Innovative modification of the hepatic portoenterostomy. Our experience of treating biliary atresia

We modified conventional hepatic portoenterostomy by pulling the remnant fibrous mass caudally and placing 5 or 6 numbered and individually clamped absorbable 5-0 double-needle sutures horizontally in the liver surface of the posterior side of the remnant fibrous mass before excision and apposing the posterior and anterior margins of the hepatic portoenterostomy to resemble a flattened isosceles triangle. Our technique was used to treat 14 cases of biliary atresia. Good postoperative biliary excretion (serum total bilirubin, <2 mg/dL) was achieved in 13 (92.9%) of 14 patients.     

Technical aspects of hepatic portal dissection in biliary atresia.

During the past 8 yr, 37 patients with a noncorrectable type of biliary atresia have undergone hepatic portoenterostomy or portocholecystostomy at the Kobe Children's Hospital. The hepatic portal dissections employed in this series were classified as "supraportal" (9 procedures), "portal" (25 procedures), and "infra-portal" (3 procedures) based on the level at which the fibrous mass at the porta hepatis was transsected as determined by the operative record and the pathologic findings. Successful biliary drainage was achieved in 19 out of 25 patients (76%) with a "portal" type of dissection, while 1 out of 9 with "supra-portal" and none out of 3 with "infra-portal" type dissections were successful in this respect. Of the 19 patients who achieved significant biliary flow, 8 have lived for 2--7 yr without jaundice and 3 others are jaundice-free for shorter intervals.     

A study of patients with long-term bile flow after hepatic portoenterostomy for biliary atresia

Thirty-five cases of biliary atresia that demonstrated the creation of internal fistula between intrahepatic bile ducts at the portahepatis and intestine and showed long-term bile flow after our hepatic portoenterostomy were investigated from various aspects. These 35 cases were divided into Group A cases, in which jaundice disappeared within three months after surgery, and Group B cases, in which persistent jaundice was seen more than three months after surgery. (1) Severe liver fibrosis and degeneration of intrahepatic bile ducts were more often seen in Group B cases than in Group A cases. (2) The age at the time of surgery and the size of intrahepatic bile ducts at the portahepatis did not have much influence on the operative results in those 35 cases. (3) Measurement of the amount of bile flow and bile acid excretion obtained from Suruga II enterostomy is useful for evaluating the postoperative results of biliary atresia patients. (4) Reoperation that includes curettage and rehepatic portoenterostomy, and serious postoperative ascending cholangitis were each closely related to postoperative persistent jaundice.     

Clinical significance of reconstruction of the right hepatic artery for biliary malignancy

Background and aims The clinical significance of resectional surgery with reconstruction of the right hepatic artery for biliary malignancy remains unclear.Patients/methods Between 1990 and 2004, six patients (5%) with cholangiocarcinoma and five patients (3%) with gallbladder carcinoma with possible involvement of the right hepatic artery underwent resectional surgery with reconstruction of the right-sided hepatic artery. The surgical procedures included extended left hemihepatectomy (n=4), left trisectionectomy (n=1), central bisegmentectomy (n=1), resection of anterior segment and inferior area of segment 4 (n=2), resection of segment 5 and inferior area of segment 4 (n=1), and extrahepatic bile duct resection (n=2). Segmental resection and reconstruction of the right (n=7), anterior (n=1), or posterior (n=3) hepatic artery was performed by end-to-end anastomosis (n=5), using the right gastroepiploic artery (n=4), the gastroduodenal artery (n=1), or an autologous venous graft (n=1).Results There was no in-hospital mortality. Histopathological arterial involvement was present in seven patients, and the surgical margin was positive in five patients. The median survival was 23 months in R0 patients (n=6), while it was 13 months in R1 patients (n=5) (p=0.16).Conclusion Reconstruction of the right hepatic artery was safely performed in patients with biliary malignancy. Arterial reconstruction can be indicated when the arterial involvement is the only obstacle to obtain negative surgical margins.     

