned to normal cytology and 2 (8%) remained atypical cells. CONCLUSION: PSC patients with atypical cells on brush cytology during ERCP and having a normal CA 19-9 are unlikely to harbor cholangiocarcinoma and are at low risk for development of cholangiocarcinoma in the near future. In these patients the atypical cells are likely as a result of inflammation from underlying PSC rather than cholangiocarcinoma.

To assess the efficacy and safety of bilateral versus unilateral biliary drainage in malignant hilar obstruction.

Background and Aims: Bile leaks are uncommon but are a painful postoperative complication of hepatobiliary interventions. Many authors advocate treating them with biliary stenting. We compared the outcomes in patients treated with endoscopic biliary sphincterotomy (EBS) alone versus EBS with biliary stenting. Methods: We reviewed charts of patients treated endoscopically for bile leak from 2009 to 2015 at our tertiary care center. Based on endoscopists’ practice preference, patients underwent EBS alone or with a biliary stent. Clinical resolution of bile leak and total number of endoscopic and nonendoscopic interventions were compared between patients treated with EBS alone versus EBS with a biliary stent. Results: Fifty-eight patients were included; etiology was cholecystectomy (52), hepatic resection (5), and liver trauma (1). The leak was from the cystic duct (22), duct of Luschka (23), common bile, or hepatic duct (2), and intrahepatic duct (11). Thirty-seven patients had EBS alone (EBS group), and 21 had stents (stent group). Single intervention resolved the bile leak in 34 (92%) patients in EBS group and 19 (90%) in the stent group (p = 0.85). Resolution was slower (p = 0.02) and more patients required second intervention (p < 0.01) in the stent group. Conclusion: EBS with or without a biliary stent is highly effective in the management of bile leak. Clinical resolution of the bile leak is quicker with EBS alone, requires fewer interventions, and may cost less.

CASE REPORT A 67-year-old woman with a history of left-sided ulcerative colitis, sclerosing cholangitis, sclerosing pancreatitis, slow transit constipation, recurrent stenosis of the pancreatic orifice, and Waldenstrom's macroglobulinemia presented for her yearly colonoscopy for colorectal cancer screening. Her outpatient medications at the time of colonoscopy included acetaminophen, alprazolam, calcium/vitamin D supplement, dexlansoprazole, estradiol, as needed ondansetron, pancrelipase before meals, and as need tramadol. Her colonoscopy showed dark black pigmentation, and it was virtually impossible to see anything with standard illumination on the colonoscope, and no active ulcerations were noted (Figure 1). She was asymptomatic with stable vitals. She reported using natural fiber supplement that has rhubarb in it. She denied any laxative use. She stopped using rhubarb extract and underwent repeat colonoscopy 1½ years later, which showed profound improvement in the previously noted dark black pigmentation (Figure 2). None of the existing outpatient medications were changed or stopped between the colonoscopies.Figure 1.: Initial colonoscopy showing dark black pigmentation without any active ulcerations.Figure 2.: Repeat colonoscopy with profound improvement in the dark black pigmentation noted on initial colonoscopy.It was thought that the anthranoid compound contained within the rhubarb was the likely source of her melanosis coli. A thorough search of the literature was done, and none of her outpatient medications were found to be associated with melanosis coli. No clear association was found between inflammatory bowel disease and melanosis coli. Only one small retrospective study was found which indicated a possible association between inflammatory bowel disease and melanosis coli; however, there are no confirmatory retrospective studies, prospective studies, or further case reports documenting this finding. In our patient, the ulcerative colitis was reasonably well controlled with left-sided chronic colitis and focal active colitis on the biopsies. This does not correlate with the extensive melanosis coli noted on the endoscopy. Melanosis coli is a distinct endoscopic finding which is often associated with chronic laxative use in which dark cellular pigment deposits in the lamina propria of the colon.1,2 This patient reported taking rhubarb for years. The active ingredient in rhubarb is anthraquinone which causes injury to the colonic epithelial cells, resulting in melanosis coli.1,2 The anthranoid products pass unabsorbed throughout the bowel until they reach the large intestine, where they are converted to their active forms.1 The anthranoid products damage the epithelial cells, causing changes in absorption, secretion, and motility.3 The active forms of anthranoid products further cause injury to the epithelial cells, leading to apoptosis (a form of cell death), which releases darkly cellular pigment (lipofuscin) that are taken up by macrophages.1–3 The pigment deposition results in a distinctive dark brown to black staining of the lining of the large intestine. The condition is benign and reversible on discontinuing the implicating agent.2,3 Disappearance of the pigment usually occurs within a year after stopping anthraquinone use.1–3 DISCLOSURES Author contributions: JS Klair performed the literature review, drafted the case, critically revised the manuscript, and is the article guarantor. S. Chandra performed the literature review, drafted the case, and critically revised the manuscript. FC Johlin critically revised the manuscript. Financial disclosure: None. Informed consent was obtained for this case report.

