Proximal Hepatic Duct Research Articles

Role of Intraoperative Frozen Section Assessment of Proximal Bile Duct Margins and the Impact of Additional Re-Resection in Perihilar Cholangiocarcinomas.

Intraoperative frozen section (FS) analysis to assess the bile duct margin status is commonly used to assess the completeness of resection during surgery for perihilar cholangiocarcinoma (pCCA) resection. However, the impact of additional re-section on the long-term outcome after obtaining an initial positive margin remains unclear. Patients diagnosed as pCCA on preoperative imaging and subjected to curative intent surgery from May 2013 to June 2021 with a minimum follow-up of 2years were included. Intraoperative FS analysis of the proximal bile duct margin was performed in all patients. A positive margin was defined by the presence of invasive cancer. Out of the 62 patients with a preoperative diagnosis of pCCA on imaging, 35 patients were included for final analyses after excluding patients with inoperable disease (on staging laparoscopy or local exploration) and other/benign pathology on the final histopathology report. Out of the 35 patients, patients with postoperative 90-day mortality were excluded from the final survival analysis. FS analysis revealed an initial positive margin in 10 (28.5%) patients. Among 10 patients who underwent re-resection to achieve negative proximal margins, only 5 patients achieved a negative margin (secondary R0). An initial positive margin was associated with poor long-term outcomes. Median disease-free survival (DFS) and overall survival (OS) were 16 and 19.6months for patients with an initial positive margin, but 36 and 58.2months for patients with an initial negative margin, respectively (p = 0.012). The median DFS and OS were significantly lower for those with secondary R0 as compared to primary R0 (16 vs. 36months for DFS, p = 0.117 and 19.6 vs. 58.2months for OS, p = 0.027, respectively). An intraoperative FS positive proximal hepatic duct margin dictates poor long-term outcomes for patients with resectable pCCA. Additional resection has a questionable benefit on survival, when a secondary negative margin is achieved.

Role of Intraoperative Assessment of Proximal Bile Duct Margin Status and Additional Resection of Perihilar Cholangiocarcinoma: Can Local Clearance Trump Tumor Biology? A Retrospective Cohort Study.

The aim of this study was to evaluate the clinical implications of the proximal bile duct margin status in resection of perihilar cholangiocarcinoma (PHCC). Intraoperative frozen section (IFS) analysis to assess the bile duct margin status is commonly used during PHCC resection. However, the impact of additional resection after obtaining a positive margin on the long-term outcome remains unclear. Among the 257 patients who underwent PHCC resection, 190 patients with a negative distal margin were included and analyzed. IFS analysis of the proximal bile duct margin was performed in all patients. A positive margin was defined by the presence of either invasive cancer, or carcinoma, insitu. IFS analysis revealed an initial positive margin in 69 (36%) patients. Among 20 patients who underwent re-resection, only 11 patients achieved a negative margin (secondary R0). An initial positive margin was associated with poor long-term outcomes: recurrence-free survival (RFS) and overall survival (OS) were 16 and 25 months for patients with an initial positive margin, but 47 and 63 months for patients with an initial negative margin, respectively (p<0.0001). In contrast, there was no difference in RFS or OS between patients with a secondary R0 margin, and those with a final R1 margin (14 vs. 16 months for RFS, p=0.98, and 23 versus 25 months for OS, p=0.63, respectively). An IFS-positive proximal hepatic duct margin dictates poor long-term outcomes for patients with resectable PHCC. Additional resection has minimal impact on survival, even when negative margin is achieved.

The morphological and histopathological assessment of Alagille syndrome with extrahepatic bile duct obstruction: the importance of the differential diagnosis with subgroup "o" biliary atresia.

The differential diagnosis between Alagille syndrome (AGS) with extrahepatic bile duct obstruction (EHBDO) and biliary atresia (BA) is difficult. We report a case series of AGS with EHBDO with detailed validation of the morphological and histopathological features for the differential diagnosis of BA. Six liver transplantations (LTs) were performed for AGS with EHBDO. All patients were diagnosed with BA at the referring institution and the diagnosis of AGS was then confirmed based on a genetic analysis before LT. We verified the morphological and histopathological findings of the porta hepatis and liver at the diagnosis of BA and at LT. All patients had acholic stool in the neonatal period and were diagnosed with BA by cholangiography. The gross liver findings included a smooth and soft surface, without any cirrhosis. The gross findings of the porta hepatis included aplasia of the proximal hepatic duct, or subgroup "o", in five patients. The histopathological examination of the EHBD also revealed obstruction/absence of the hepatic duct. There were no patients with aplasia of the common bile duct. Aplasia of the hepatic duct and the macroscopic liver findings may help in to differentiate between AGS with EHBDO and BA.

