A Multicenter, Randomized, Open-Label Study to Compare the Efficacy and Safety of Tacrolimus and Corticosteroids in Combination With or Without Mycophenolate Mofetil in Liver Transplantation Recipients Infected With Hepatitis B Virus.

BACKGROUND: Mycophenolate mofetil exhibits pharmacologic mechanisms different from calcineurin inhibitors. Therefore, the dose of calcineurin inhibitors can be reduced along with side effects for effective immunosuppression. We aimed to evaluate the efficacy and safety of tacrolimus and corticosteroid in combination with or without mycophenolate mofetil in living donor liver transplantation (LDLT) recipients infected with hepatitis B virus (HBV). METHODS: A randomized, open-label, comparative, multicenter, phase IV study was conducted with 119 patients from January 2014 to September 2017. In the full analysis set population, 58 and 59 patients were included in the study group (triple-drug regimen: TacroBell + My-rept + corticosteroid) and the control group (dual-drug regimen: TacroBell + corticosteroid), respectively. In the per protocol set population, 49 and 42 patients were included in the study and control groups, respectively. RESULTS: In the full analysis set population, the incidence of biopsy-proven acute cellular rejection (rejection activity index score ≥4) was 3.4% in the study group; however, this finding was not observed in the control group (P = .468). Hepatitis B virus recurrence was observed in one patient in the control group. No cases of biopsy-proven acute cellular rejection and HBV recurrence were observed in the per protocol set population. The incidences of serious adverse events were 25.9% and 18.0% in the study and control groups, respectively; however, the difference between the groups was not statistically significant (P = .376). CONCLUSION: Although the study involved a small number of patients, the triple-drug regimen can be considered safe and effective for immunosuppression after living donor liver transplantation in patients infected with HBV.

Fates of retained hepatic segment IV and its prognostic impact in adult split liver transplantation using an extended right liver graft.

PURPOSE: When splitting a liver for adult and pediatric graft recipients, the retained left medial section (S4) will undergo ischemic necrosis and the right trisection graft becomes an extended right liver (ERL) graft. We investigated the fates of the retained S4 and its prognostic impact in adult split liver transplantation (SLT) using an ERL graft. METHODS: This was a retrospective analysis of 25 adult SLT recipients who received split ERL grafts. RESULTS: The mean model for end-stage liver disease (MELD) score was 27.3 ± 10.9 and graft-recipient weight ratio (GRWR) was 1.98 ± 0.44. The mean donor age was 26.5 ± 7.7 years. The split ERL graft weight was 1,181.5 ± 252.8 g, which resulted in a mean GRWR of 1.98 ± 0.44. Computed tomography of the retained S4 parenchyma revealed small ischemic necrosis in 16 patients (64.0%) and large ischemic necrosis in the remaining 9 patients (36.0%). No S4-associated biliary complications were developed. The mean GRWR was 1.87 ± 0.43 in the 9 patients with large ischemic necrosis and 2.10 ± 0.44 in the 15 cases with small ischemic necrosis (P = 0.283). The retained S4 parenchyma showed gradual atrophy on follow-up imaging studies. The amount of S4 ischemic necrosis was not associated with graft (P = 0.592) or patient (P = 0.243) survival. A MELD score of >30 and pretransplant ventilator support were associated with inferior outcomes. CONCLUSION: The amount of S4 ischemic necrosis is not a prognostic factor in adult SLT recipients, probably due to a sufficiently large GRWR.

Over 500 Liver Transplants Including More Than 400 Living-Donor Liver Transplants in 2019 at Asan Medical Center.

