Analysis of patients with biliary atresia coming to liver transplantation

Seventy-two patients with end-stage liver disease underwent liver transplantation between March 1981 and March 1984; 35 (49%) with biliary atresia, the remainder with other disorders. This provided us with a unique opportunity to analyze factors leading to liver failure in patients who had undergone biliary drainage procedures for "uncorrectable" biliary atresia. Four patients in the biliary atresia group were excluded (no corrective procedure done, 3; "correctable" biliary atresia, 1), leaving 31 patients for study. Transplantation survival was 84% for the study group and 73% in children with other primary liver disorders. Most patients were less than 3 months old at the time of initial surgery, had minimal liver disease, and had accepted corrective operations by experienced surgeons. Despite these "favorable" factors, bile drainage was rarely achieved. All patients with continued bile drainage at the time of transplantation had repeated episodes of cholangitis, and cholangitis was associated with cessation of bile drainage in half of those with transient function. Findings at hepatectomy suggested that in four cases where bile drainage was never achieved, reexploration may have been successful. Complications included those associated with hepatic failure and portal hypertension. Of note were a high incidence of bone disease and a 43% incidence of stomal hemorrhage in patients with stomas. The short-term survival after transplantation was comparable in the biliary atresia group and the children with other disorders. This suggests that while the presence of a previous biliary drainage procedure may increase the technical difficulty of transplantation, it does not decrease survival.     

再次肝移植治疗移植肝失功的经验分析

目的 总结再次肝移植治疗移植肝失功的临床经验。方法 回顾分析1993年4月至2005年4月期间施行的9例再次肝移植受者临床资料。再次肝移植的原因包括肝动脉血栓（2／9）,门静脉血栓（1／9）,胆道并发症（6／9）;9例再次肝移植均为尸肝移植,3例采用经典原位肝移植,6例采用背驮式肝移植,6例采用Roux-en-Y胆肠内引流,1例供受体门静脉间用供体脾静脉搭桥,1例供体肝动脉与供体腹主动脉之问用供体脾动脉搭桥。结果 全组无手术死亡,5例术后未出现并发症,1例术后门静脉吻合口狭窄,3例术后6个月内死亡。结论 首次肝移植后由于胆道和血管并发症导致移植肝失功是再次肝移植的主要适应证,不失时机地进行再次肝移植是治疗移植肝失功惟一有效的方法。     

Therapeutic strategies in symptomatic portal biliopathy

SUMMARY BACKGROUND DATA: Chronic portal obstruction can lead to formation of portal cavernoma (PC). Half of all patients with PC will develop cholestasis, termed portal biliopathy, and some will progress to symptomatic biliary obstruction. Because of the high hemorrhage risk associated with biliary surgery in patients with PC, the optimal therapeutic strategy is controversial. METHODS: Retrospective review of a single hepatobiliary center experience, including 64 patients with PC identified 19 patients with concurrent symptomatic biliary obstruction. Ten patients underwent initial treatment with a retroperitoneal splenorenal anastomosis. For the remaining 9 patients, portal biliopathy was managed without portosystemic shunting (PSS). Outcomes, including symptom relief, the number of biliary interventions, and survivals, were studied in these 2 groups. RESULTS: Within 3 months of PSS, 7 of 10 patients (70%) experienced a reduction in biliary obstructive symptoms. Five of these 10 patients subsequently underwent uncomplicated biliary bypass, and none has recurred with biliary symptoms or required biliary intervention with a mean follow-up of 8.2 years. For patients without PSS, repeated percutaneous and endobiliary procedures were required to relieve biliary symptoms. Four of the 9 patients with persistent PC required surgical intrahepatic biliary bypass, which was technically more challenging. With a mean follow-up of 8 years, 1 of these 9 patients died of severe cholangitis, 1 remained jaundiced, and 7 were asymptomatic. CONCLUSIONS: This study, which represents the largest published experience with the surgical treatment of patients with symptomatic portal biliopathy, indicates that retroperitoneal splenorenal anastomosis improves outcomes and should be the initial treatment of choice.     

