The role of surgery in Caroli's disease

BACKGROUND: Caroli's disease is a rare congenital disorder characterized by multifocal segmental dilation of the intrahepatic bile ducts. Whether conservative or surgical strategies should be preferred is still a matter of debate. The aim of this study was to evaluate the role of surgery in the management of Caroli's disease. STUDY DESIGN: From April 1998 until August 2005, 12 consecutive patients with Caroli's disease were treated in the Department of General, Visceral, and Transplantation Surgery, University Hospital Essen, Germany. All patients were intended to receive liver resections or liver transplantations. RESULTS: There were seven men and five women, with a median age of 39 years (range 7 months to 70 years). Eight patients had monolobar and four patients had bilobar liver involvement. All patients had a history of recurrent cholangitis, with up to 16 unsuccessful conservative treatment attempts. Nine patients (75%) underwent liver resection and two (17%) had liver transplantation. Intraoperatively, three patients (25%) were found to have cholangiocarcinoma, of which one was unresectable. There was no mortality and only low morbidity (16%) postoperatively. After a median followup of 31 months, 11 patients are well with no recurrent symptoms. CONCLUSIONS: Surgery can offer a definite therapy, with an acceptable morbidity and virtually no mortality in localized Caroli's disease. In diffuse disease, the use of extended resections or liver transplantation can provide good longterm results.     

Caroli's disease: liver resection and liver transplantation. Experience in 33 patients

BACKGROUND: The aim of this study was to review and discuss our observations on 33 patients who underwent surgical treatment for Caroli's disease (CD), focusing on diagnosis, current surgical management, and long-term outcome. METHODS: Between May 1993 and June 2004, 642 liver resections and 286 liver transplantations in 252 patients were performed in our department of surgery. Thirty-three patients were referred to our center for diagnostic and therapeutic management of CD. Prior surgical interventions for hepatobiliary disorders, current diagnostic and surgical procedures, procedure-specific complications, duration of hospital stay, duration of follow-up, outpatient information, and long-term outcome were reviewed. RESULTS: Fifteen male and 18 female patients were treated in this study. Initial symptoms and signs of the disease noted in our patients included right upper quadrant pain, fever, and jaundice. In 2 of the 33 patients, we noted clinical evidence of cirrhosis followed by histologic confirmation. One patient suffered from variceal bleeding. In 26 patients, diagnoses were established by a combined endoscopic retrograde cholangiopancreatography, ultrasonography, and computed tomographic studies. The disease was localized in 25 and diffuse in 8 patients. Liver resection was carried out in 29 patients. Partial hepatectomies were performed in 27 of these 29 at our institution. Two female patients with the diffuse disease underwent orthotopic liver transplantation. Thirteen of the 31 patients who underwent surgery at our institution had an uneventful postoperative course. Fourteen patients had minor postoperative complications and responded well to medical management. Four patients had major complications that required further surgical treatment. Two patients died of complications related to postoperative hemorrhage and sepsis. Two patients with intrahepatic cholangiocarcinoma died because of primary tumor progress. One patient with cholangiocarcinoma died 1 year after a successful left hepatectomy because of metastatic disease recurrence. The long-term results of the 26 surviving patients were assessed during a mean follow-up of 3.7 years (range, 1-11 years). All 26 patients remained free of biliary symptoms or complications. In 25 patients, surgery including liver transplantation was curative. CONCLUSIONS: Partial hepatectomy for localized CD is potentially curative. In patients with diffuse CD, liver transplantation provides gratifying long-term results.     

Monolobar Caroli's Disease and Cholangiocarcinoma

Caroli''s Disease (CD) is a rare congenital disorder characterized by cystic dilatation of the intrahepatic bile ducts. This report describes a patient with cholangiocarcinoma arising in the setting of monolobar CD. In spite of detailed investigations including biliary enteric bypass and endoscopic retrograde cholangiography, the diagnosis of mucinous cholangiocarcinoma (CCA) was not made for almost one year. The presentation, diagnosis and treatment of monolobar CD and the association between monolobar CD and biliary tract cancer are discussed. Hepatic resection is the treatment of choice for monolobar CD.     