Mirizzi syndrome - a reconstruction reconstrued: Technique for bile duct reconstruction using a vascularized gallbladder pedicle

Presenter: Prakash Jayanthi MD | Michigan State University College of Human Medicine Background: Mirizzi’s Syndrome is rare, with < 1% incidence in developed countries. No best treatment algorithm is available. Correct preoperative identification and planning is associated with less morbidity, with operative preference based on surgeon experience. The greater the degree of inflammation/erosion, the more complex the treatment - Csendes Type 1 Mirizzi patients benefit from cholecystectomy alone; Types 2-4 may require subtotal cholecystectomy/choledochoplasty versus biliary-enteric reconstruction; Type 5 usually requires fistula takedown/reconstruction. Methods: A 74-year old female presented with severe RUQ pain. CECT Abd/Pelvis: pneumobilia with air in gallbladder fossa. Perforated, gangrenous cholecystitis was suspected. MRI/MRCP: 3.5 cm gallstone impacted in the gallbladder neck, proximal hepatic duct dilatation, likely adjacent abscess. Bilioenteric fistula was suspected due to pneumobilia. Open cholecystectomy was planned for a diagnosis of Csendes Type V - fistula with complete bile duct wall erosion. Normally, fistula takedown with bilio-enteric reconstruction is preferred; however, the fistula ruptured, with resultant severe inflammation making bilio-enteric reconstruction hazardous. Hence, choledochoplasty using a well-vascularized pedicle of gallbladder neck was performed. Results: Intraoperatively, severe inflammation obscured normal anatomy. The gallbladder was taken dome-down. A broad neck of the gallbladder was attached to the CHD, consistent with Mirizzi’s Syndrome. Subtotal cholecystectomy was performed, leaving a remnant cuff vascularized via cystic artery. Frozen sections were sent, with no evidence of cancer. A pericholecystic abscess, likely from a ruptured bilioenteric fistula, was debrided. A 3.5cm gallstone caused near complete erosion of anterior bile duct - Csendes Type V. The back wall of the duct was identified, with proximal channel towards the liver and distal channel towards duodenum. Choledochoscopy demonstrated no obstruction or lesions. Extensive xanthogranulomatous inflammation precluded biliary-enteric reconstruction, with further dissection concerning for possible Right Hepatic artery compromise. The gallbladder flap approximated easily- therefore choledochoplasty was decided upon. Proximal/distal bile duct was cannulated by 18Fr T-tube; choledochoplasty was performed over it, using interrupted 3-0 Maxon suture without narrowing luminal diameter, re-creating an anterior wall for the bile duct common channel. Pressurized saline through T-tube showed no leak. T-tube cholangiogram confirmed patency and absence of stenosis/obstruction. Several weeks later, a T-tube cholangiogram showed satisfactory repair, with appropriate passage of contrast. Hence, the tube was then removed. The patient did not have any complications. Conclusion: Although rare, Mirizzi’s Syndrome is well-known and historically classified into Type 1 - Gallstone compression of bile duct; Type 2 - Common hepatic/bile duct erosion. Csendes et al. (1989) classified Mirizzi syndrome based on degree of erosion of duct wall into 4 types. Type 5 including cholecystoenteric fistula +/- gallstone ileus was added in 2007. Preoperative suspicion is paramount as bile duct injury approaches 17% incidence, with preoperative diagnosis only made in 62% of patients. Open repair is the gold standard. Cholecystectomy should be sufficient in type 1; any duct wall erosion makes a total cholecystectomy potentially unsafe. Bilioenteric reconstruction, e.g. choledochoplasty or hepaticojejunostomy, may be required for continuity. Choledochoscopy is used to confirm patency, as are cholangiograms in patients with T-tube. This case highlights this disease's unpredictable nature, and the need for familiarity with options for repair.