Multidetector computed tomography angiography in the evaluation of potential living donors for liver transplantation: single-center experience in China

Objective

To evaluate relevant arterial, hepatic, and portal venous anatomy using multidetector computed tomography (CT) angiography in potential living liver donors at a single liver transplantation center in China.

Methods

One hundred two consecutive potential liver donors underwent CT angiography in the arterial, portal, and hepatic venous phases with a 16-row CT scanner. All source and reconstructed images were evaluated for hepatic vasculature anatomy by an experienced radiologist and a surgeon in consensus. The anatomic variants of arterial system, portal venous system, and hepatic veins were characterized according to the classification system of Michels, Akgul, and Nakamura respectively. In 42 donors of right hepatic lobectomy, CT findings were compared with the results of surgery.

Results

Of 102 candidates, 63 had type I, 8 type II, 12 type III, 3 type IV, 11 type V, 2 type VI, 2 type VIII, and 1 type IX hepatic arterial anatomy. According to the classification of the portal venous system created by Akgul, type A was seen in 81 subjects. Type B, type C, and type E were revealed in 15, 4, and 2 subjects, respectively. According to the classification of the right hepatic drainage pattern by Nakamura, type 1 drainage was seen in 71 subjects (69.6%), type 2 in 22 candidates (21.6%), and type 3 in 9 subjects (8.8%). Forty five right inferior hepatic veins were identified in 41 potential donors, and 68.9% of these veins were larger than 5 mm in diameter. CT angiography findings were confirmed in all donors who underwent operations.

Conclusions

Multidetector CT angiography can successfully show the relevant hepatic vascular anatomy in potential liver donors.     

彩色多普勒超声在检测正常移植肝脏及其并发症中的应用价值

目的 探讨彩色多普勒超声在检测正常移植肝脏及并发症中的应用价值。方法 用彩色多普勒超声观察41例肝移植患者肝脏动、静脉血流变化及胆管、肝实质声像图改变。主要观察最大血流速度、时间平均血流速度、阻力指数及胆管的内径。结果 41例中17例（41．5％）发生移植物排斥反应，其彩色多普勒显像（CDFI）显示13例（76．4％）门静脉平均血流速度减低；15例（88．25％）肝静脉波幅降低，呈单相锯齿波或负二相波；9例（52．9％）肝动脉阻力指数升高；5例（29．4％）胆管轻度扩张。41例中5例（12．2％）肝动脉血栓形成，主要表现为门静脉周围检测肝动脉血流信号消失。检测中要注意操作技术及一些假阳性和假阴性影响因素。肝移植术后3例（17．3％）早期轻度胆管扩张，2周内恢复。20例（48．8％）胆管扩张明显并逐渐加重，原于胆管狭窄（2例）、胆管结石（15例）及其他（3例）。结论 彩色多普勒显像技术对正常移植肝脏的确定及并发症的检出具有重要价值；对提高术后存活率有重要意义。     

Liver transplant donor candidates: associations between vascular and biliary anatomic variants.