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Caroli病是一种先天性的肝内胆管囊性扩张症。外科治疗的目的包括清除病灶,防止胆管癌变;通畅胆汁引流,缓解症状。肝切除是目前最常用的外科治疗方式,对局限性的Caroli病可达到根治的效果,病人术后胆管炎等症状可长期缓解。而对于少部分弥漫型病人,也可选择性清除肝内主要的扩张病灶,并进行密切的随访,如病人进一步进展,肝移植常成为治疗的选择。对于多数弥漫型病人,肝移植是最终的治疗选择,可获得满意的长期效果。     

Liver transplantation for Wilson's disease: long-term results and quality-of-life assessment

BACKGROUND: Wilson's disease associated with severe liver disease is effectively cured by orthotopic liver transplantation (OLT). However, there are also anecdotal reports of improved or resolved neurologic symptoms after OLT in patients with stable or normal liver function. Side effects with conventional chelating agents are common, and it has been suggested that OLT should be considered in patients with severe progressive neurologic symptoms. However, the decision to apply this therapeutic modality to a subgroup of patients without significant liver disease is a quality-of-life issue. METHODS: Long-term follow-up and quality-of-life data were obtained prospectively for 24 patients who underwent OLT between 1988 and 2000 for Wilson's disease associated with severe liver disease. In long-term survivors, quality of life was assessed using the 36-Item Short Form 36 Health Survey Questionnaire. RESULTS: One patient who had multiorgan failure before OLT died within 24 hr of surgery and two patients died within 1 year because of immunosuppressant-related complications. There have been no deaths or graft loss in patients who have undergone transplantation since 1994, and after a median follow-up of 92 months, all survivors have satisfactory graft function (5-year patient and graft survival, 87.5%), with quality-of-life scores (assessed in 86% of survivors) comparable to age- and sex-matched controls from the general population. CONCLUSIONS: The authors' results suggest that liver transplantation can be safely performed in patients with Wilson's disease, with excellent long-term results and quality of life. Further study of the utility of liver transplantation in the management of patients with severe neurologic symptoms is justified.     

Iterative cytoreductive surgery associated with hyperthermic intraperitoneal chemotherapy for treatment of peritoneal carcinomatosis of colorectal origin with or without liver metastases

INTRODUCTION: The aim of this study was to evaluate the results of an aggressive strategy in patients presenting peritoneal carcinomatosis (PC) from colorectal cancer with or without liver metastases (LMs) treated with cytoreductive surgery (CS) and hyperthermic intraperitoneal chemotherapy (HIPEC). PATIENTS AND METHODS: The population included 43 patients who had 54 CS+HIPEC for colorectal PC from 1996 to 2006. Sixteen patients (37%) presented LMs. Eleven patients (25%) presented occlusion at the time of PC diagnosis. Ascites was present in 12 patients (28%). Seventy-seven percent of the patients were Gilly 3 (diffuse nodules, 5-20 mm) and Gilly 4 (diffuse nodules>20 mm). The main endpoints were morbidity, mortality, completeness of cancer resection (CCR), and actuarial survival rates. RESULTS: The CS was considered as CCR-0 (no residual nodules) or CCR-1 (residual nodules <5 mm) in 30 patients (70%). Iterative procedures were performed in 26% of patients. Three patients had prior to CS + HIPEC, 10 had concomitant minor liver resection, and 3 had differed liver resections (2 right hepatectomies) 2 months after CS + HIPEC. The mortality rate was 2.3% (1 patient). Seventeen patients (39%) presented one or multiple complications (per procedure morbidity = 31%). Complications included deep abscess (n = 6), wound infection (n = 5), pleural effusion (n = 5), digestive fistula (n = 4), delayed gastric emptying syndrome (n = 4), and renal failure (n = 3). Two patients (3.6%) were reoperated. The median survival was 38.4 months (CI, 32.8-43.9). Actuarial 2- and 4-year survival rates were 72% and 44%, respectively. The survival rates were not significantly different between patients who had CS + HIPEC for PC alone (including the primary resection) versus those who had associated LMs resection (median survival, 35.3 versus 36.0 months, P = 0.73). CONCLUSION: Iterative CS + HIPEC is an effective treatment in PC from colorectal cancer. The presence of resectable LMs associated with PC does not contraindicate the prospect of an oncologic treatment in these patients.     

