The anatomy of the porta hepatis in patients with preduodenal portal vein,with special reference to portoenterostomy for biliary atresia

The aims of this study were to demonstrate the anatomic relationship between portal vein (PV) branches and hepatic ducts in the porta hepatis in individuals with preduodenal portal vein (PDPV) and to examine the validity of portoenterostomy (Kasai procedure) for patients with biliary atresia (BA) and PDPV. The porta hepatis of four subjects with PDPV without BA was dissected. Similar dissection supplemented by computer-aided three-dimensional reconstruction of the hilar structures was undertaken in a patient with BA and PDPV who underwent an unsuccessful Kasai procedure and died at 5 months of age. In three of the four subjects with PDPV alone, PV branches and extrahepatic bile ducts were abnormally arranged in the porta hepatis, some of the hepatic ducts entering the liver substance posterior, and occasionally lateral, to the main PV branches. The three-dimensional study showed that the original right main intrahepatic bile duct was located anterolateral to the portoenterostomy, which had been placed just anterior and to the right of the PV bifurcation. These findings strongly suggest that the PV may not be a reliable landmark for the Kasai procedure in patients with BA and PDPV.     

胆道闭锁肝内外胆系组织病理形态学分析

目的 通过胆道闭锁（BA）肝门纤维块、肝脏组织的病理及其超微结构观察，对肝门成纤维细胞分化程度进行评分，并与肝纤维化分级进行相关分析。方法 选取BA患儿作为研究对象，术中取肝门纤维块及肝脏组织标本；研究同期选取疑似BA经术中胆道造影除外BA，诊断为胆汁淤积综合征和先天性胆管扩张症患儿作为对照组，留取肝脏组织标本。在光镜和电镜下观察标本的病理改变，以及肝细胞、毛细胆管和肝门成纤维细胞的超微结构。采用SPSS 14.0软件，半定量比较BA与对照组肝脏纤维化的差异，检验肝门纤维块成纤维细胞活跃程度与肝纤维化分级的相关性。结果 2005年7月至2006年5月复旦大学附属儿科医院收治的21例BA Kasai根治术病例，手术平均年龄（66±20）d；对照组为5例胆汁淤积综合征和10例先天性胆管扩张症患儿。BA组肝组织病理改变主要是肝内门脉区胆管炎症及纤维化形成，肝纤维化程度明显高于同年龄胆汁淤积综合征和先天性胆管扩张症患儿；肝门纤维块毛细胆管增生，部分管腔闭锁、狭窄，腔内炎细胞浸润及部分淤胆，大量间质成分增生；电镜下肝门成纤维细胞活跃、肝脏毛细胆管上皮微绒毛缺失、肝细胞及肝血窦内电子致密物质增多及部分毛细胆管扩张；肝门成纤维细胞分化程度与肝组织纤维化程度相关（P=0.04）。结论 BA肝组织病理改变主要是肝内门脉区胆管炎症及严重纤维化形成；超微结构改变提示肝门部成纤维细胞活跃，其分化程度与肝纤维化程度相关。     

先天性胆管扩张症合并肝内胆管扩张及复杂胆道畸形的诊断与治疗对策

目的研究先天性胆管扩张症合并肝内胆管扩张及复杂胆道畸形的特殊检查方法及其临床意义，探讨在标准术式即囊肿切除胆道重建的基础上对肝内胆管扩张及可能存在的复杂胆道畸形处理的方法。方法对于合并肝内胆管扩张患儿，以术中胆管造影、胆道镜观察等仔细进行术中检查，了解肝总管、胆囊管、左右肝管、肝内胆管扩张及狭窄情况，以及胰胆管共同通道的病理变化，同时仔细检查有无复杂胆道畸形的存在并予以手术处理。结果全组57例，33．3％合并肝内胆管扩张，其中10例为肝内胆管囊样扩张，其汇入肝总管的开口呈瓣膜状、隔膜状或细短管状狭窄。9例为自肝门向肝内胆管起始端逐渐变细的锥形扩张，无明显狭窄。2例合并迷走胆管，3例为左右肝管分别汇入扩张的胆总管囊肿。结论对合并肝内胆管扩张者进行相应的特殊检查具有重要的临床意义，可以指导在标准根治术，即囊肿切除胆道重建的基础上对近端肝内胆管狭窄矫治、可能存在的迷走胆管、复杂胆道畸形及远端胰胆管共同通道病变的处理。     