Our objective was to investigate the coexistence of vascular and biliary anatomic variants, the latter of which are known to increase the risk of biliary complications in living liver donor transplantation. A total of 108 consecutive liver donor candidates were examined by magnetic resonance (MR) imaging that included 2 MR cholangiography methods, T2-weighted MR cholangiography and mangofodipir-enhanced T1-weighted three-dimensional (3D) MR cholangiography, as well as gadolinium-enhanced MR angiography and venography of the liver. Images were interpreted by at least 2 investigators in consensus for definition of hepatic arterial, portal venous, and biliary anatomy. A subset of 51 subjects underwent laparotomy for right hepatectomy. Of the 108 subjects examined, 50 (46%) demonstrated normal hepatic artery, portal vein, and biliary anatomy. Variants of the hepatic artery were found in 27 of 108 (25%) subjects, of the portal vein in 12 of 108 (11%) subjects, and of the bile ducts in 30 of 108 (28%) subjects. Of the 27 subjects with hepatic arterial variants, 8 (30%) also had variant biliary anatomy. The association between hepatic arterial variants and biliary variants was not statistically significant (P >.5). However, of the 12 subjects with portal vein variants, 7 (58%) had biliary variants, and in 6 of 7 cases, the right posterior hepatic duct was anomalous. By chi-square analysis, the association between portal venous and biliary variants was significant (P =.012). In conclusion, over half of subjects with portal vein variants were found to have anomalous biliary anatomy, which always involved the hepatic ducts of the right lobe. The association between portal venous and biliary variants is statistically significant, while there is no significant association between hepatic arterial and biliary variants.     

Indication and operative techniques of extended right hepatic lobectomy for advanced gallbladder cancer

Gallbladder cancer extends directly to the hepatic hilum and/or the right portal pedicle in the hepatic hilum type and the bed and hilum type of disease. Extended right hepatic lobectomy (ERHL), caudate lobectomy, lymph node dissection, and biliary reconstruction are necessary for radical resection of the tumor. It was previously thought that this extensive surgery carried high risk, with a hospital death rate of 20%, and had little survival benefit. However, it is now feasible with lower risk due to improvement in biliary decompression techniques, prevention of intrahepatic segmental cholangitis, introduction of preoperative portal embolization, etc. Long-term survival has been achieved after surgery unless there is hepatic, peritoneal, or paraaortic metastasis. Hilar hepatic involvement is more advanced in gallbladder cancer than in bile duct cancer, and portal vein resection and reconstruction are inevitable. All six such patients in our department over the past two years underwent concomitant portal reconstruction and have survived postoperatively.     

Histologic oddities at the porta hepatis in biliary atresia

Purpose

Highly unusual histologic findings at the porta hepatis in 3 infants who underwent Kasai portoenterostomy for biliary atresia are reported.

Methods

Portoenterostomy was performed using a standard operative technique. Serial transverse sections of the excised portal plate were examined by light microscopy along with sections from the distal extrahepatic biliary remnants, gallbladder, and liver biopsy.

Results

Of 61 consecutive infants who underwent Kasai portoenterostomy for biliary atresia, 3 were found to have highly unusual histologic features at the porta hepatis. All had type 3 biliary atresia. Two had hilar biliary ductules lined in part by squamous epithelium, and the third had a focus of mature hyaline cartilage surrounded by perichondrium adjacent to biliary ductules. In each case, these unusual histologic features were localized to the porta hepatis in the region of the transected portal plate.

Conclusions

The presence of hyaline cartilage at the portal plate is likely to be an expression of defective morphogenesis, thus supporting the concept of disordered embryogenesis in the etiology of biliary atresia. Squamous epithelium within biliary ductules might also reflect a similar mechanism but could alternatively be an unusual metaplastic response to inflammation at this site.     

Intrahepatic Segmental Portal Vein Thrombosis After Living-Related Donor Liver Transplantation

Background

Intrahepatic segmental portal vein thrombosis after living-related liver transplantation (LRLT) is uncommon. The cause remains unclear.

Methods

After providing written informed consent, 25 recipients receiving LRLT at our institution from January 2011 to September 2013 were enrolled in this study. We performed triphase computerized tomographic (CT) study of the liver graft of each recipient 1 month after LRLT. The patencies of hepatic artery, portal vein, and hepatic vein were evaluated in detail. The triphase CT scans of the liver of each donor before transplantation also were reviewed. Thrombosis of the intrahepatic segmental portal vein was defined as the occlusion site of the portal vein being intrahepatic. Extrahepatic portal vein thrombosis was excluded in this study.