Choice of transplantation techniques and indications for liver transplantation in polycystic liver disease in patients with no signs of end-stage liver disease

OBJECTIVE: Since the initiation of the Liver Transplant Program, 500 liver procedures have been performed. Polycystic liver disease (PLD) and polycystic kidney-liver disease (PKLD) have been rare indications for orthotopic liver transplantation (OLT). Only 7 patients (1.4%) underwent transplantation due to PLD and PKLD. MATERIALS AND METHODS: The group consisted of 4 patients who underwent OLT (0.8%) and 3 patients who received simultaneous liver kidney transplantation (LKT; 0.6%). Our objective was to analyze the indications for either OLT or combined LKT as well as indications for surgical techniques during OLT among patients with PLD or PKLD. RESULTS: The main indication for OLT was massive hepatomegaly causing severe physical handicaps, fatigue, and clinically advanced malnutrition. All 3 patients with indications for combined LKT were dialysis-dependent. None of the patients had symptoms of end-stage liver disease and/or hepatic failure. In 4 cases, a portal bypass was applied, and the piggy-back method used in the other 3 cases. The hepatectomy caused no uncommon difficulty. In cases of simultaneous transplantations, the kidney was implanted separately after OLT. All patients are alive following the transplantation; major surgical complications have occurred. CONCLUSIONS: Patients with PLD can undergo OLT safely with good results. They benefit from the relief of abdominal distension and anorexia. Patients with PKLD who are dialysis-dependent should undergo simultaneous LKT. The surgical technique was solely dependent on the intraoperative conditions determined during the dissection phase.     

Endoscopic stenting of the gallbladder for symptomatic gallbladder disease in patients with end-stage liver disease awaiting orthotopic liver transplantation.

Cholecystectomy in patients with advanced cirrhosis is associated with excessive morbidity and mortality. Because open cholecystectomy in patients with Child's class C cirrhosis has a reported mortality rate as high as 83%, symptomatic gallbladder disease in patients awaiting orthotopic liver transplantation (OLT) poses a unique clinical problem. The goal of this study is to determine whether the treatment of symptomatic gallbladder disease with endoscopic stenting of the gallbladder effectively reduces biliary symptoms and complications or the need for cholecystectomy. Thirteen patients with symptomatic gallbladder disease with and without cholelithiasis and advanced cirrhosis who were candidates for OLT underwent placement of a biliary stent from the gallbladder to the duodenum at endoscopic retrograde cholangiography. In each patient, biliary symptoms and complications ceased after stent placement. Seven patients underwent successful OLT 1 to 24 months after the procedure. One patient subsequently became a noncandidate for OLT and died of diabetes complications 3 years after the procedure. Five others are awaiting OLT (6 to 28 months postprocedure). One patient had recurrent pericholecystic fluid collection requiring percutaneous drainage and antibiotic therapy 8 months after the procedure. No patient has had recurrent symptoms, and currently all patients are free of complications. None required surgical intervention of the gallbladder or biliary tree. We conclude that endoscopic stenting of the gallbladder is the preferred treatment for symptomatic gallbladder disease in patients with end-stage liver disease awaiting OLT. This approach is noninvasive, safe, and effective in preventing potential morbidity and mortality.     

Orthotopic liver transplantation for unresectable hepatoblastoma: a single center's experience.