BACKGROUND: More than 400 liver transplants were performed at Asan Medical Center (AMC) in 2011, and over 500 liver transplants including 420 living-donor liver transplants (LDLTs) were performed in 2019. Herein, we report the methodology of these procedures. METHODS: Since the first adult LDLTs at AMC using the left and right lobes were successfully performed, various innovative techniques and approaches have been developed: modified right lobe, dual graft, donor exchange for ABO incompatibility, expansion of indications and no-touch techniques for hepatocellular carcinoma, intraoperative cine-portogram and additional intervention for large collaterals, management of portal vein thrombosis (PVT) and stenosis, salvage LDLT after major hepatectomy, and timely LDLT for patients with acute-on-chronic liver failure. RESULTS: Four hundred twenty LDLTs in 403 adult and 17 pediatric patients and 85 deceased-donor liver transplants in 74 adult and 11 pediatric patients were performed. The number of deceased-donor liver transplants remained constant since 2011, but the number of LDLTs increased steadily. One hundred thirty patients (25.7%) required urgent liver transplantations and 24 patients with acute-on-chronic liver failure underwent LDLT. PVT including grade 1,2,3, and 4 was reported in 91 patients (18.0%), and Yerdel's grade 2, 3, and 4 PVT was reported in 47 patients (51.6%); all patients with PVT were successfully treated. Adult LDLTs for hepatocellular carcinoma and ABO incompatibility accounted for 52.6% and 24.3% of the cases, respectively. In-hospital mortality in 2019 was 2.97%. CONCLUSION: Continual efforts to overcome challenging problems in LDLT with various innovations and dedication of the team members during the perioperative period to improve patient outcomes were crucial in increasing the number of liver transplantations at Asan Medical Center.

Management of intractable pancreatic leak from iatrogenic pancreatic duct injury following resection of choledochal cyst in an adult patient.

Iatrogenic pancreatic duct injury can occur during resection of the choledochal cyst (CC). We herein present a case of postoperative pancreatic fistula (POPF) developed after resection of the CC in an adult patient with variant anomalous union of pancreatobiliary duct. The 55-year-old female patient underwent surgery after the diagnosis of CC-associated gallbladder cancer. During surgery, the CC mass was accidentally pulled out, by which the intrapancreatic CC portion was torn out from the main pancreatic duct. Since the pancreatic duct stump was not identified due to its small size, repair was not possible. The excavated defect at the pancreas head was closed securely combined with insertion of multiple drains. Postoperative POPF and peripancreatic fluid collection developed and the patient had to be fasted for 4 weeks. She was first discharged at 6 weeks after surgery. At 10 weeks, she was readmitted due to progression of peripancreatic fluid collection, which was controlled by percutaneous drain insertion. At 6 months, she was readmitted again due to repeated progression of peripancreatic fluid collection, which were controlled by endoscopic transmural duodenocystostomy. It took 8 months to resolve the pancreatic duct injury-associated pancreatitis. The experience in this case suggests that iatrogenic pancreatic duct injury during resection of CC can induce catastrophic complications, thus special attention should be paid to prevent pancreatic duct injury.

Pretransplant Hepatic Malignancy Increases Risk of De Novo Malignancy after Liver Transplantation.

BACKGROUND: Hepatocellular carcinoma (HCC) recurrence and development of de novo malignancy (DNM) after liver transplantation (LT) are the major causes of late recipient death. METHODS: We analyzed the incidence of extrahepatic DNM following living donor LT according to the status of pretransplant hepatic malignancy. We selected 2,076 adult patients who underwent primary LDLT during 7 years from January 2010 to December 2016. RESULTS: The pretransplant hepatic malignancy group (n = 1,012) showed 45 cases (4.4%) of the following extrahepatic DNMs: posttransplant lymphoproliferative disease (PTLD) in 10; lung cancer in 10; stomach cancer in 6; colorectal cancer in 5; urinary bladder cancer in 3; and other cancers in 11. The pretransplant no hepatic malignancy group (n = 1,064) showed 25 cases (2.3%) of the following extrahepatic DNMs: colorectal cancer in 3; stomach cancer in 3; leukemia in 3; lung cancer in 3; PTLD in 2; prostate cancer in 2; and other cancers in 9. Incidences of extrahepatic DNM in the pretransplant hepatic malignancy and no hepatic malignancy groups were as follows: 1.1% and 0.5% at 1 year, 3.2% and 2.0% at 3 years, 4.6% and 2.5% at 5 years, and 5.4% and 2.8% at 8 years, respectively (P = 0.006). Their overall patient survival rates were as follows: 97.3% and 97.2% at 1 year, 91.6% and 95.9% at 3 years, 89.8% and 95.4% at 5 years, and 89.2% and 95.4% at 8 years, respectively (P < 0.001). Pretransplant hepatic malignancy was the only significant risk factor for posttransplant extrahepatic DNM. CONCLUSION: Our results suggest that patients who had pretransplant hepatic malignancy be followed up more strictly because they have a potential risk of primary hepatic malignancy recurrence as well as a higher risk of extrahepatic DNM than patients without pretransplant hepatic malignancy.