Results

Among the 25 patients, 2 (8%) developed thrombosis of intrahepatic segmental portal vein. One 47-year-old man received LRLT for hepatitis B viral infection–related liver cirrhosis (Child-Pugh class C) with 3 hepatocellular carcinomas (total tumor volume <8 cm). Another 53-year-old man received LRLT for alcoholic liver cirrhosis (Child-Pugh class C). Both had developed progressive jaundice and cholangitis 1 month after surgery. Intrahepatic biliary stricture was found on the follow-up magnetic resonance images. However, liver triphase CT study demonstrated occlusion of intrahepatic portal vein of segment 8 in each patient. Radiologic interventions and balloon dilatation therapy via percutaneous transhepatic biliary drainage route improved the symptoms and signs of cholangitis and obstructive jaundice for both.

Conclusions

Thrombosis of intrahepatic segmental portal vein is not common but is usually associated with complications of intrahepatic bile duct. Early detection is important, and follow-up CT study of liver is suggested.     

磁共振成像和磁共振胆道成像对肝门部胆管癌诊断和手术可切除性的估价

目的 探讨磁共振成像（MRI）和磁共振胆道成像（MRC）联合应用对肝门部胆管癌的诊断及手术可切除性的估价。方法 从1997年3月至1999年5月在对280例阻塞性黄疸进行了经肝脏MRI和MRC检查中,有38例诊断为肝门部胆管癌（13.6%,38/280),并经手术和病理证实。结果 38例肝部部胆管癌中,24例MRI平扫,在肝门区可见较高信号肿块影,14例行增强扫描,8例可见病灶呈不均匀性强化,MRI肿瘤的显示率为84%（32/38）。MRI发现4例肝转移,7例门静脉主干或分支受累,MRC均可见肝门区阻塞,肝内胆管扩张,MRC定位准确性为100%。本组切除率为24%（9/38）,其中I型4例,Ⅱ型3例,Ⅲ型a、b各1例,Ⅳ型无1例能切除,本组手术不能切除的原因主要为肝转移、肝门部肿块已侵及门静脉或肝动脉,结论 MRI和MRC联合应用有助于肝门部胆管癌的诊断及评估手术的可切除术。     

Surgical treatment of choledochal cysts

Biliary cystic disease is uncommon in Asia and very rare in Europe and the Americas. Patients with biliary cysts may present as infants, children, or adults. When patients present as adults, they are more likely to have stones in the gallbladder, common duct, or intrahepatic ducts and to present with biliary colic, acute cholecystitis, cholangitis, or gallstone pancreatitis. With increasing age at presentation, the risks of intrahepatic strictures and stones, segmented hepatic atrophy/hypertrophy, secondary biliary cirrhosis, portal hypertension, and biliary malignancy all increase significantly. Factors to be considered when performing surgery on patients with biliary cystic disease include: (1) age, (2) presenting symptoms, (3) cyst type, (4) associated biliary stones, (5) prior biliary surgery, (6) intrahepatic strictures, (7) hepatic atrophy/hypertrophy, (8) biliary cirrhosis, (9) portal hypertension, and (10) associated biliary malignancy. In general, regardless of age, presenting symptoms, biliary stones, prior surgery or other secondary problems, surgery should include cholecystectomy and excision of extrahepatic cyst(s). With respect to the distal bile duct, the surgical principle should be excision of a portion of the intrapancreatic bile duct with care to not injure the pancreatic duct or a long common channel. Resection of the pancreatic head should be reserved for patients with an established malignancy. With respect to the intrahepatic ducts, surgery should be individualized depending on whether (1) both lobes are involved, (2) strictures and stones are present, (3) cirrhosis has developed, or (4) an associated malignancy is localized or metastatic. When the liver is not cirrhotic, hepatic parenchyma should be preserved even when strictures and stones are present. If cirrhosis is advanced, hepatic transplantation may be indicated, but this sequence of events is unusual. If a malignancy has developed, oncologic principles should be followed. Whenever possible, resection of a localized tumor including adjacent hepatic parenchyma and regional lymph nodes should be performed.     