Biliary atresia with aneurysmal dilatation of hepatic artery: A rare anomaly

INTRODUCTIONThe coexistent biliary atresia with aneurysmal dilatation of hepatic artery is a rare association. To know these anomalies will avoid many per operative complications. It is also important to mention that these children require liver transplantation in the long run then these vascular anomalies become more relevant.PRESENTATION OF CASEA four month old male child presented with features of biliary atresia. On exploration a cystic expansile mass was detected beneath thread like common bile duct. Subsequent aspiration and studies proved it to be aneurysmal dilatation of hepatic artery.DISCUSSIONWith biliary atresia many vascular and cardiac malformations have been described but aneurysmal dilatation of hepatic artery is a rare association. These anomalies may have impact on aetiopathogenesis of biliary atresia and also future liver transplantation.CONCLUSIONAwareness of rare association of hepatic artery aneurysm with biliary atresia will help in understanding aetiopathogenesis of biliary atresia and planning liver transplantation in such cases.     

Connective tissue growth factor expression is increased in biliary epithelial cells in biliary atresia

Background/Purpose

Connective tissue growth factor (CTGF) has been implicated in the pathogenesis of hepatic fibrosis and is elevated in the serum of children with biliary atresia (BA). The objective of this study was to evaluate hepatic CTGF messenger RNA (mRNA) expression and its relationship to hepatic histology in children with BA.

Methods

Connective tissue growth factor mRNA expression was evaluated by in situ hybridization in 26 liver biopsies from 11 patients with BA, 11 with other diseases, and 4 autopsy controls. Serial sections were immunostained with cell-specific markers to characterize the cells expressing CTGF. Biopsies were scored for CTGF expression (0-4) and inflammation and fibrosis (1-4).

Results

High levels of CTGF expression were observed in 9 of 11 BA with localization to biliary epithelial cells and vascular endothelial cells. Connective tissue growth factor mRNA expression was correlated with fibrosis in BA and all livers. In the 11 patients with other liver diseases, 7 had CTGF expression limited to hepatic stellate cells and vascular endothelial cells. None of the 4 livers in children without liver disease had significant levels of CTGF.

Conclusions

In BA livers, novel biliary epithelia CTGF mRNA expression is high and correlates with severity of fibrosis. These data support a role for biliary epithelial cell signaling in fibrogenesis.     

Revision of porto-enterostomy in congenital biliary atresia

For the patients with insufficient bile flow following porto-enterostomy for congenital biliary atresia, removal or resection of granulation or scar tissue at the porta hepatis has been performed. Of 11 reoperations, constant bile excretion was obtained in four. Sufficient resection of scar tissue at the porta hepatis was most important for revision of porto-enterostomy. For resection of scar tissue, a special scissors devised by us was used.     

腹腔镜与开腹行肝门肠吻合术治疗小儿先天性胆道闭锁效果的对比研究

目的探讨腹腔镜下行肝门空肠吻合术治疗先天性胆道闭锁的效果。方法2003年8月~2005年9月,对26例胆道闭锁(Ⅲ型)患儿在腹腔镜下行肝门空肠吻合术,充分显露肝门,游离并于肝门纤维块处切除胆囊,提起距Tre itz韧带20 cm处空肠,经脐将空肠提出腹壁外并逐渐拉出远端40 cm范围,距Tre itz韧带20 cm横断空肠并封闭远端,将近端与远侧30~35 cm处空肠行端侧吻合,把肠管送回腹腔。将肝支空肠襻经结肠后隧道上提至肝下,肝门空肠端侧吻合。与同期同年龄段34例胆道闭锁(Ⅲ型)行开腹肝门空肠吻合术的患儿进行对照,比较两组手术时间、术中出血量、手术前后肝功能指标、平均住院天数和住院费用及术后转归情况。结果腹腔镜组手术出血量少于开腹组[(15.4±5.0)m l vs(33.8±19.4)m l,t=-4.709,P=0.000],住院费用高于开腹组[(19 153.9±619.5)元vs(15 116.7±898.4)元,t=19.607,P=0.000];两组手术时间、住院天数无显著差异。术后腹腔镜组血清总胆红素、直接胆红素、ALT和AST比开腹组下降明显。腹腔镜组术后切口疝1例;开腹组急性肝衰竭、伤口裂开各1例,伤口感染2例,两组早期并发症发生率无显著性差异(2χ=0.395,P=0.530)。术后4个月时随访,腹腔镜组13例(占50%)患儿退黄;开腹组18例(53%)患儿退黄。结论腹腔镜肝门空肠吻合术治疗小儿先天性胆道闭锁微创效果明显,方法安全、有效。     