BACKGROUND Bilateral vs unilateral biliary stenting is used for palliation in malignant biliary obstruction. No clear data is available to compare the efficacy and safety of bilateral biliary stenting over unilateral stenting. AIM To assess the efficacy and safety of bilateral vs unilateral biliary drainage in inoperable malignant hilar obstruction. METHODS PubMed, Embase, Scopus, and Cochrane databases, as well as secondary sources (bibliographic review of selected articles and major GI proceedings), were searched through January 2019. The primary outcome was the re-intervention rate. Secondary outcomes were a technical success, early and late complications, and stent malfunction rate. Pooled odds ratio (OR) and 95% confidence interval (CI) were calculated for each outcome. RESULTS A total of 9 studies were included (2 prospective Randomized Controlled Study, 5 retrospective studies, and 2 abstracts), involving 782 patients with malignant hilar obstruction. Bilateral stenting had significantly lower re-intervention rate compared with unilateral drainage (OR = 0.59, 95% CI: 0.40-0.87, P = 0.009). There was no difference in the technical success rate (OR = 0.7, CI: 0.42-1.17, P = 0.17), early complication rate (OR = 1.56, CI: 0.31-7.75, P = 0.59), late complication rate (OR = 0.91, CI: 0.58-1.41, P = 0.56) and stent malfunction (OR = 0.69, CI: 0.42-1.12, P = 0.14) between bilateral and unilateral stenting for malignant hilar biliary strictures. CONCLUSION Bilateral biliary drainage had a lower re-intervention rate as compared to unilateral drainage for high grade inoperable malignant biliary strictures, with no significant difference in technical success, and early or late complication rates.

BACKGROUND:Bacteremia due to cholangitis can occur as a complication of biliary instrumentation. Biliary sepsis can result from frequent endoscopic retrograde cholangiopancreatography (ERCP).METHODS:We hypothesized that routine use of antibiotics in patients who require frequent ERCPs leads to cholangitis resistant to empiric antibiotics used to treat biliary sepsis. We retrospectively reviewed patients with frequent biliary instrumentation and blood stream infection due to cholangitis. Conventional empiric antibiotics were defined as broad-spectrum antibacterial agents predominantly used for community-acquired infections and surgical prophylaxis. Broad-spectrum antibacterial agents used for hospital-onset/multidrug-resistant infections were defined as broad-spectrum MDR antibiotics.RESULTS:Seventy-eight patients had bacteremia secondary to cholangitis from biliary obstruction. Over 50% of bacteria were not sensitive to conventional empiric antibiotics for biliary sepsis. Thirty-seven patients did not receive post-procedural antibiotics and forty-one patients did. Of the ones who did, 58% later had a bloodstream infection with bacteria resistant to the antibiotic used for prophylaxis, and 26 patients (63%) required a broad-spectrum MDR antibiotic for treatment. The number of ERCPs was not associated with resistance to prophylactic antibiotics (p 0.7103) or needing broad-spectrum MDR antibiotics for treatment of cholangitis-associated bacteremia (p 0.1868). Routine use of antibiotic prophylaxis after ERCP was associated with trend toward need for broad-spectrum MDR antibiotics for cholangitis-associated bacteremia, Chi-square 3.7, 0 0.0540.CONCLUSION:Bacterial resistance to conventional empiric antibiotics is an emerging problem. Blood cultures are needed to guide therapy.