Hepatocellular carcinoma with biliary tumor thrombi: aggressive operative approach after appropriate preoperative management

BACKGROUND: The aim of this study was to clarify clinicopathologic characteristics of, and to evaluate an aggressive treatment strategy for, hepatocellular carcinoma with biliary tumor thrombi. METHODS: From 1980 to 1999, a total of 132 patients underwent hepatectomy for hepatocellular carcinoma. Of these, 17 patients had macroscopic biliary tumor thrombi and were retrospectively analyzed. RESULTS: The operative procedures included right hepatic trisegmentectomy (n = 1), right or left hepatic lobectomy (n = 11), and segmentectomy or subsegmentectomy (n = 5). In 13 patients, tumor thrombi extended beyond the hepatic confluence and was treated by thrombectomy through a choledochotomy in 8 patients and extrahepatic bile duct resection and reconstruction in 5 patients. The 3- and 5-year survival rates were 47% and 28%, respectively, with a median survival time of 2.3 years. These survival rates were similar to those achieved in 115 patients without biliary tumor thrombi. In a multivariate analysis, expansive growth type and solitary tumors were independent prognostic variables for favorable outcome after operation, whereas biliary tumor thrombi was not a significant prognostic factor. CONCLUSIONS: Surgery after appropriate preoperative management of hepatocellular carcinoma with biliary tumor thrombi yields results similar to those of patients without biliary involvement. Hepatectomy with thrombectomy through a choledochotomy appears to be as effective as a resection procedure.     

劈离式肝移植术后胆管并发症

目的 探讨体外劈离式肝移植术后胆管并发症的危险因素及其防治措施.方法 2006年6月至2010年9月,我院共施行劈离式肝移植术33例,其中1例于术后10 d死亡,予以排除.其余32例患者中男性18例,女性14例,平均年龄33.4岁(6个月～65岁).胆管重建方式胆管端端吻合20例,胆肠吻合12例.胆管并发症的诊断依靠T管造影、经皮经肝胆管造影(PTC)、经内镜逆行胆胰管造影、磁共振胰胆管造影(MRCP)等方法.胆管并发症定义为存在需要外科、介入、内镜等方法治疗的胆漏或胆管狭窄.结果 受者中位随访时间13.5个月(3～54个月).32例患者中11例患者发生12次胆管并发症(37.5％),其中肝断面胆漏3例(9.3％),胆管吻合口漏4例(12.5％),左肝管残端漏1例(3.1％),胆管吻合口狭窄1例(3.1％),缺血性胆管狭窄3例(9.3％).8例发生胆漏的受者中6例经手术或穿刺放置引流后痊愈,2例因腹腔内感染死亡.单因素分析表明,移植物类型、胆管重建方式等均不是肝断面胆漏的危险因素.结论 与全肝移植和活体肝移植相比,劈离式肝移植术后胆管并发症尤其是胆漏更为常见.进一步防治胆管并发症是改善劈离式肝移植预后的重要因素.     

Intrahepatic cholangiojejunostomy for unresectable malignant biliary tumors with obstructive jaundice