BACKGROUND/PURPOSE: Complete surgical resection after chemotherapy is the definitive treatment for hepatoblastoma. However, orthotopic liver transplantation (OLT) is now accepted as a treatment modality for patients with unresectable tumours. The aim of this study was to review a single center's experience of OLT for unresectable hepatoblastoma. METHODS: A retrospective review of 8 patients with unresectable hepatoblastoma who were referred for liver transplantation was conducted. RESULTS: The patients assessed had an age range of 5 to 105 months at presentation; median, 24 months, (5 boys; 3 girls). Two patients have familial adenomatous polyposis, and one has right hemihypertrophy. All 8 patients had received standard chemotherapy according to SIOP (International Society of Pediatric Oncology) protocols. Extrahepatic metastases were found in 3 patients at diagnosis, but none had detectable metastases at the time of OLT. Four patients continued chemotherapy while awaiting OLT. Three patients received whole grafts, and 5 received reduced grafts. The median follow-up period was 22 months (range, 2 to 78 months). Five patients are alive and well, although 1 patient had a second OLT for biliary cirrhosis secondary to biliary stricture at 6 years. Three patients died: one 26 days post OLT of sepsis and two of disease recurrence at 22 months and 70 months posttransplant. The actuarial survival rate is 88% and 65% at 1 and 5 years, respectively, whereas the overall survival rate is 62.5%. CONCLUSION: OLT for unresectable hepatoblastoma without extra hepatic metastases is highly successful with a low recurrence rate.     

Resection or transplantation for hepatocellular carcinoma in cirrhotic patients: outcomes based on indicated treatment strategy

BACKGROUND: Surgical resection has been the treatment of choice for hepatocellular carcinoma (HCC), but the resection rate remains low in cirrhotic patients and recurrence is common. Unfavorable results compared with benign disease and the shortage of organ donors have led to a restricted indication for orthotopic liver transplantation (OLT) for HCC. STUDY DESIGN: The aim of this study was to analyze the results of our surgical approach to HCC in patients with cirrhosis. The first treatment strategy indicated in these patients was OLT. From January 1990 to May 1999, 85 patients underwent OLT and the remaining 35 had surgical resection. RESULTS: One-, 3-, and 5-year survival rates were 84%, 74%, and 60% versus 83%, 57%, and 51%, respectively, in the OLT and resection groups (p = 0.34). Hepatic tumor recurrence was much less frequent in the OLT group than in the resection group. The 1-, 3-, and 5-year disease-free survival rates were 83%, 72%, and 60% versus 70%, 44%, and 31%, respectively (p = 0.027). In the multivariate Cox regression analysis, macroscopic vascular invasion was the only factor independently associated with death or recurrence after OLT (p = 0.006). After partial liver resection, the tumors significantly associated with mortality and recurrence in the multivariate analysis were solitary or multiple tumors greater than 2cm with microscopic vascular invasion (pathologic pT3) (p = 0.01). CONCLUSIONS: Our results confirm that in cirrhotic patients, OLT may provide better outcomes than liver resection in carefully selected HCC and that longterm survival is similar to the results of OLT in cirrhotic patients without tumors.     

Prolonged disease-free survival after orthotopic liver transplantation plus adjuvant chemoirradiation for cholangiocarcinoma