Biliary brushing cytology is a common diagnostic tool to evaluate pancreatic and biliary strictures. Although this technique has been shown to have a high specificity, it has a relatively low reported sensitivity. False negatives are usually attributed to scant cellularity and poor cellular preservation. In this pilot study, we sought to determine impact of sheath rinsing on cellular yield of biliary brush cytology for biliary stricture.

Melanosis coli is a dark pigmentation of colonic wall that is often identified at the time of colonoscopy. We present a second reported case of melanosis coli in a patient taking rhubarb containing herbal laxative. 67-year-old Caucasian female with ulcerative proctosigmoiditis (in clinical remission), sclerosing pancreatitis, and slow transit constipation, noted to have black coloration of the whole colon during a screening colonoscopy. The pigmentation was very dense, limiting the mucosal examination on both standard and peak illumination (Figure 1, panel A/B). This was new from a colonoscopy 2 years prior. Minimal coble stoning was noted in the sigmoid colon. Mucosal biopsies confirmed diagnosis of melanosis coli and chronic colitis with focal mild activity in the sigmoid colon. Her medication list included alprazolam, ascorbic acid, dexlansoprazole, dicyclomine, omega-3 fatty acids, pancrelipase, tramadol and multivitamin and an herbal fiber supplement containing rhubarb. She stopped using rhubarb extract and repeat colonoscopy 2 years later showed complete resolution of melanosis coli (Figure 1, panel C). The cobblestoning of sigmoid colon was also resolved and no active colitis. Melanosis coli is pigmentation of colonic mucosa secondary to lipofuscin deposition in macrophages in the lamina propria. It is considered a result of increased apoptosis. Lysosomal digestion on cellular organelles forms lipofuscin granules. These granules are then taken up by the macrophages. Our patient reported taking rhubarb containing herbal laxative. Rhubarb has anthraquinones which passes unabsorbed through the small bowel and get activated in the colon, where they are converted to active forms. These in turn cause epithelial cell damage leading to altered absorption, secretion and motility. The epithelial cells damage leads to apoptosis. Melanosis coli is considered benign and is reversible on discontinuing implicating agent. Disappearance of the pigment usually occurs within a year after a patient stops taking anthraquinone but microscopic feature could take longer to clear.Figure: A/B - initial colonoscopy showing severe melanosis coli. Fig 1C - Repeat colonoscopy showing improved melanosis coli.

BACKGROUND:Only a small proportion of patients with biliary tree infection grow microorganisms in blood cultures. Antibiotics chosen or tailored based on organisms identified on blood cultures have a potential for under-treatment and unfavorable outcomes, including recurrent infection and early stent occlusion. In our current practice, we collect bile for culture if an Endoscopic Retrograde Cholangio-Pancreatography (ERCP) is performed in patients with suspected cholangitis. In this study, we compare the microbial yield of blood cultures and ERCP-obtained bile cultures in patients with ascending cholangitis.METHODS:We reviewed medical records of all the patients treated for ascending cholangitis who had blood cultures and ERCP-obtained bile cultures at a tertiary care center between 2010 and 2016. Bile was collected for culture before injecting contrast, via a catheter after discarding the initial 3 mL.RESULTS:Ninety-three patients were included with mean age of 71 (±15) years. Out of 93 patients, 11 (12%) had prior sphincterotomy, 29 (31%) had an indwelling biliary stent, and malignant obstruction was the most common etiology (34%). ERCP-obtained bile cultures were positive in 90 out of 93 (97%) patients with monomicrobial growth in 34 out of 93 (39%) patients. Mixed intestinal flora was noted in 3 patients. Blood cultures were positive in only 30 out of 93 patients (32%) and 24 out of 93 (26%) patients had monomicrobial growth. Totally 26 out of 30 patients (87%) grew the same organism as the bile culture, 3 grew an organism different from bile cultures, and one had no growth in the bile culture. On multivariable analysis, the presence of an indwelling biliary stent was the lone factor associated with polymicrobial growth, 83 vs. 52%, p = 0.007.CONCLUSION:ERCP-obtained bile cultures are a reliable and feasible mechanism to evaluate patients with suspected biliary tree infection. This technique has a significantly higher yield when compared to blood culture. Selection and tailoring of antibiotics based on bile culture in the management of ascending cholangitis are advised.