We reviewed our experience with intrahepatic cholangiojejunostomy as a palliative therapy for patients with unresectable malignant diseases involving the ductal confluence or the common hepatic duct. Fifteen patients with malignant biliary obstruction were treated by cholangiojejunostomy at our hospital. Two patients had intrahepatic cholangiocarcinoma, 7 had gallbladder carcinoma, 5 had bile duct carcionoma, and 1 had pancreatic carcinoma. Segment III cholangiojejunostomies were performed in 14 patients and segment V cholangiojejunostomy in 1. Contraindications for surgical resection were locoregional invasion of tumors involving the proper and/or common hepatic artery and portal vein in 15 patients and the presence of hepatic metastases in 6 patients. Liver metastases were detected in 5 of the 7 patients with gallbladder carcinoma. Postoperative complications occurred in 2 patients (13%), but there was no leakage of the cholangioenteric anastomosis in our series. There was no operative mortality after cholangiojejunostomy. Of the 9 patients who survived for more than 6 months after surgery, 7 showed a significant improvement in performance status (PS) (82 ± 10%) 3 months after the surgery compared with the preoperative PS (70 ± 7%). Four of the 9 patients had recurrent cholangitis as a late complication, but 4 were completely free from jaundice. Median survival after cholangioenteric bypass was 9 months (range, 2–25 months). With respect to tumor location, the median survival time was 4 months (range, 2–25 months) in patients with gallbladder carcinoma and 15.5 months (range, 12–22 months) in those with bile duct carcinoma. While the median survival period after surgery was only 3 months (range, 2 to 8 months) in the 5 patients with hepatic metastases from gallbladder carcinoma, 2 patients without liver metastasis survived for 9 and 25 months after segment III cholangioenteric bypass. In conclusion, cholangiojejunostomy can provide useful palliation for malignant biliary obstruction when combined with careful patient selection. Received: September 5, 2000 / Accepted: November 8, 2000     

Interventional radiologic procedures in liver transplantation

Postoperative biliary and vascular complications contribute significantly to morbidity and mortality in liver transplantation. Interventional radiologists are an integral part of the multidisciplinary team necessary for optimizing the management of these complications. During a 15-year period, 39 cadaveric and 25 living related liver transplantations were performed at the Chang Gung Memorial hospital, Taiwan. Of 64 liver transplant recipients, 9 (3 adult and 6 pediatric) underwent 13 interventional radiological procedures for the treatment of biliary sludge-casts (n = 2), bile duct occlusion or stenosis (n = 2), hepatic veins thrombosis (n = 1), hepatic veins stenosis (n = 1), portal vein stenosis with splenorenal shunting (n = 1), biloma (n = 1), and infected fluid collection or ascites (n = 4). Antegrade or retrograde interventional approach was used to successfully treat all biliary complications, and all percutaneous drainage procedures were effective in the control of intra-abdominal fluid collections. Portal vein stenosis was treated by balloon dilatation, and the associated splenorenal shunt was closed by metallic coil embolization via transhepatic catheterization of the portal vein. Hepatic vein stenosis was effectively treated by balloon dilatation and expandable metallic stent deployment via transfemoral and jugular venous approaches, respectively. Hepatic vein thrombosis was only partially lysed by transvenous streptokinase administration, and surgical thrombectomy was needed to achieve complete recanalization. The total success rate of the interventional procedures was 92 % with no procedure-related complications. The overall survival rate in this series is 89 %, and all patients who underwent living related liver transplantation maintain to date a 100 % survival rate. We can conclude that interventional radiological procedures are very useful for managing biliary and vascular complications after liver transplantation. These techniques provide a cure in most situations, thus obviating the need for further surgical intervention or re-transplantation. Received: 6 March 2000 Accepted: 5 May 2001     

Biliary complications following adult right lobe ex vivo split liver transplantation.