2147 Adenomatous Polyp of the Biliojejunal Anastomosis and Bile Duct in a Familial Adenomatous Polyposis (FAP) Patient: A Multidisciplinary Approach to a Clinical Dilemma

INTRODUCTION: Duodenal cancer is the second most common malignancy in FAP patients, and advanced polyposis often requires duodenal resection. Post-duodenectomy FAP patients are at risk of developing polyps in the post-anastomotic jejunum. We present a challenging case of a post-duodenectomy FAP patient who developed adenomatous polyp at the biliojejunal anastomosis and bile duct. CASE DESCRIPTION/METHODS: A 36 year-old female with FAP, status-post proctocolectomy, pancreas-sparing duodenectomy for Spiegelman stage IV polyposis was found to have polyposis in the jejunum on surveillance capsule endoscopy. Push-enteroscopy revealed polyposis in the jejunum with a 15 mm polyp involving the biliojejunal anastomosis (Image 1). Given that this polyp was non-lifting, it was removed with hybrid endoscopic submucosal dissection (ESD) (Image 2). After polyp removal, there was suspicion the polyp extended into the distal common bile duct (CBD) which was confirmed with biopsies revealing low-grade dysplastic adenoma. After a multidisciplinary discussion, cholangioscopy with Habib EndoHPB radiofrequency ablation (RFA) (Boston Scientific, Marlborough, MA, USA) was planned (Image 3). The adenomatous tissue extended from the distal CBD to the proximal common hepatic duct (CHD) which was confirmed by biopsies. Biliary RFA treatment was inadequate evidenced by poor tissue response noted on cholangioscopy and repeat positive biopsies. We theorized that this was due to poor tissue apposition with the thin RFA catheter as there was no stricture. Given the patient’s young age, extent of bile duct involvement, and poor tissue response to RFA, we determined that a completion Whipple surgery would be the best long-term option. DISCUSSION: We present a unique case of a post-duodenectomy FAP patient with adenomatous polyp extending from the biliojejunal anastomosis to the proximal hepatic duct. We were able to remove the polyp at the biliojejunal anastomosis with hybrid ESD, but the biliary adenomatous tissue was not adequately treated by biliary RFA. The thin RFA catheter had poor contact on the bile duct wall as there was no stricture. If a balloon RFA biliary catheter existed that could provide better tissue contact with a normal diameter bile duct, maybe this could have been treated endoscopically. This case highlights the importance of multidisciplinary management of complex polyposis in FAP patients.

Bile Duct Papillary Adenoma Degenerating Into Cholangiocarcinoma

A 79 year old Caucasian female was incidentally discovered to have transaminitis on routine labs and underwent right upper quadrant ultrasound revealing a dilated bile duct. MR Abdomen revealed a solid mass in the proximal common hepatic duct. She was asymptomatic without jaundice, weight loss, pruritus or abdominal pain. Additional and repeat labs showed a mildly elevated carbohydrate antigen 19-9 (CA-19-9) at 41.49 and normal carcinoembryonic antigen (CEA). Endoscopic Ultrasound (EUS) revealed a 1.5 x 2 cm mass in the common hepatic duct and fine needle aspiration showed atypical glandular papillary cells with no evidence of neoplasm. Endoscopic Retrograde Cholangiopancreatography (ERCP) with cholangioscopy demonstrated a frond-like mass in the common hepatic duct with pathology indicative of dysplastic epithelium consistent with low-grade dysplasia without frank malignancy. Repeat CA-19-9 level normalized at 30.11. She underwent pre-operative evaluation and was found to have severe aortic stenosis necessitating valve replacement and thus biliary surgery was delayed. Repeat labs four months later showed highly elevated CA-19-9 at 1382.10 concerning for degeneration of adenoma to malignancy. She underwent diagnostic laparoscopy with resection of the extrahepatic biliary tree including bifurcation, Roux-en-Y hepaticojejunostomy, hilar lymphadenectomy and cholecystectomy. Hepatic duct mass pathology showed a 3 x 1.4 cm exophytic papillary adenoma with high grade dysplasia, within which was a 0.3 cm moderately differentiated adenocarcinoma involving the left proximal hepatic duct invading into but not through the hepatic duct wall with negative margins. Final diagnosis was Stage I cholangiocarcinoma (T1N0M0) within a papillary bile duct adenoma. Bile duct adenomas (BDA) are rare bile duct tumors accounting for roughly 6% of extrahepatic tumors. Adenomas of the extrahepatic bile ducts may present with obstructive symptoms or may be asymptomatic, found incidentally on physical exam or imaging. Though defined as a benign tumor, BDAs have carcinogenic potential. The incidence and timing of malignant transformation is not well defined and thus prompt surgical resection is indicated for treatment. In our case, after a necessary delay in therapy, CA-19-9 levels dramatically increased and her tumor that was initially felt to be BDA degenerated to cholangiocarcinoma.Figure 1Figure 2Figure 3

Heterotopic Pancreas within the Proximal Hepatic Duct, Containing Intraductal Papillary Mucinous Neoplasm

We report a unique first case of benign heterotopic pancreas arising within the proximal hepatic bile duct, containing a focus of intraductal papillary mucinous neoplasm (IPMN). The condition was diagnosed on pathological explant after left hepatic lobectomy with total extrahepatic bile duct excision.