Bile leaks are uncommon but are a painful post-operative complication of hepatobiliary interventions. Many authors advocate treating these patients with biliary stenting. We compared the outcomes in patients treated with endoscopic biliary sphincterotomy (EBS) alone versus EBS with biliary stenting.

The incidence of intrahepatic cholangiocarcinoma has been increasing in the last few decades. Interestingly over this period, the incidence of extrahepatic cholangiocarcinoma remained stable. However, the proportion of patients with early stage, small or localized tumors remained the same. This reflects a true increase in the incidence without a corresponding significant gain in our ability to detect cholangiocarcinoma at an earlier stage. As a result, the cholangiocarcinoma related mortality has also increased significantly. Primary sclerosing cholangitis (PSC) is a major risk factor for cholangiocarcinoma in the Western population. Although identification of cholangiocarcinoma at an earlier stage has significantly better outcomes, our ability to detect these early cancers is limited. Tumor markers (CA 19-9 and CEA) may have value for screening in some settings, but effectiveness for detecting small early stage tumors is questionable and magnetic resonance cholangiopancreatography is used in some centers without robust evidence to support its utility. Currently endoscopic retrograde cholangiopancreatography (ERCP) with brush cytology or biopsy is not recommended as screening modality in PSC due to its low sensitivity. However, ERCP with brush cytology is recommended for sampling biliary and pancreatic strictures, but sensitivity continues to be low. False negative results are usually attributed to limitation in cell acquisition and corresponding low cellularity. Techniques have been attempted to improve cell acquisition, including use of intraductal biopsies, alone or in combination with brush cytology and use of large bristle versus small bristle brushes have been examined. Diagnostic accuracy of biliary brushing at our center has been higher than what is reported in the literature. We theorize that it might be due to the fact that we submit both the brush (cut from the neck) and sheath rinse which, to our knowledge, is not a common practice. The underlying hypothesis for this technique is that during the brushing, cells may be dislodged from the brush into the protective sheath. The sheath scrapes the duct wall in the area of stricture potentially further dislodging cellular material into the sheath. The aim of this study was to determine impact of incorporating sheath rinsing on cellular yield of biliary brush cytology. This prospective study was performed at am US tertiary care center. The study protocol was approved by the institutional review board. Consecutive patients who underwent ERCP over a one month period for biliary strictures were enrolled. The procedure was performed by an advanced endoscopist with more than 20 years of experience performing ERCP. The specimens were obtained during the ERCP by brushing the common bile duct, common hepatic duct, and right and left hepatic ducts. A ConMed sheathed colonoscopy brush was used. The brush diameter is 3.0 mm and the sheath outer diameter is 2.1 mm. The brush and the sheath were advanced above the stricture using a monorail technique. The lead edge of the sheath and the brush were advanced and withdrawn through the stricture for a total Received: May 25, 2017 Revised: August 25, 2017 Accepted: August 26, 2017 Correspondence: Frederick C. Johlin Division of Gastroenterology and Hepatology, University of Iowa Hospitals and Clinics, 200 Hawkins drive, 4553D JCP, Iowa City, IA 52242, USA Tel: +1-319-356-4030, Fax: +1-319-535-6399, E-mail: fredjohlin@msn.com