Is renal replacement therapy necessary in deceased donor liver transplantation candidates with hepatorenal syndrome?: a 2-year experience at a high-volume center.

PURPOSE: Hepatorenal syndrome (HRS) is a fatal complication in patients with end-stage liver disease awaiting liver transplantation (LT). HRS often develops in patients with high model for end-stage liver disease (MELD) score. This study investigated the outcomes of peritransplant management of HRS in a high-volume LT center in Korea for 2 years. METHODS: A total of 157 recipients that deceased donor liver transplantation (DDLT) from January 2017 to December 2018 were included. In-hospital mortality (IHM) was analyzed in relation to pre- and posttransplant application of renal replacement therapy (RRT). RESULTS: Primary diagnoses for DDLT were alcoholic liver disease (n = 61), HBV-associated liver cirrhosis (n = 48), retransplantation for chronic graft failure (n = 24), and others (n = 24). Mean MELD score was 34.6 ± 6.2 with 72 patients at Korean Network for Organ Sharing MELD status 2 (45.9%), 43 at status 3 (27.4%), 36 at status 4 (22.9%), and 6 at status 5 (3.8%). Pretransplant RRT was performed in 16 patients (10.2%) that did not show IHM. Posttransplant RRT was performed in 69 patients (44.0%), for whom IHM incidence was 15.9%. In 53 patients that had undergone de novo posttransplant RRT, IHM incidence increased to 20.8%. IHM in the 88 patients not requiring RRT was 2.3%. CONCLUSION: The majority of adult DDLT recipients in Korean MELD score-based allocation system have very high MELD scores, which is often associated with HRS. Pretransplant RRT appears to improve posttransplant survival outcomes. We thereby recommend that, if indicated, pretransplant RRT be performed while awaiting DDLT.

Changes in the indications for living donor liver transplantation: single-institution experience of 3,145 cases over 10 years.

Background: To understand the changing demands and recent trends in the indications for living donor liver transplantation (LDLT), the present study aimed to analyze the indications for LDLT performed in a high-volume transplantation center over 10 years. Methods: The liver transplantation database at our institution was searched to identify patients who underwent LDLT during a 10-year period from January 2008 to December 2017. The study subjects (n=3,145) were divided into two groups: adult patients (n=3,019, 92.7%) and pediatric patients (n=126, 3.9%). Results: In the adult recipients, the primary diagnoses were hepatitis B virus (HBV)-associated liver cirrhosis (n=1,898, 62.9%), alcoholic liver disease (n=482, 16.0%), hepatitis C virus-associated cirrhosis (n=203, 6.7%), acute liver failure (n=127, n=4.2%), and other diseases (n=157, 5.2%). The mean Model for End-Stage Liver Disease score was 15.6±8.8 (range, 6-40). The proportion of patients with HBV-associated liver disease gradually decreased, but the proportion of those with alcoholic liver disease increased. Hepatocellular carcinoma (HCC) was diagnosed in 1,467 patients (48.6%). The mean proportion of patients with HCC was 63.1% among those with HBV-associated liver disease. In pediatric recipients, the primary diagnoses were biliary atresia (n=51, 40.5%), liver failure of various causes (n=37, 29.4%), metabolic disease (n=22, 17.5%), hepatoblastoma (n=12, 9.5%), and infectious diseases (n=4, 3.2%). Conclusions: Our results showed that there were some significant changes in the indications of LDLT. We believe that our results may reflect the real changes in the indications of LDLT and they will be useful for predicting further changes in the future.

Long-term patency and complications of ringed polytetrafluoroethylene grafts used for middle hepatic vein reconstruction in living-donor liver transplantation.