Analysis of the pathomorphology of the intra- and extrahepatic biliary system in biliary atresia

OBJECTIVE: Aim of the study was to investigate the pathomorphological changes in the liver and triangular cord of the porta hepatis in biliary atresia and assess the relationship between the degree of differentiation of fibroblasts in the triangular cord of the porta hepatis and the liver fibrosis scores. METHODS: From September 2005 to May 2006, 21 patients with biliary atresia (66+/-20 days old) underwent a Kasai procedure. The liver biopsy and the remnant of the porta hepatis were conserved. Five cases with cholestasis syndrome and 10 cases with choledochal cyst were used as a control group. Liver biopsies were performed in the control group. The micro- and ultrastructure of the liver and the remnant of the porta hepatis were assessed in the biliary atresia and the control group. Ultrastructural features were examined by transmission electron microscopy. The semiquantitative differences in liver fibrosis grading between the biliary atresia and the control group was evaluated with a 3-grade staging system. The degree of differentiation of fibroblasts (FB) in the triangular cord of the porta hepatis was assessed as follows: 1) juvenile type: above 50 % FBs were juvenile, 2) senior type: above 50% FBs were senior, 3) median type: between the former 2 types. The differentiation scores of FB in the porta hepatis were assessed in relation to the liver fibrosis score. RESULTS: 1) The pathological changes with BA are characterized by inflammation and fibrosis in the hepatic portal area. The fibrosis scores in the biliary atresia group (I: 2 cases, II: 12 cases, III: 7 cases) were significantly higher than in the control group (I: 8 cases, II: 5 cases, III: 2 cases; p=0.01. 2) In the biliary atresia group, the extrahepatic biliary system of all cases showed a triangular cord in the porta hepatis. The triangular cord of porta hepatis was characterized by hyperplasia of canaliculi, atresia or stenosis of the bile ducts, inflammation infiltration, cholestasis, and interstitial fibrosis. 3) The ultrastructural features of BA showed active fibroblasts, a loss of microvilli, dense deposits in the hepatocytes and liver sinusoid, and dilatation of canaliculi. 4) The differentiation scores of FB in the porta hepatis were positively related to the liver fibrosis score (p=0.04). CONCLUSION: The main pathological changes of biliary atresia are inflammation and fibrosis in the hepatic portal area. The ultrastructural features of biliary atresia suggested that the differentiation scores of FB in the triangular cord of the porta hepatis were positively related to the liver fibrosis score.     

Treatment of extrahepatic bile duct atresia: results and long-term prognosis after hepatic portoenterostomy

Since 1972, 64 infants suffering from extrahepatic bile duct atresia have been examined in our Department. Between 1975 and 1984 conventional or extended hepatic-portoenterostomy was performed on 41 patients. These infants were classified into two groups on the basis of age at the time of surgery in relation to the duration of cholestasis. Of 12 patients operated upon 5 or more years ago, 8 (66%) are still alive with favorable bile flow and 4 (34%) have died from hepatic failure caused by biliary cirrhosis. Of 22 infants above 8 weeks of age who have been treated so far, 7 (31%) are alive with good bile flow and 15 (69%) have died within the past 3 years. In contrast, 12 (63%) of 19 infants operated upon before 8 weeks of age are healthy with good bile flow and only 7 (37%) have died from cirrhosis. The course of the disease was analyzed with regard to the following findings: (1) age at surgery with respect to the duration of cholestasis; (2) extent of liver fibrosis at surgery; (3) cross-section of ductular structures in the porta hepatis; (4) numerical and morphological changes in the interlobular bile ducts; (5) extent of dissection of the porta hepatis; (6) postoperative hepatoportitis. Principles determining the long-term follow-up of this dynamic obliterative process can be deduced from these investigations. Accordingly, a favorable prognosis can be expected if the following conditions are fulfilled: no cholestasis for more than 6 weeks; low-grade fibrosis at the time of surgery, with a mean diameter of the ductular structures in the porta hepatis of more than 450 m; no numerical or morphological alterations in the interlobular bile ducts; no postoperative hepatoportitis. Offprint requests to: P. Schweizer at the above address in Hannover     