肝移植术后肝动脉狭窄患者胆管并发症的治疗

目的 探讨原位肝移植术（OLT）后肝动脉狭窄（HAS）患者胆管并发症的发生率及肝动脉介入治疗对胆管并发症的预防和治疗作用.方法 回顾分析本中心2003年10月至2006年3月22例OLT后HAS患者胆管并发症的发生情况,并分析HAS的介入治疗对胆管并发症的预防和治疗作用.结果 22例OLT术后HAS患者,3例于术后近期行再次肝移植术,1例死于严重感染.其余18例HAS患者中,GGT和ALP升高发生率为100%,胆管狭窄（BS）的发生率为61.1%（11/18）,胆漏的发生率为5.6%（1/18）.12例患者接受肝动脉介入治疗后,6（50%）例发生BS,经胆道介入治疗后,0例治愈,6（100%）例好转;2例患者未行肝动脉介入治疗,1例发生BS,经保守治疗治愈;4例患者在出现BS后诊断HAS,均接受肝动脉和胆道介入治疗,0例治愈,3（3/4）例好转,1（1/4）例无效.结论 OLT后合并HAS患者的胆管并发症发生率较高,肝动脉介入治疗对HAS患者胆管并发症的预防和治疗作用不理想.     

Traumatic common hepatic artery injury causing isolated right hepatic ischemia due to a left accessory artery. A case report

IntroductionHepatic arterial liver flow is renowned for its redundancy. Previous studies have demonstrated that the common hepatic artery is not essential for liver survival. We present a case of a 31year-old involved in a high-speed motor vehicle accident whose liver survived thanks to the presence of an accessory hepatic artery.Presentaton of the caseWe present the case of a 31year-old male who sustained a traumatic injury of the proper hepatic artery following a motor vehicle accident. The patient suffered temporary right liver lobe ischemia due to the presence of an accessory left hepatic artery. This resulted in the selective formation of ‘biliary lakes' distinctively within the territory of the right hepatic artery supply.Simultaneously the patient developed a pseudo-aneurysm of the proper hepatic artery which required radiology intervention. At the time of pseudo-aneurysm embolisation, a rich network of arterial collaterals had formed between the accessory left hepatic and the inferior phrenic artery. On follow up the biliary lakes to the right lobe had resolved, but a small area at the periphery of the right lobe had encountered atrophy.DiscussionThis case report is an ‘in vivo’ demonstration of liver resilience to arterial flow re-distribution and demonstrates the ability of the biliary epithelium to recover from and ischemic injury.ConclusionParenchymal liver survival is mostly independent from flow within the common hepatic artery. Acute and chronic liver parenchyma changes following interruption of hepatic artery flow can still occur.     

Successful revision of portoenterostomy in an infant with biliary atresia

We present a case report of a boy with biliary atresia who, after hepatoportoenterostomy performed on day 21 of life, had immediate resolution of cholestasis and remained anicteric until 3.5 months of age. He then abruptly developed acholic stools. Nuclear medicine imaging study showed no excretion. Broad-spectrum antibiotics and corticosteroids were administered but did not lead to clinical improvement; a surgical revision of the original anastomosis was undertaken at 4 months of age. At 14 months of age, the child is anicteric and growing well. In this case, successful revision of hepatoportoenterostomy averted the need for liver transplantation.     