Biliary complications are common following split liver transplantation (SLT). We analyzed the incidence, treatment, and outcome of biliary complications following adult right lobe ex vivo SLT performed between November 1992 and January 2005. There were 72 patients, of which 70 were analyzed. Early postoperative deaths resulted in 2 being excluded from the analysis. There were 44 males (median age, 48 yr; range, 19-70 yr). Biliary reconstruction was by duct-to-duct (DD) anastomosis in 52 (74%) and Roux-en-Y hepaticojejunostomy (RYHJ) in 18 (26%) patients. Until mid-2001, no T-tube was used for DD anastomosis (DD/non-T-tube) in 26 (37%) patients; subsequent to this, DD over a T-tube (DD/T-tube) was performed in 26 (37%) patients. Eighteen (26%) biliary complications occurred in 16 patients. Two anastomotic leaks of RYHJ were associated with hepatic artery thrombosis. The most frequent biliary complication was parenchymal radical leak from the transected liver surface (11%; 8/70), with anastomotic leaks in 6% (4/70) and strictures in 4% (3/70). There were also 2 cases of biliary leaks from T-tube exit site following T-tube removal, and 1 leak from the donor cystic duct stump. DD anastomosis without a T-tube was associated with a higher rate of cut surface and anastomotic biliary leaks (7/26), compared to the DD/T-tube group (1/26; P = 0.05). Six patients (9%) died following biliary complications, including 3 due to cut surface leaks in the DD/non-T-tube group and 2 cases with fatal biliary peritonitis following T-tube removal. A patient in the RYHJ group died due to biliary sepsis associated with hepatic artery thrombosis. In conclusion, biliary complications following right lobe ex vivo SLT are associated with significant morbidity and mortality. Our results suggest that T-tube biliary drainage of DD anastomosis may reduce parenchymal cut surface and biliary anastomotic leaks. However, bile leak following T-tube removal could lead to potentially fatal biliary peritonitis, which should always be anticipated and treated promptly.     

Ⅲ型肝门部胆管癌的外科治疗（附35例分析）

目的总结Ⅲ型肝门部胆管癌的手术经验。方法回顾性分析我院1999年1月至2006年12月，行手术切除的35例Ⅲ型肝门部胆管癌的临床资料。Ⅲa型16例，行肝门部胆管切除8例，行联合右半肝＋右侧尾状叶切除7例，行联合右半肝＋尾状叶切除、门静脉分叉部切除主干左支吻合1例。Ⅲb型19例，行肝门部胆管切除8例，行联合左半肝＋左侧尾状叶切除9例，行联合左半肝＋尾状叶切除、门静脉分叉部切除主干右支吻合1例．行联合左半肝＋尾状叶切除、门静脉分叉部切除主干右支吻合、肝固有动脉分叉部切除主干右支吻合1例。结果本组32例获得随访，随访时间18～113个月。肝门部胆管切除病例术后病理根治性切除率为37．5％，联合肝叶切除病例术后病理根治性切除率73．7％，3例联合肝叶切除＋血管切除病例均获术后病理根治性切除。肝门部胆管切除术后并发症发生率为31．3％，联合肝叶切除组术后并发症发生率为31．6％。3例联合肝叶切除＋血管切除病例术后均无胆肠吻合口漏、肝断面坏死、胆漏等严重并发症。结论联合肝叶切除，必要时行受累分叉部血管切除重建，有益于提高Ⅲ型肝门部胆管癌的根治性切除率，且不增加术后并发症的发生率。     

Results of a new strategy for reconstruction of biliary injuries having an isolated right-sided component

Poor results after repair of biliary injuries arc most common when injuries are above the bifurcation of the left and right hepatic ducts or involve aberrant ducts. We have developed a novel approach to the right-sided component of such injuries. Preoperatively all isolated sections of the biliary tree are intuhated percutaneously. At surgery the left duct is found by the Hepp-Couinaud approach. Dissection is continued to the right, staying within the coronal plane of the left hepatic duct, and continuing across the gallbladder plate into segment 5 between the hepatic parenchyma and the Wallerian sheath of the right portal pedicle. Hepatic parenchyma, anterior to the sheath, is resected. After a length of portal pedicle is exposed, right-sided bile ducts are opened on their anterior surface, using the percutaneous transhepatic stents as a guide, and hepaticojejunostomy is performed. Twenty-three patients were treated from May 1993 to February 1999. Injury types and (number uf patients) were as follows: B (n = 2), C (n = 5), E4 (n = 10), and E5 (n = 6). There were no perioperative deaths. Follow-up ranged from 8 months to 7 years (median 3 years). There have been no cases of restricture, reoperation, or jaundice, and no interventional procedures. Serum bilirubin is normal in all patients. Alkaline phosphatasc is normal or less than two times the normal value in 21 of 22 living patients. This novel approach brings the benefits of the Hepp-Couinaud approach to the right hepatic ducts. Very satisfactory results were obtained in the most severe types of biliary injury. Presented at the Forty-First Annual Meeting of The Society for Surgery of the Alimentary Tract, San Diego, Calif., May 21–24, 2000.     