胆道闭锁肝内外胆系免疫炎性反应与临床预后相关分析

目的 测定胆道闭锁(BA)肝组织和肝门纤维块中白细胞分化抗原(CD68)、单核细胞趋化蛋白-1(MCP-1)和细胞间粘附因子-1(ICAM-1)的含量,分析肝内外组织免疫炎性改变与临床预后相关性,旨在了解其在发病过程的重要性.方法 取2005年7月至2006年5月在本院行BA Kasai根治术的21例患儿的肝门纤维块及肝脏组织标本;对照组为5例胆汁淤积综合征患儿和10例胆总管囊肿患儿的肝脏组织标本.采用免疫组化染色法进行CD68、MCP-1和ICAM-1染色.选用LEICA DM研究级生物显微镜,QWIN软件环境下测量抗体阳性细胞面积百分比和平均光密度.随访19例BA患儿术后3个月直接胆红素下降比率,与肝组织CD68、MCP-1和ICAM-1表达量进行相关性分析.结果 BA组肝组织CD68、MCP-1、ICAM-1表达较对照组明显增强,但肝门纤维块几乎没有表达;CD68、MCP-1、ICAM-1阳性表达量和表达强度明显高于对照组,其中ICAM-1最为显著;除了MCP-1外,CD68、ICAM-1在肝脏表达的表达量与表达强度呈线性正相关,相关系数分别是r=0.838,P=0.0002;r=0.565,P=0.035;BA组肝组织CD68阳性表达面积百分比与术后3个月直接胆红素下降率呈负相关(r=-0.645,P=0.023).结论 CD68阳性表达是BA免疫炎性反应过程的一个重要环节,MCP-1和ICAM-1在这一环节中起了一定的作用;肝组织CD68阳性表达可能提示预后不佳.     

Immunohistochemical and molecular biological investigations regarding the pathogenesis of extrahepatic biliary atresia. (Part 1: immunohistochemical studies).