A Unique Case of Noncirrhotic Portal Hypertension Secondary to Extrahepatic Sarcoidosis

Introduction: Sarcoidosis is a multisystem disease of unknown etiology characterized by the presence of non-caseating granulomas. Portal hypertension is a rare manifestation of sarcoidosis, first reported in 1949. Here we report the first case of extrahepatic sarcoidosis with granulomatous lymph node involvement at the porta hepatis, compression of portal vein leading to portal hypertension and recurrent episodes of gastrointestinal bleeding due to jejunal varices. A 57-year-old black female presented with pruritus, jaundice, elevated liver enzymes, and thrombocytopenia. Diagnosed with ophthalmic and pulmonary sarcoidosis in 1998, a mass at the porta hepatis causing intrahepatic dilation was now seen on CT abdomen. ERCP revealed a stricture at the proximal hepatic duct below the bifurcation, relieved by placing a biliary stent. Diagnostic exploratory laparotomy showed unresectable massive lymphadenopathy, extending from porta hepatis to the inferior aspect of liver, encroaching the vessels and bile ducts. Intrahepatic right bile duct choledoco-Roux-en-Y-jejunostomy was completed. Biopsy showed marked fibrosis and noncaseating granulomas of lymph nodes. Liver biopsy was free of sarcoidosis. Repeat episodes of pruritis and jaundice were treated with high-dose steroids and mycophenolate mofetil. Patient was readmitted with melena and severe anemia. Imaging showed slight decrease in size of the mass but effacement of extrahepatic portal vein, non-visualization of the left portal vein, and severe narrowing of right portal vein. Double balloon enteroscopy revealed large, bleeding perianastomotic jejunal varices. Endoscopic therapy failed and varices were surgically oversawn. Mycophenalate mofetil was restarted along with steroids. A year later recurrent episodes of severe bleeding developed. High-dose pulse steroid therapy was initiated and mycophenalte was increased. Mesocaval shunting was completed with temporary control of bleeding before occlusion. Patient was kept on high-dose steroids, mycophenalate, octreotide and nadolol. Later, CT abdomen revealed reduction in size of mass with partial visualization of the right branch of the portal vein. TIPS was completed via cannulation of the right portal vein, leading to resolution of portal hypertension and further episodes of GI bleed. Extrahepatic portal hypertension in the setting of porta hepatis sarcoidosis can be life threatening and very challenging to treat. Here we described how a combination immunosuppressant and radiologically placed shunt can be used successfully to treat recurrent episodes of GI bleed in such a patient.

U-graft anastomosis for anomalous portal venous branching reconstruction in right lobe living donor liver transplantation

U-graft anastomosis for anomalous portal venous branching reconstruction in right lobe living donor liver transplantation

Microvascularization of the Canine Hepatic Ducts

The angioarchitecture of the proximal and distal segments of the hepatic duct in the dog was investigated by means of vascular corrosion casts under a scanning electron microscope. The results of observations indicated a change of the pattern of vascularization of the hepatic duct along with the increasing distance of the hepatic duct from the liver and increasing diameter of the duct. In the proximal hepatic duct, the main blood vessels run along the duct as a pair of supplying arteriole and voluminous collecting venule, while in the distal segments of the hepatic duct on the opposite margin of the duct two vascular triads were observed, composed of two venules and one medial arteriole. On the surface of both segments of the hepatic duct, there are well-anastomosed outer venous plexuses. In the distal segments of the hepatic duct, the outer venous plexus accompanies a fine outer arterial rete. Observations of the intramural network indicate the presence of single terminal arterioles running to mucosa and supplying a subepithelial capillary network. Differences were observed in the blood drainage from the mucosa, as in the proximal segment of the hepatic duct single post-capillary venules are found, while in the distal segment in the mucosa a well-developed mucosal venous plexus is formed. In the well-developed venous system of the hepatic duct no valves were observed.