Orthotopic liver transplantation (OLT) alone for unresectable cholangiocarcinoma is often associated with early disease relapse and limited survival. Because of these discouraging results, most programs have abandoned OLT for cholangiocarcinoma. However, a small percentage of patients have achieved prolonged survival after OLT, suggesting that adjuvant approaches could perhaps improve the survival outcome. Based on these concepts, a protocol was developed at the Mayo Clinic using preoperative irradiation and chemotherapy for patients with cholangiocarcinoma. We report our initial results with this pilot experience. Patients with unresectable cholangiocarcinoma above the cystic duct without extrahepatic or extrahepatic metastases were eligible. Patients initially received external-beam irradiation plus bolus fluorouracil (5-FU), followed by brachytherapy with iridium and concomitant protracted venous infusion of 5-FU. 5-FU was then administered continuously through an ambulatory infusion pump until OLT After irradiation, patients underwent an exploratory laparotomy to exclude metastatic disease. To date, 19 patients have been enrolled onto the study and have been treated with irradiation. Eight patients did not go on to OLT because of the presence of metastasis at the time of exploratory laparotomy (n = 6), subsequent development of malignant ascites (n = 1), or death from intrahepatic biliary sepsis (n = 1). Eleven patients completed the protocol with successful OLT Except for 1 patient, all had early-stage disease (stages I and 11) in the explanted liver. All patients who underwent OLT are alive, 3 patients are at risk at 12 months or less, and the remaining 8 patients have a median follow-up of 44 months (range, 17 to 83 months; 7 of 9 patients > 36 months). Only 1 patient developed tumor relapse. OLT in combination with preoperative irradiation and chemother apy is associated with prolonged disease-free and overall survival in highly selected patients with early-stage cholangiocarcinoma.     

Staging, resectability, and outcome in 225 patients with hilar cholangiocarcinoma

OBJECTIVE: To analyze resectability and survival in patients with hilar cholangiocarcinoma according to a proposed preoperative staging scheme that fully integrates local, tumor-related factors. SUMMARY BACKGROUND DATA: In patients with hilar cholangiocarcinoma, long-term survival depends critically on complete tumor resection. The current staging systems ignore factors related to local tumor extent, preclude accurate preoperative disease assessment, and correlate poorly with resectability and survival. METHODS: Demographics, results of imaging studies, surgical findings, pathology, and survival were analyzed prospectively in consecutive patients. Using data from imaging studies, all patients were placed into one of three stages based on the extent of ductal involvement by tumor, the presence or absence of portal vein compromise, and the presence or absence of hepatic lobar atrophy. RESULTS: From March 1991 through December 2000, 225 patients were evaluated, 77% of whom were seen and treated within the last 6 years. Sixty-five patients had unresectable disease; 160 patients underwent exploration with curative intent. Eighty patients underwent resection: 62 (78%) had a concomitant hepatic resection and 62 (78%) had an R0 resection (negative histologic margins). Negative histologic margins, concomitant partial hepatectomy, and well-differentiated tumor histology were associated with improved outcome after all resections. However, in patients who underwent an R0 resection, concomitant partial hepatectomy was the only independent predictor of long-term survival. Of the 9 actual 5-year survivors (of 30 at risk), all had a concomitant hepatic resection and none had tumor-involved margins; 3 of these 9 patients remained free of disease at a median follow-up of 88 months. The rates of complications and death after resection were 64% and 10%, respectively. In the 219 patients whose disease could be staged, the proposed system predicted resectability and the likelihood of an R0 resection and correlated with metastatic disease and survival. CONCLUSION: By taking full account of local tumor extent, the proposed staging system for hilar cholangiocarcinoma accurately predicts resectability, the likelihood of metastatic disease, and survival. Complete resection remains the only therapy that offers the possibility of long-term survival, and hepatic resection is a critical component of the surgical approach.     

Combined liver resection and reconstruction of the supra-renal vena cava: the Paul Brousse experience