INTRODUCTION: The pathogenetic model for biliary atresia presently most favored is that EHBA is the result of a peri- or postnatal bile duct lesion. Several authors demonstrated inflammatory infiltrations in the mesenchymal areas of the liver and thus concluded an infectious genesis. An association of rota-, reo- (and CMV) virus infection with EHBA was suspected, but the presence of these viruses in EHBA could not be reproduced. In view of this controversial debate we found it to be indicated to investigate tissue blocks from the porta hepatis and liver biopsies in children with EHBA by histo- and immunohistochemistry for the quality and quantity of leukocyte infiltrations. METHODS: 31 tissue excidates of the porta hepatis were gained on the occasion of hepatoportoenterostomy, fixed in 4 % buffered formalin and embedded in paraffin. The presence of leukocyte infiltrations and their subpopulations was demonstrated by histochemical reactions and immunohistochemical staining methods using specific antibodies against surface markers. The number of leukocytes and their subpopulations was counted in three different regions of the porta hepatis, the obliterated extrahepatic bile duct, the fibrous mass of the porta hepatis and the transition zone between the fibrous mass and liver parenchyma. A statistical analysis was done. RESULTS: In EHBA, leukocyte infiltrations consist mainly of macrophages. Antigen-presenting cells and lymphatic cells play a minor role. Lymphatic cells could only be detected in 6 out of 31 tissue preparations. Antigen-presenting cells could only be detected via anti-F13a antibody which shows cross-reactivities, i.e. against macrophages and embryonal tissue. Evaluating the density of leukocyte infiltrations with regard to the different anatomical regions of the porta hepatis we could demonstrate that leukocyte infiltrations are scarce around the rudiment of the bile duct whereas the highest leukocyte density could be found in the fibrous mass of the porta hepatis and the intrahepatic fibrous septs interconnecting the fibrous mass of the porta hepatis with liver parenchyma. Liver parenchyma was mainly free of leukocyte infiltrations with the exception of neutrophilic granulocytes. Regardless of the subpopulations, leukocytes were mainly arranged around the bile ducts of the fibrotic septa. CONCLUSIONS: Most tissue preparations from children operated on during the 4th-8th week of life show only small leukocyte infiltrations and in the majority of cases no immunocompetent lymphocytes. This leads to the conclusion that a virus infection as an underlying cause for EHBA is very unlikely. Most probably, the observed leukocyte infiltrations are due to an unspecific phagocytotic activity. Comparing our results to reports from Hadchouel et al (9) and Landing et al (12) led us to believe that a pathologic immunoreaction with a possible defective antigen elimination could also be considered as a reason for EHBA.     

Endoscopic findings at porta hepatis and clinical outcome after portoenterostomy in biliary atresia

Postoperative endoscopic examination of the porta hepatis through an exteriorized enterostomy was undertaken in 19 children with biliary atresia (BA). Endoscopic findings were classified into three types: (1) ductal (D) type with distinct bile-duct orifice and good bile drainage; (2) oozing (O) type without distinct bile-duct orifice but good bile flow; and (3) covered (C) type with no bile-duct orifice and no bile discharge. The postoperative course of the D-type patients was excellent with immediate disappearance of jaundice and good liver function. In the O type, all but 1 patient became jaundice-free, but the duration of disappearance of jaundice after the operation was significantly longer than for the D type (P <0.05). All of the patients with type C died within 3 years after portoenterostomy. Histologic studies of the extrahepatic biliary remnant showed no significant difference in the area of the largest bile-duct lumen or the total area of all bile duct openings among the three types. The age at operation was youngest in type D, and there was a significant difference between types D and C (P <0.05). Endoscopic examination of the porta hepatis reaffirmed the importance of early diagnosis and early operation in the treatment of BA. Type D may have an excellent prognosis while type C may require liver transplantation. Correspondence to: S. Matsuo at his present address     

Advanced biliary atresia : Is portoenterostomy justified in all infants?

During 1989–98, of the 127 patients with biliary atresia, 23 were seen with advanced biliary atresia (ABA) presenting with (i) at more than 120 days of age (ii) established cirrhosis and (iii) features of portal hypertension. Sixteen of these underwent exploratory laparotomy, dissection at the porta hepatis and hepatico-portoenterostomy (HPE) (group I). The remaining 7 infants underwent portal dissection and sump drainage only (instead of HPE). The drainage if any was evaluated for any change in colour, volume and concentration in the post-operative period (group II). Age and presentation were the same in both the groups. In group-II, HPE was considered only if the bile flow was noted after portal dissection. Bile flow was seen in 3/16 in group I and 0/7 in group II. The fall in serum bilirubin during the first seven post operative days was noted in 2/16 in group I and 0/7 in group II. No drain output was recorded in any of the group II infants. The incidence of complications and the duration of hospital stay was significantly higher in group I patients. The sump drainage as an alternative procedure to HPE not only served the purpose of evaluating the patients with ABA for the possible bile flow in the post operative period but also avoided the need for a major operative procedure like HPE.     