Background: Homologous vein allografts are adequate for reconstruction of the middle hepatic vein (MHV) in living-donor liver transplantation (LDLT). However, supply is a matter of concern. To replace homologous vein allografts, polytetrafluoroethylene (PTFE) grafts were used. This study aimed to assess the long-term patency rates and complications of PTFE grafts used for MHV reconstruction of LDLT in a high-volume liver transplantation center. Methods: We analyzed the patency rates of PTFE-interposed MHV in 100 LDLT recipients and reviewed complications including PTFE graft migration. Results: The mean age was 53.5±5.4 years and male to female ratio was 73:27. Primary diagnoses were hepatitis B virus infection (n=71) and other (n=28). Mean model for end-stage liver disease score was 16.2±8.3. V5 reconstruction was performed as either single anastomosis (n=85) or double anastomoses (n=14). No V5 reconstruction was required in one patient. V8 reconstruction was performed as single anastomosis, double anastomoses, and no reconstruction in 75, 0, and 25 patients, respectively. During a mean follow-up of 6 years, three recipients required early MHV stenting within 2 weeks. After 3 months, there were no episodes of congestion-associated infarct, regardless of MHV patency. Patency rates of PTFE-interposed MHV were 54.0%, 37.0%, and 37.0% at 1, 3, and 5 years, respectively. Unwanted PTFE graft migration occurred in two recipients, and the actual incidence was 2% at 5 years. Conclusions: PTFE grafts combined with small-artery patches demonstrated acceptably high short- and long-term patency rates. Since the risk of unwanted migration of PTFE graft is not negligibly low, lifelong surveillance is necessary to detect unexpected rare complications.

Hemashield Vascular Graft Is a Preferable Prosthetic Graft for Middle Hepatic Vein Reconstruction in Living Donor Liver Transplantation.

BACKGROUND Because of the supply shortage for homologous vein allografts, we previously used ringed Gore-Tex vascular grafts for middle hepatic vein (MHV) reconstruction in living donor liver transplantation. However, owing to the subsequent unavailability of ringed Gore-Tex grafts, we replaced them with Hemashield vascular grafts. This study aimed to compare the patency of Hemashield grafts with that of ringed Gore-Tex grafts. MATERIAL AND METHODS This was a retrospective double-arm study between the study group that used Hemashield grafts (n=63) and the historical control group that used ringed Gore-Tex grafts (n=126). RESULTS In the Gore-Tex and Hemashield groups, mean age was 53.1±6.2 and 54.3±10.4 years; model for end-stage liver disease score was 16.5±8.3 and 17.5±9.9; and graft-recipient weight ratio was 1.11±0.23 and 1.12±0.25, respectively. In the Gore-Tex graft group, V5 reconstruction was done in single (n=107, 84.9%), double (n=17, 13.5%), and none (n=2, 1.6%). V8 reconstruction was done in single (n=95, 75.4%), double (n=1, 0.8%), and none (n=30, 23.8%). In the Hemashield group, V5 reconstruction was done in single (n=43, 68.3%), double (n=19, 30.2%), and triple (n=1, 1.6%). V8 reconstruction was done in single (n=45, 71.4%), double (n=9, 14.3%), and none (n=9, 14.3%). One-year conduit patency rates in the Gore-Tex and Hemashield groups were 54.8% and 71.6%, respectively (p=0.048). CONCLUSIONS MHV reconstruction using Hemashield vascular grafts demonstrated higher short-term patency rates than those associated with ringed Gore-Tex vascular grafts. We suggest that the Hemashield vascular graft is one of the best prosthetic materials for MHV reconstruction.

Long-term results of conjoined unification venoplasty for multiple portal vein branches of the right liver graft in living donor liver transplantations.

Background: Autologous portal vein Y-graft (PYG) interposition has been the standard procedure for reconstruction of double portal vein (PV) orifices of right liver grafts during living donor liver transplantations. However, it has the disadvantage of being vulnerable to anastomotic stenosis. A refined technique of conjoined unification venoplasty (CUV) was developed to secure PV reconstruction. Methods: We reviewed the surgical outcomes in PV reconstructions using CUVs in 21 cases which were followed up for >3 years. Results: The mean age of recipients was 51.7±4.9 years. The model for end-stage liver disease score was 15.3±6.4. The graft-recipient weight ratio was 1.12±0.21. Recipient PYGs were harvested in all cases. All living donors were blood relatives or relatives through marriage with type III PV anomalies. The number of right liver graft PV orifices was two in 19 cases and three in two cases. For the central intervening vein patch, a PV segment was used in six cases, and an autologous greater saphenous vein patch was used in the remaining 15 cases. The 21 patient cohort displayed a 100% 4-year patient survival rate. None of them underwent any PV interventions including interventional stenting. Serial follow-up computed tomography scans revealed that the reconstructed PV showed early reshaping with a stable streamlined configuration for over 3 years. Conclusions: PV reconstruction using the CUV technique appears to be significantly more effective in preventing PV complications. We believe that CUV is a useful technique to reconstruct right liver grafts with multiple PV orifices.

Cross-sectional analysis of immunosuppressive regimens focused on everolimus after liver transplantation in a Korean high-volume transplantation center.