BACKGROUND: Liver tumors with inferior vena cava (IVC) involvement may require combined resection of the liver and IVC. This approach, with its high surgical risks and poor long-term prognosis, was precluded until the development of neoadjuvant chemotherapy, portal vein embolization, reinforced vascular prostheses, and technical advances in liver transplantation. METHODS: We reviewed 22 cases of hepatectomy with retrohepatic IVC resection and reconstruction. The patients had a median age of 51.5 years (range, 32.8-75.3 years). Indications for resection were: liver metastases (n = 9), cholangiocarcinoma (n = 8), hepatocellular carcinoma (n = 2), other cancers (n = 3). The liver resections carried out included 18 first, 3 second, and one third hepatectomy. Segment 1 (caudate lobe) was included in the specimen in 19 cases (86%). Resection concerned 1 to 6 liver segments (median = 5.0). Vascular control was achieved by vascular exclusion of the liver preserving the caval flow (n = 1), standard vascular exclusion of the liver (n = 12), in situ cold perfusion of the liver (n = 9). Ex situ surgery was not necessary in any case. Venovenous bypass was used in 12 cases. The IVC was reconstructed with a ringed Gore-Tex tube graft (n = 10), primarily (n = 8), or by caval plasty (n = 4). A main hepatic vein was reimplanted in 6 cases: into the native IVC (n = 4) or into a Gore-Tex tube graft (n = 2). RESULTS: One patient died (4.5%) due to catheter infection, 7 days after in situ cold perfusion with replacement of the vena cava. Eight patients (36%) had no complications and 14 patients (64%) had 23 complications. In all but 1 case, the complications were transient and successfully controlled. The patients stayed in intensive care for 3.3 +/- 2.0 days and in the hospital for 17.7 +/- 7.8 days. All vascular reconstructions were patent at last follow-up. With median follow-up of 19 months, 10 patients died of tumor recurrence and eleven were alive with (n = 5) or without (n = 6) disease. Actuarial 1-, 3-, and 5-year survival rates were 81.8%, 38.3%, and 38.3%, respectively. CONCLUSIONS: IVC resection and reconstruction combined with liver resection can be safely performed in selected patients. The lack of alternative treatments and the spontaneous poor prognosis justify this approach, provided that surgery is carried out at a center specialized in both liver surgery and liver transplantation. The development of adjuvant chemotherapy regimens is required to improve the long-term results of this salvage surgery.     

Surgical management of hilar cholangiocarcinoma

OBJECTIVE: To assess the surgical management of hilar cholangiocarcinoma over a time period when liver resection was considered standard management. SUMMARY BACKGROUND DATA: Hilar cholangiocarcinoma remains a difficult challenge for surgeons. An advance in surgical treatment is the addition of liver resection to the procedure. However, liver resection in the setting of liver dysfunction caused by biliary obstruction can be associated with increased mortality. METHODS: Between 1997 and 2004, 80 patients with hilar cholangiocarcinoma having surgery were reviewed. Fifty-three patients had attempted curative resections, 14 patients had palliative bypasses, while 13 patients had findings that precluded any further intervention. Twenty-three patients required portal vein resection and reconstruction to achieve negative margins, 3 of which also required reconstruction of the hepatic artery. RESULTS: Patients undergoing resection had a 9% operative mortality, with morbidity of 40%. Patients who demonstrated lobar hypertrophy preoperatively due to tumor involvement of the contralateral liver or induced with portal vein embolization (PVE) had a significantly lower operative mortality than those patients without hypertrophy. Median overall survival in patients resected was 40 months, with 5-year survival of 35%. Negative margins were achieved in 80% of cases and were associated with improved survival. Five-year survival in patients undergoing resection with negative margins was 45%. CONCLUSION: Combined liver and bile-duct resection can be performed for hilar cholangiocarcinoma with acceptable mortality, though higher than that for liver resections performed for other indications. The use of PVE in cases where hypertrophy of the remnant liver has not occurred preoperatively may reduce the risk of operative mortality.     

Congenital intrahepatic bile duct dilatation is a potentially curable disease: long-term results of a multi-institutional study