Living related donor liver transplantation in children

Objective

The objective of this study was to report our experience with pediatric orthotopic liver transplantation (OLT) with living related donors.

Methods

We performed a retrospective chart analysis of 121 living related donor liver transplantations (LRDLT) from June 1998 to June 2010.

Results

Indications were biliary atresia (BA; n = 81), primary sclerosing cholangitis (n = 5), α-1 antitrypsin deficiency (n = 4); cholestasis (n = 9), fulminant hepatic failure (n = 8), autoimmune hepatitis (n = 2), Alagille syndrome (n = 4), hepatoblastoma (n = 3), tyrosinemia (n = 2), and congenital hepatic fibrosis (n = 3). The age of the recipients ranged from 7-174 months (median, 22) and the weights ranged from 6-58 kg (median, 10). Forty-nine children (40.5%) weighed ≤10 kg. The grafts included the left lateral segment (n = 108), the left lobe (n = 12), and the right lobe (n = 1). The donors included 71 mothers, 45 fathers, 2 uncles, 1 grandmother, 1 grandfather, and 1 sister with a median age of 29 years (range, 16-53 ys) and a median weight of 68 kg (range, 47-106). Sixteen patients (12.9%) required retransplantation, most commonly due to hepatic artery thrombosis (HAT; n = 13; 10.7%). The other complications were biliary stenosis (n = 25; 20.6%), portal vein thrombosis (PVT; n = 11; 9.1%), portal vein stenosis (n = 5; 4.1%), hepatic vein stenosis (n = 6; 4.9%), and lymphoproliferative disorders (n = 8; 6.6%). The ultimate survival rate of recipients was 90.3% after 1 year and 75.8% after 3 years. Causes of early death within 1 month were HAT (n = 6), PVT (n = 2), severe graft dysfunction (n = 1), sepsis (n = 1), and intraoperative death in children with acute liver failure (n = 2). Causes of late deaths included lymphoproliferative disease (n = 3), chronic rejection (n = 2), biliary complications (n = 3), and recurrent disease (n = 3; hepatoblastoma and primary sclerosing cholangitis).

Conclusions

Despite the heightened possibility of complications (mainly vascular), LRDLT represented a good alternative to transplantation from cadaveric donors in pediatric populations. It was associated with a high survival ratio.     

Extrahepatic portal vein thrombosis is associated with an increased incidence of cholelithiasis

Background/Purpose

The effect of portal flow deprivation to the liver on bile composition and the biliary system remains undefined in children. This report catalogues the authors’ experience with biliary tract problems in children with extrahepatic portal vein thrombosis (EHPVT).

Methods

Twenty-nine children with symptomatic idiopathic EHPVT were evaluated for the Rex shunt procedure (mesenterico-left portal bypass) over a 4-year period. The authors retrospectively reviewed all operative reports and pre- and postoperative abdominal ultrasound findings with regard to associated congenital anomalies and abnormal biliary tract findings.

Results

Seven of the 29 patients with EHPVT (24%) had associated nonbiliary congenital abnormalities. Twenty-four of 29 (83%) patients had detectable biliary tract pathology by ultrasound examination. Biliary symptoms developed in 3 of the 9 (33%) patients with either stones or sludge (10.3% of all patients). Two patients were treated by cholecystectomy. There was no statistical correlation between biliary tract pathology and the age of presentation, symptoms of portal hypertension, gender, or underlying medical condition.

Conclusions

The authors have noted a high incidence of biliary tract pathology in patients with EHPVT compared with the normal population and a 10% incidence of symptomatic biliary pathology in this series.