BACKGROUND/AIMS:The vast majority of serous cystic neoplasms of the pancreas are benign, and small, asymptomatic lesions, which are generally managed with observation. However, some of these tumors may attain a large size and occasionally metastasize.METHODS:In this study, we present a 78-year-old man with serous cystadenocarcinoma of the pancreas with liver metastases treated by distal pancreatectomy and liver ablation, who went on to develop new liver metastases 5 years after the initial operation. We perform a literature review to determine the number of these malignant neoplasms previously reported and to identify features associated with malignant lesions.RESULTS:Literature reveals that metastatic serous cystadenocarcinomas of the pancreas are rare tumors, occurring in less than 3% of cases of serous cystic neoplasms. All malignant cases reported have been in tumors >4 cm in size.CONCLUSIONS:Serous cystic neoplasms of the pancreas >4 cm have malignant potential and therefore should be considered for surgical management.

Background Unresectable tumors of the pancreatic head are encountered in up to 20% of patients taken for resection. The objective of this study was to evaluate the complications and outcome associated with palliative surgical procedures to help guide management decisions in these patients. Methods Patients with pancreatic head adenocarcinoma taken to the operating room with curative intent who did not undergo pancreatectomy were evaluated. Results From 1997 to 2013, 50 patients were explored and found be unresectable due to M1 disease (n=27, 54.0%) or vascular invasion (n=23, 46.0%). Among unresectable patients, 34 (68.0%) had a palliative procedure performed including double bypass (n=13), biliary bypass (n=7), gastrojejunostomy (n=5), or cholecystectomy (n=9). Complications occurred in 22 patients (44.0%), and patients who had a palliative operation had a longer hospital stay and more major complications. Overall survival was reduced in patients treated with a palliative operation. Conclusions Despite advancements in endoscopic palliation, operative bypasses are still commonplace in patients with unresectable pancreatic head cancer. In this study, patients treated with operative procedures had a high rate of complications without a notable improvement in outcome. These findings highlight the importance of identifying unresectable disease prior to surgery and support a selective approach to palliative operations. J. Surg. Oncol 2014; 109:697-701. (c) 2014 Wiley Periodicals, Inc.

This retrospective study reviews our experience in surveillance and early detection of cholangiocarcinoma (CC) and in using en bloc total hepatectomy-pancreaticoduodenectomy-orthotopic liver transplantation (OLT-Whipple) to achieve complete eradication of early-stage CC complicating primary sclerosing cholangitis (PSC). Asymptomatic PSC patients underwent surveillance using endoscopic ultrasound and endoscopic retrograde cholangiopancreatography (ERCP) with multilevel brushings for cytological evaluation. Patients diagnosed with CC were treated with combined extra-beam radiotherapy, lesion-focused brachytherapy, and OLT-Whipple. Between 1988 and 2001, 42 of 119 PSC patients were followed according to the surveillance protocol. CC was detected in 8 patients, 6 of whom underwent OLT-Whipple. Of those 6 patients, 4 had stage I CC, and 2 had stage II CC. All 6 OLT-Whipple patients received combined external-beam and brachytherapy radiotherapy. The median time from diagnosis to OLT-Whipple was 144 days. One patient died 55 months post-transplant of an unrelated cause, without tumor recurrence. The other 5 are well without recurrence at 5.7, 7.0, 8.7, 8.8, and 10.1 years. In conclusion, for patients with PSC, ERCP surveillance cytology and intralumenal endoscopic ultrasound examination allow for early detection of CC. Broad and lesion-focused radiotherapy combined with OLT-Whipple to remove the biliary epithelium en bloc offers promising long-term, tumor-free survival. All patients tolerated this extensive surgery well with good quality of life following surgery and recovery. These findings support consideration of the complete excision of an intact biliary tree via OLT-Whipple in patients with early-stage hilar CC complicating PSC.