大鼠肝移植重建肝动脉对胆管超微结构和并发症的影响

目的 观察大鼠原位肝移植重建肝动脉对肝内胆管上皮细胞缺血再灌注损伤后超微结构及术后胆道并发症的影响.方法 228只SD大鼠分为假手术组(8只)、肝移植重建肝动脉组(55对)和未重建肝动脉组(55对).重建肝动脉组和未重建肝动脉组分别于肝脏复流后0.5、3、6、12、24、36、48 h取材,用透射电镜观察肝内胆管上皮细胞的超微结构,通过计算机图像分析系统对线粒体形态计量分析;观察术后胆道并发症.结果 两组肝内胆管上皮细胞损伤均有加重,表现为线粒体肿胀、嵴模糊或消失、微绒毛减少等超微结构改变,至24 h达高峰,以后逐渐恢复.术后两组线粒体平均面积和周径随时间的延长逐渐增大,线粒体数密度随时问延长而减少.在24 h,两组缺血再灌注损伤最显著,之后均开始缓解.在24、36、48 h,两组线粒体平均面积、平均周径比较,差异均有统计学意义(t=-3.566,-7.780,-4.730,-4.610,-2.599,-5.370,P＜0.05);在36、48 h,两组线粒体平均数密度比较,差异有统计学意义(t=-4.619,4.000,P＜0.05).重建肝动脉组的胆道并发症发生率低于未重建肝动脉组(x2=4.286,P＜0.05).结论 大鼠肝移植重建肝动脉对肝内胆管上皮细胞缺血再灌注损伤后的超微结构具有保护作用,有利于术后恢复和减少胆道并发症的发生.     

Cholangiocyte secretion of chemokines in experimental biliary atresia

Biliary atresia (BA) is a disease of the newborn that results in obstruction of the biliary tree. The cause of BA remains unknown; however, recent studies using the murine model of biliary atresia have found that rotavirus infection of the biliary epithelial cell (cholangiocyte) triggers an inflammatory response. We hypothesized that rotavirus infection of cholangiocytes results in the release of chemokines, important mediators of the host immune response.

Methods

In vivo, Balb/c pups were injected with rhesus rotavirus (RRV) or saline, and, their extrahepatic bile ducts were microdissected 2, 5, 7, and 14 days after injection. Next, an immortalized cholangiocyte cell line (mCl) was incubated with RRV or serum-free media. Qualitative and quantitative chemokine assessment was performed using enzyme-linked immunosorbent assay, polymerase chain reaction, and immunohistochemistry.

Results

In vivo, increased levels of the chemokines macrophage inflammatory protein 2, monocyte chemotactic protein 1, KC and Regulated upon Activation, Normal T Expressed and Secreted were found in RRV-infected murine bile ducts. In vitro, infected mCl cells produced increasing amounts of these same chemokines in relation to dose and time.

Conclusion

These novel results suggest that chemokine expression by RRV-infected cholangiocytes may trigger a host inflammatory process that causes bile duct obstruction. Understanding how viral infection initiates this response may shed light on the pathogenesis of biliary atresia.     

Expression of the interferon-induced Mx proteins in biliary atresia

Biliary atresia (BA) is a rare disease of the newborn for which the Kasai procedure is curative only for a few of the patients. The dilemma is that all therapeutic attempts to cure the disease are symptomatic because the etiology is still unclear. One theory suggests a progressive inflammatory process, possibly induced by a viral infection. The aim of the present study was to investigate the activity of type I interferons (IFNs) in the livers of patients with BA. Mx proteins, which mediate an early innate immune response, are a very sensitive marker for type I IFN activity (eg, to viral infection). Liver biopsies were taken during the Kasai procedure from 13 newborns with BA who were serologically negative for hepatotropic viruses. Age-matched controls originated from 7 patients with neonatal cholestasis (eg, inspissated bile syndrome), 3 aborted fetuses, and a 10-year-old child. The immunostaining procedure (alkaline phosphatase anti-alkaline phosphatase) was performed with Mx-specific monoclonal antibody. Immunostaining for Mx proteins was positive in the hepatocytes of all newborns with BA, whereas the intrahepatic bile ducts were positive in all but one. In the control group, 8 of 11 liver samples were Mx-negative. This is the first study dealing with the detection of type I IFN activity in the liver of patients with BA. This observation supports the etiologic consideration of type I IFN-mediated immune response. Although positive findings of viruses in patients with BA are still inconsistent, the present study retraces the progressive inflammatory process in BA one more step toward its beginning.     