Experimental hepatoportoenterostomy: duct mucosal healing with hybrid biliary-intestinal epithelium forming an autoanastomosis

Permanent bile flow following hepatoportoenterostomy for biliary atresia is presumed to follow mucosal healing of the biliary tract to the intestinal epithelium, but the morphology of the anastomosis and the histology of the neo-biliary duct is not known. Experimental portoenterostomies were constructed in five normal 10-kg mini-pigs at approximately 3 months of age to study the healing of the anastomosis. The extrahepatic bile duct was resected in continuity with an en-bloc 1.5 × 0.3-cm segment of liver at the porta hepatis and biliary drainage was achieved with a Roux-en-Y jejunal limb. The bile duct remnant at the porta hepatis measured 3 mm in diameter. HIDA scan performed at 2 months showed prompt excretion of the radioisotope and normal function of the jejunal limb. Animals were killed at 2 weeks, 1 month, and 3 months following operation and the intact hepatobiliary anastomosis examined by routine histology and histochemical staining for acid and neutral mucins. At 2 weeks the biliary epithelial ducts were hyperplastic and showed evidence of cellular regeneration and cholangitis. By 1 month the biliary and intestinal epithelia were in close approximation. At 3 months the biliary-intestinal epithelium was healed, forming a funnel-like autoanastomosis with normal proximal biliary epithelium and distal intestinal epithelium. An intervening zone of hybrid biliary-intestinal epithelium showed basal crypts with characteristics of biliary epithelium, while the superficial glandular epithelium resembled intestinal villi. Mucin histochemistry of the hybrid epithelial lining revealed predominantly neutral mucins in the basal region of the mucosa characteristic of biliary mucosa. The luminal epithelium revealed a high acid mucin content, including goblet cells. A microvillus brush border, rich in neutral mucin, was also present apically on the luminal most cells. We conclude that: (1) hepatoportoenterostomy in normal mini-pigs is a reproducible model to study healing of the anastomosis; (2) a continuous, intact hepatobiliary-intestinal mucosa is present within 3 months following portoenterostomy; and (3) the neo-epithelium has a hybrid biliary-intestinal appearance. This study may have application to understanding the cause of cholangitis following portoenterostomy for biliary atresia.     

Induction of bile ducts in embryonic liver by mesenchyme: a new perspective for the treatment of biliary atresia?

Presently only those forms of Extrahepatic Biliary Atresia (EHBA) with minimal or no intrahepatic manifestations can be treated successfully by extensive hepatoportoenterostomy. Intraoperative macro- and microscopic observations show that the typical pathogenetic manifestations in EHBA are most prominent at the porta hepatis. We therefore postulate that EHBA is the result of a defective embryonic development of the porta hepatis. In rat embryos hepatic bile duct formation is initiated at the porta hepatis and in this context mesenchyme from the periportal region seems to play a major inductive role. In order to demonstrate the role of invading periportal mesenchyme for the process of bile duct rudiment formation we established an organ culture model of the embryonic porta hepatis by recombining periportal mesenchyme with peripheral liver fragments from 15 days old rat embryos (Carnegie Stage 21). The degree of mesenchyme invasion as well as the formation of mesenchyme-surrounded liver cell clusters, rosettes or vesicles (bile duct rudiments) were assessed. Mesenchyme from the porta hepatis invaded the peripheral liver fragments and induced the formation of mesenchyme-surrounded liver cell clusters and rosettes with the beginning of lumen formation. Kidney mesenchyme recombined with liver fragments as a mesenchymal alternative showed almost the same effect, lung mesenchyme showed only a very weak inductive effect. To assess the effect of a diffusible factor versus direct cell contact, a millipore filter with and without paraffin coating was interposed between mesenchyme containing tissue and peripheral liver tissue fragments. Without direct cell contact to mesenchyme no hepatoblast cluster or rosette formation could be observed. Comparing this result to the normal development of the liver in rats our investigations suggest that the embryogenesis of the porta hepatis is probably defined by the following two developmental steps: First, differentiation of the intrahepatic bile duct system which is induced by invading mesenchyme originating from the extrahepatic periportal region and realized by epithelium mesenchyme interaction. Second, fusion of extra- and intrahepatic bile duct systems at the level of the later porta hepatis. Disturbances of this complex process can possibly lead to biliary atresia. Further investigations regarding details of the role of the mesenchyme, its inductive factors and the kidney mesenchyme's inductive potential in liver development may provide a new perspective for future treatment of biliary atresia.     