OBJECTIVE: To report clinical presentation, perioperative outcome, and long-term results of surgical management of congenital intrahepatic bile duct (IHBD) dilatations (including Caroli disease) in a multi-institutional setting. SUMMARY BACKGROUND DATA: Congenital IHBD dilatations are a rare congenital disorder predisposing to intrahepatic stones, cholangitis, and cholangiocarcinoma. The management remains difficult and controversial for bilobar forms of the disease or when concurrent congenital hepatic fibrosis is associated. METHODS: From 1976 to 2004, 33 patients (range 11 to 79 years) were retrospectively enrolled. Disease extent into the liver was unilobar in 26 patients and bilobar in 7 patients (21%). Cholangiocarcinoma, congenital hepatic fibrosis, and intrahepatic stones were present in 2, 10, and 20 patients, respectively. Transplantations or liver resections were performed in 5 and 27 patients, respectively, whereas 1 asymptomatic patient was managed conservatively. RESULTS: Postoperative mortality was nil. Postoperative complications occurred in 16 of 32 operated patients (50%) and additional procedures for residual stones were required in 5 patients. During a median follow-up of 80 months (1 patient being lost for follow-up) no patient developed metachronous carcinoma. Six patients (30%) developed recurrent intrahepatic stones but satisfactory late outcome was achieved in 27 patients (87%). CONCLUSIONS: Partial or total liver resection achieves satisfactory late outcome in congenital IHBD dilatations, when the affection is treated at an early stage and when the extent of liver resection is tailored to intrahepatic disease extent and takes into consideration the presence and severity of underlying chronic liver and renal diseases.     

Multimodality treatment for patients with hepatocellular carcinoma: Analysis of prognostic factors in a single western institution series

There are few Western studies evaluating prognostic factors for survival in patients with hepatocellular carcinoma (HCC) and the influence on survival of various therapeutic options including ortbotopic liver transplantation (OLT). A retrospective analysis was performed of 122 patients with HCC treated at the University of Alabama at Birmingham from January 1990 through December 1999. Clinicopathologic and treatment factors were analyzed with overall survival as the main outcome variable. Median age was 62 years. Most patients were male (74%) and white (79%). Eighty patients (66%) had associated cirrhosis. Sixty-three percent of patients presented with American Joint Committee on Cancer (AJCC) stage III or lV tumors. The median follow-up for survivors was 22 months. The l-, 3-, and 5-year actuarial survival rates for the entire cohort were 46%, 24%, and 17%, respectively. On multivariate analysis, ablative surgery (P = 0.003), AJCC stages I and II (P = 0.0012), and absence of vascular invasion (P = 0.0001) were found to be independent favorable characteristics. Forty-four patients underwent surgical resection (including OLT, n = 20) or a surgical ablative procedure. All but two nonsurgical patients died of disease. The actuarial l-, 3-, and S-year survival rates for this group were 80%, 71%, and 61%, respectively. On multivariate analysis of the surgical group, only vascular invasion was associated with poor prognosis (P = 0.001). OLT was associated with a favorable prognosis on univariate analysis (P = 0.02). Forty percent of patients who received transplants underwent local/regional treatment before transplantation and the outcome in these patients was no different from that in other transplant patients. Surgical treatment is the only potential curative option for HCC, and qualifying for liver transplantation may be a favorable prognostic factor in surgical patients. Local/regional therapy prior to transplantation may provide a bridge to OLT without an increase in tumor-related mortality.     

肝门部胆管癌外科治疗的争议与进展

肝门部胆管癌（HCCA）主要发生在肝总管和左右肝管及其汇合部,其约占胆管癌的50.0%～70.0%,是胆道系统常见的恶性肿瘤,且其发病率亚洲最高。目前,手术是治疗HCCA的唯一有效途径。HCCA因早期症状缺乏或不典型,往往患者出现黄疸或者明显腹痛等临床症状,已到进展期才能明确诊断,且其解剖位置特殊、复杂,肿瘤具有沿胆管生长及浸润性生长的生物学特性,其根治性手术切除率及长期生存率均较低。HCCA的外科治疗已取得了较大的进展,但仍然是外科医生所面临的挑战之一。在外科治疗方面,目前还存在许多争议,新的争议点也在不断出现,譬如是否需要实施术前胆道引流及方式、剩余肝体积不足的解决、肝切除的范围、淋巴清扫范围、联合血管切除及肝移植的价值、微创手术应用等。笔者基于当前最新发表文献探讨这些争议,以期对HCCA正确认识和理解,规范化HCCA的治疗,改善患者预后。     