Background: The mammalian target of the rapamycin inhibitor has dual inhibitory effects on cell growth and angiogenesis. This study aimed to analyze the usage of everolimus on actual immunosuppression (IS) regimens through a cross-sectional study in a high-volume liver transplantation (LT) center. Methods: Our institutional LT database was searched for adult patients who underwent primary LT surgery between January 2010 and December 2016. We identified 2,093 LT recipients with observation periods of 1 to 8 years. Results: We divided the 2,093 recipients into three groups according to the posttransplant follow-up period as follows: group A (12-36 months; n=680), group B (37-60 months; n=560), and group C (>60 months; n=853). The individual IS agents were tacrolimus in 1,807 patients (86.3%), cyclosporine in 169 patients (8.1%), mycophenolate mofetil (MMF) in 1,310 patients (62.6%), and everolimus in 115 patients (5.5%). The most common IS regimens were tacrolimus-MMF combination and tacrolimus monotherapy, regardless of the posttransplant period. Patients with pretransplant malignancies were administered everolimus more frequently than those without pretransplant malignancies (P<0.001). In 102 patients with hepatocellular carcinoma recurrence or de novo malignancies, IS regimens included everolimus-tacrolimus in 41 patients (40.2%), tacrolimus-MMF in 27 patients (26.4%), tacrolimus in 20 patients (19.6%), MMF in 10 patients (9.8%), cyclosporine in three patients (2.9%), and cyclosporine-MMF in one patient (1.0%). Conclusions: Administration of everolimus after LT has been gradually increasing with the expansion of indications in our institutional practice. Currently, the role of everolimus is minimal and not comparable to that of tacrolimus, but it has a unique position in the field of IS after LT.

Absence of antitumor effects of metformin in sorafenib-treated patients with hepatocellular carcinoma recurrence after hepatic resection and liver transplantation.

BACKGROUNDS/AIMS: Hepatocellular carcinoma (HCC) recurrence following hepatic resection (HR) and liver transplantation (LT) remains a great concern. We assessed the antitumor effects of metformin in patients treated with sorafenib for HCC recurrence after HR or LT. METHODS: The two clinical retrospective studies involved metformin therapy of 304 HR patients and 74 LT recipients who were treated with sorafenib. RESULTS: In the study involving patients who had undergone HR, death occurred in 245 of the 304 patients (80.6%) during a median follow-up of 10.2 months after sorafenib administration. The metformin HR group (group 1; n=40) showed no prognostic difference in progression-free and overall survival rates compared with the all-HR control group (group 3; n=241) and propensity score-matched HR control group (group 4; n=80). In the clinical study of recipients exposed to LT, death occurred in 62 of the 74 patients (83.8%) during a median follow-up of 13.6 months (range: 3-76 months) after sorafenib administration. The metformin LT group (group 5; n=14) showed no prognostic difference in progression-free and overall survival rates compared with the all-LT control group (group 7; n=43) and propensity score-matched LT control group (group 8; n=28). CONCLUSIONS: Our clinical studies demonstrated absence of synergistic antitumor effects of metformin. Further high-volume studies are necessary to assess the role of metformin in patients treated with sorafenib for advanced HCC.

Long-term complete response after transcatheter arterial chemoembolization and stereotactic body radiation therapy in a patient with hepatocellular carcinoma at the caudate lobe.

It is expected that a combination of transcatheter arterial chemoembolization (TACE) with stereotactic body radiation therapy (SBRT) may induce synergistic therapeutic effects in hepatocellular carcinoma (HCC), which would result in a high rate of complete therapeutic response. In this study, we present the 5-year clinical course of a patient who had HCC at the caudate lobe, which was treated with TACE and SBRT. A 53-year-old male was diagnosed with an 8 cm-sized HCC at the caudate lobe with compression of the inferior vena cava (IVC). For fear of pulmonary metastasis, we decided to perform sequential TACE-radiotherapy instead of upfront hepatectomy, although the tumor appeared resectable. The first session of TACE, SBRT with 12 fractions, and the second session of TACE were sequentially performed. The patient was administered metformin for chemoprevention. Over the course of a 5-year follow-up, there was no evidence of HCC recurrence. We reported the clinical sequence of a patient showing complete therapeutic response of HCC at the caudate lobe after a combination of TACE and radiotherapy. This type of combined locoregional treatment can be a therapeutic option for HCC at the caudate lobe with marginal resectability.