Traumatic Disruption of the Proximal Hepatic Duct: Endoscopic Treatment After Failure of Surgical Repair

Traumatic Disruption of the Proximal Hepatic Duct: Endoscopic Treatment After Failure of Surgical Repair

Transpapillary Microwave Coagulation Therapy for Recanalizing Self-Expandable Metallic Stents Occluded by Tumor Ingrowth: Initial Experience

Percutaneous microwave coagulation for recanalizing stents occluded by tumor ingrowth has been reported. With this technique, however, the percutaneous drain diminishes the quality of life in patients with unresectable tumors and a limited prognosis. Transpapillary microwave ablation was attempted in three patients with occluded stents. After a sheath had been inserted into the proximal hepatic duct across the occluded region, a microwave electrode was introduced into the intrahepatic duct via the sheath. We used microwave therapy with an output power of 40 W, based on our previous in vitro study. Except in one patient, the stents were successfully recanalized with one or two attempts. In one patient who underwent ablation in the intrahepatic duct, a 1.8-mm electrode enabled recanalization of the stent. In another who underwent ablation in the extrahepatic duct, however, a larger electrode was required. There were no procedure-related complications. Transpapillary microwave coagulation of occluded stents appears to be an alternative to percutaneous microwave coagulation with an electrode fitting the stent size. The technique might be easier with the use of a redesigned electrode with a guide wire lumen.

Malignant change in the biliary tract after excision of choledochal cyst

Choledochal cyst is a rare congenital condition with a high risk of malignant change if untreated. The risk of malignancy after surgical excision of choledochal cyst is not known. Forty-eight patients with choledochal cysts managed over a 21-year period were reviewed, to determine the risk of malignant change after cyst excision. Thirty-nine of 48 patients had no carcinoma at first admission; their mean(s.d.) age was 20(18) years. Thirty-seven of 39 patients underwent cyst excision and cholecystectomy followed by hepaticoenterostomy. Cyst excision was incomplete in 28 of the 37 patients because dilated portions of the biliary ducts remained proximally and/or distally. In these 37 patients, no carcinoma has developed in the remnant proximal hepatic duct or the terminal bile duct after mean(s.d.) follow-up of 9.1(6.4) years. In the remaining nine patients, biliary carcinoma was diagnosed at the first visit. Six patients died from recurrence with a mean(s.d.) survival time of 13(11) months, while three patients were alive and free from recurrence 2 months, 1 year and 7 years after operation. Malignant change has not been observed after total or subtotal excision of choledochal cysts in this series.

Suture granuloma masquerading as malignancy of the biliary tract

Eighteen years after having a duodenal leiomyosarcoma resected, a patient presented with weight loss, pruritus, and abdominal pain. ERCP was consistent with a cholangiocarcinoma with proximal hepatic duct stricture and nonfilling of the cystic duct. CAT scan revealed no extrinsic masses compressing the gallbladder or biliary tract. At surgical exploration, the patient was found to have a suture granuloma with surrounding fibrosis within the common bile duct. There was no evidence of malignancy.

In vitro hepatic insulin resistance in chronic pancreatitis in the rat

To investigate the effect of chronic pancreatitis (CP) on in vitro hepatic sensitivity to insulin, the suppression of glucagon-stimulated hepatic glucose production (HGP) by insulin was examined during isolated liver perfusion (ILP) in CP and sham-operated rats. CP was induced at laparotomy by infusion of 50 μl 99% oleic acid into the common bile duct during temporary occlusion of the proximal hepatic duct in 250- to 350-g Sprague-Dawley rats. Eight to sixteen weeks later, single-pass ILP was performed on fed animals. Glucagon (100 pg/ml) was infused for 30 min; the final 20 min of perfusion was performed with (a) no insulin, (b) 25 μ/ml insulin, or (c) 100 μU/ml insulin. CP and sham rats demonstrated comparable HGP responses to glucagon during the 0- to 10-min period (5.2 ± 0.5 vs 5.9 ± 0.5 mg/g/min, P = NS). CP rats demonstrated an HGP response to glucagon alone more evanescent than that in sham rats (20–30 min of HGP, 6.6 ± 0.6 vs 9.5 ± 0.4 mg/g/min, P < 0.05). Sham rats showed a dose-dependent inhibition of HGP by insulin, however (percentage 20–30 min of HGP/0–10 min of HGP for 0, 25, and 100 μU/ml insulin: 166 ± 12, 125 ± 7, and 101 ± 5%, P < 0.01), whereas CP rats showed no effect of insulin (130 ± 6, 123 ± 7, 134 ± 7%, P = NS). Pre- and postperfusion liver glycogen contents revealed comparable decreases in liver glycogen in both groups: insulin inhibition of HGP in sham rats was accompanied by higher postperfusion glycogen content. These data demonstrate a loss of insulin-mediated suppression of hepatic glucose production in livers obtained from pancreatitic rats. We conclude that CP is accompanied by a primary hepatic resistance to insulin; this defect may play a role in the etiology of pancreatogenic diabetes.