肝外胆管癌87例临床治疗分析

目的:分析不同部位肝外胆管癌的临床特点、手术治疗效果及预后影响因素。方法:回顾性分析2004年5月—2014年4月收治的87例肝外胆管癌患者资料。结果:87例患者中,肝门胆管癌58例,胆总管下端癌29例,患者均以黄疸为主要表现;56例行根治性手术切除,包括肝门胆管癌33例(56.9%,33/58),胆总管下端癌23例(79.3%,23/29),其余患者行姑息性减黄治疗或未予治疗。肝门胆管癌患者根治术后1、2、3年生存率分别为62.2%、35.1%、27.0%;AJCC分期和淋巴转移是总生存期的独立影响因素,而AJCC分期、淋巴转移、肝脏侵犯是无瘤生存期的独立影响因素(均P0.05)。胆总管下端癌患者术后1、2、3年存活率分别为91.6%、54.2%、37.5%;影响总生存期和无瘤生存期的独立危险因素均为AJCC分期(均P0.05)。肝门胆管癌与胆总管下端癌患者间,全部患者的总生存期、根治术后患者中位生存期与无瘤生存期及非根治术治疗后患者的中位生存期均无统计学差异(均P0.05)。结论:对于不同位置的肝外胆管癌,根治性切除均是有效治疗方式,AJCC分期系统可有效评估预后。     

Surgical management of symptomatic hydatid liver disease: experience from a Western centre

Background

Hydatid liver cysts are rare in North America. The objective of this study was to determine the optimal surgical management for hydatid liver cysts treated outside endemic areas.

Methods

We reviewed the cases of consecutive patients who underwent management of hydatid liver cysts. Radical liver resections were compared with other types of procedures. Clinical presentation, investigations, perioperative outcomes and long-term follow-up were evaluated. We evaluated disease recurrence using the Kaplan–Meier method.

Results

Forty patients underwent surgery for hydatid liver cysts. Most patients had single (68%) right-sided (46%) cysts with a median size of 10 cm. Most (83%) underwent liver resection with or without drainage/marsupialization. Radical liver resection was carried out in 60% (19 major, 5 minor). Additional procedures were required in 50% (biliary fistulization 30%, diaphragmatic fistulization 20% or paracaval location/ fusion 8%). Postoperative complications occurred in 48%. The median follow-up was 39 months. The 3-year recurrence-free survival was significantly different between patients who had radical resection and those who had other procedures (100% v. 71%, p = 0.002).

Conclusion

The surgical management of hydatid liver cysts in North America remains rare and challenging and is frequently associated with fistulizing complications. Excellent long-term outcomes are best achieved using principles of radical liver resection that are familiar to North American surgeons.     

Orthotopic liver transplantation at the Island Hospital. Initial experiences 1985-1990

Between 1985 and 1990 22 orthotopic liver transplantations (OLT) were realized in 19 patients. Active infection and diffuse splanchnic venous thrombosis were the only contra-indications to the intervention. Sixteen patients were transplanted electively; three had to be retransplanted urgently. Three patients had an urgent primary transplant. The incidence of surgical complications related to liver implantation was fair. One patient (5%) developed a late portal vein thrombosis; another patient (5%) had to be retransplanted because of hepatic artery thrombosis. All patients presented one or more major postoperative complications. All, but one, patients had a rejection of the allograft; five of them needed treatment with mono- or polyclonal antilymphocytic sera to reverse the rejection. One patient was retransplanted because of a hyperacute rejection. The six-month survival in this series is 68.5% (13 of 18 patients); one patient died 7 months post-OLT due to a neurological complication of her Wilson disease. Quality of life (from 6 to 64 months post-OLT) is excellent in the 12 long-term survivors. This small experience of the Bernese transplantation program shows that liver transplantation is a safe surgical procedure allowing excellent quality of life in a majority of patients.