A novel approach to the management of late-onset liver failure in biliary atresia

Purpose

Late-onset hyperbilirubinemia in patients who have undergone a successful portoenterostomy (PE) for biliary atresia (BA) is usually considered evidence of ongoing severe liver failure. The authors recently have treated 2 patients who had acute hyperbilirubinemia years and months after a successful PE and had dilated intrahepatic cysts. A combined operative and percutaneous approach reestablished drainage and a reduction in their bilirubin levels.

Methods

Data from 2 cases of BA and late-onset hyperbilirubinemia from obstruction were reviewed and analyzed.

Results

Two patients (15-year-old boy and a 2.5-year-old girl) presented with increasing serum bilirubin after a PE for BA in infancy. Both had extensive preoperative workup, which showed intrahepatic biliary dilatation in one and a large bile lake in the other. They underwent attempted percutaneous transhepatic cholangiography and stenting, followed by an intraoperative transhepatic approach in which the dilated ducts were connected to the PE. A rapid and sustained reduction in the serum bilirubin level was noted in these patients.

Conclusions

When patients with BA after a successful PE present with sudden onset of hyperbilirubinemia, imaging for biliary obstruction should be carried out. If biliary dilatation is found, then a combined radiologic and operative approach may help improve the bile flow and delay the need for liver transplant.     

A technique for hepatic artery anastomosis during orthotopic liver transplantation

Hepatic artery thrombosis remains the most common technical complication that causes graft failure following orthotopic liver transplantation. The Hepatic artery anastomosis should be performed using meticulous technique and adequate magnification. We report a very low incidence of Hepatic artery thrombosis (1.3%) utilising a modified microvascular 120° triangulating technique in 150 adult liver transplants.     

Reconstruction of the hepatic artery using the gastroduodenal artery

Although injury of the hepatic artery is not common during the performance of biliary and pancreatic resections, the hepatic artery if involved by tumor extension can be injured during its dissection.Several methods for reconstruction of the hepatic artery have been described and although each technique is applicable in a specific situation, autologous tissue conduits are preferable in contaminated operative fields. We report here another transposition technique to reconstruct the proper hepatic artery using the gastroduodenal artery, which may provide a local autologous artery for repair of hepatic artery damage during pancreaticoduodenectomy.     

Central hepatic bisectionectomy with hepatic artery and biliary tracts reconstruction for the patient with nodular type intrahepatic hilar cholangiocarcinoma: A case report

Introduction and importanceA central hepatic bisectionectomy (CHBS) for a hilar cholangiocarcinoma (CCA) is technically challenging because bilateral biliary reconstruction is required after resection. On the other hand, hepatic artery resection and reconstruction in a major liver resection are also technical procedures. In this report, we describe our radical CHBS with hepatic artery and biliary tracts reconstruction for a patient with nodular type intrahepatic hilar CCA.Case presentationA 76-year-old man was referred for further investigation of an incidental hepatic tumor. The hepatic tumor was located from medial sector to anterior sector with encasement of the anterior branch of the right hepatic artery. Based on these findings, we performed a CHBS with right hepatic artery and biliary tracts reconstruction. The histopathological findings revealed that the tumor consisted of moderately differentiated tubular adenocarcinoma with tumor necrosis without a fibrous capsule. In this area, tumors cells had invaded branches of the hepatic vein; however, there was no destructive invasion to the hepatic artery. Consequently, he was diagnosed with a nodular type intrahepatic hilar CCA with pT2aN0M0.Clinical discussionA CHBS is usually performed with the intent of anatomically preserving a patient’s liver as much as possible. Concomitant resection and reconstruction of the hilar vessels and biliary tracts with CHBS is one of the most technically challenging procedures in liver resections.ConclusionA CHBS with hepatic artery and biliary reconstruction may be a promising alternative if expert surgeons perform it on strictly selected patients.