Post-resection Prognosis of Combined Hepatocellular Carcinoma-Cholangiocarcinoma According to the 2010 WHO Classification.

BACKGROUND: Combined hepatocellular carcinoma and cholangiocarcinoma (cHC) has wide histological diversity. We intended to investigate the prognostic influence of tumor types of cHC. METHODS: We analyzed the clinical and pathological features of cHC along 2010 WHO classification. Study group was 100 cHC patients who underwent primary resection. Control group comprised 200 propensity score-matched patients with intrahepatic cholangiocarcinoma (ICC). RESULTS: In cHC group, tumor diameter was 4.4 ± 2.8 cm and 95 patients had single tumor. They were classified as classical type in 46 and subtypes with stem cell (SC) features in 54. Subtypes with SC features included typical in 16, intermediate cell in 22, and cholangiolocellular in 16. Their 1- and 3-year tumor recurrence rates were 31.7 and 59.8%; and 1- and 3-year patient survival rates were 92.5 and 77.3%, respectively. Tumor recurrence (p = 0.008) and patient survival (p = 0.005) rates were different according to tumor types. Further stratification by subtypes with SC features resulted in prognostic stratification in tumor recurrence (p = 0.045) and patient survival (p = 0.042). However, tumor stage was the only independent risk factor for tumor recurrence and patient survival. Comparing with ICC control group, cHC group showed no significant difference in rates of tumor recurrence (p = 0.523), but better survival outcomes (p = 0.008). Median post-recurrence patient survival period was 20 months in cHC patients and 6 months in ICC patients (p = 0.001). CONCLUSIONS: Our results indicated that there would be close relationship between the post-resection prognosis and histological types according to the 2010 WHO classification, but these histological types did not become an independent prognostic factor.

Preoperative prognostic values of α-fetoprotein (AFP) and protein induced by vitamin K absence or antagonist-II (PIVKA-II) in patients with hepatocellular carcinoma for living donor liver transplantation.

BACKGROUND: Adult living donor liver transplantation (LDLT) is one of the best treatments for hepatocellular carcinoma (HCC). However, when recurrence of HCC after LDLT occurs, the prognosis is poor because of rapid progression. Preoperative level of α-fetoprotein (AFP) and protein induced by vitamin K antagonist-II (PIVKA-II) reportedly correlate with recurrence of HCC after LDLT. METHODS: We examined AFP and PIVKA-II preoperatively as predictors of HCC recurrence in 461 patients who underwent LDLT using right liver graft for HCC from May 2007 to December 2013. RESULTS: Among these, 77 patients (16.7%) who experienced recurrence were retrospectively reviewed. Multivariate analysis revealed tumor size >5 cm, AFP >150 nag/mol and PIVKA-II >100 maul/mol as significant independent risk factors for recurrence. The median time to recurrence was 10 months. The median survival time after recurrence was 26 months, and the 1-, 3- and 5-year survival rates after recurrence were 80.5%, 58%, and 28.3% respectively. CONCLUSIONS: Preoperatively, not only morphology of the tumor but also AFP and PIVKA-II levels can offers important information for the recurrence after LDLT for HCC. Thus, combination of tumor markers might be used for expansion of pre-existing strict selection criteria of liver transplantation for HCC.

Spontaneous rupture of intrahepatic bile duct following portal vein embolization in a patient with perihilar cholangiocarcinoma: a case of successful curative resection.

We herein present a case of spontaneous rupture of intrahepatic bile duct in a patient with perihilar cholangiocarcinoma, which were successfully treated by curative resection. A 60-year-old male patient with perihilar cholangiocarcinoma was decompressed with single percutaneous transhepatic biliary drainage. Two days after right portal vein embolization, the patient suffered from paralytic ileus with marked abdominal distension. Imaging study revealed that marked fluid collection around the liver and whole abdomen, suggesting intrahepatic bile duct rupture. With abdominal drainage and biliary decompression for 2 weeks, the biliary rupture was controlled. To enhance the safety of right hepatectomy, additional right hepatic vein embolization was performed. The patient underwent routine surgical procedures for right hepatectomy, caudate lobectomy and bile duct resection, and recovered uneventfully and discharged 18 days after surgery. This is the first report of a case of spontaneous rupture of intrahepatic bile duct in a patient with perihilar cholangiocarcinoma.