Bile-duct diameter at the porta hepatis and liver biopsies in Indian children with biliary atresia

Fibrous remnants from the porta hepatis and wedge biopsies of the liver were studied in 60 patients with biliary atresia. The patients were divided into three groups on the basis of bile duct diameter — I: no ducts; II: <50 m; III: >50 m. The liver damage was classified into three grades on the basis of ductular and parenchymal histopathologic changes and extent of inflammatory pathology. There was no correlation between severity of hepatic damage and duct diameter, although the levels of serum bilirubin and alkaline phosphatase varied in direct proportion to the degree of hepatic damage. There was an inverse relationship between age and duct diameter, but neither correlated with bile flow at surgery or in the postoperative period. The data suggest that Indian children with biliary atresia follow a different clinical course compared to those from Japan and Western countries. Correspondence to: V. Bhatnagar     

Extrahepatic biliary atresia: from diagnosis to liver transplantation

Thirty infants were diagnosed with extrahepatic biliary atresia (EBA) from July 1978 to July 1989; 28 have undergone a Kasai or Lilly-Altman modification of the Kasai portoenterostomy; 2 were excluded from surgery because they presented after 3 months of age and had advanced biliary cirrhosis. Immediate postoperative drainage (>30 ml/day was achieved in 24 patients (86%), with 14 (50%) surviving free of jaundice. The average follow-up was 2.25 years (range 3 months to 10 years); the longest survivor is 10.3 years old. The overall survival was 64%, and 5-year survival 50%. Of 24 infants operated on at <12 weeks of age, 14 are free of jaundice (58%), 4 are alive with jaundice (17%), and 6 have died (25%). No correlation existed between subsequent bile drainage and duct size at the porta hepatis, presence or absence of hepatic fibrosis, giant-cell transformation, or hepatic inflammation. Complications included cholangitis (57%), progressive hepatic failure (39%), portal hypertension (21%), gastrointestinal bleeding (21%), esophageal varices (18%), stomal hemorrhage (11%), seizures (7%), rickets (3.5%), biliary calculus in the Roux-en-Y (3.5%), and hepatic abscess (3.5%). Five infants required revision, with 1 patient undergoing six reoperations, each followed by successful re-establishment of bile drainage. One infant had a successful liver transplant, and 2 are currently candidates for transplantation. Based on this analysis, an algorithm has been formulated for the diagnostic evaluation and subsequent surgical management from initial portoenterostomy to orthotopic liver transplantation for infants diagnosed with EBA. From our review of this experience and the literature on EBA and orthotopic liver transplantation, we have concluded that portoenterostomy has a substanial chance (P <0.05) of providing bile drainage and survival is comparable to that after liver transplantation. Although ultimate failures occur at a steadily increasing rate, portoenterostomy delays transplantation until children are older and can more easily undergo transplantation with its attendant risks of immunosuppression and complications.     

Non-correctable biliary atresia with large extrahepatic cyst: a report of two cases.

Biliary atresia constitutes a serious problem in early infancy, due to the concomitant cholestasis. The conventional classification falls into 3 main types: type I, atresia of the common bile duct; type II, atresia of the hepatic duct; and type III, atresia of the porta hepatis. We report 2 unusual cases of biliary atresia type III with a cystic structure that could be mistaken for correctable biliary atresia or choledochal cyst.     

A treatment for biliary hypoplasia: tube cholecystostomy and irrigation.

PURPOSE: Biliary Hypoplasia (BH), characterised by a small ductal system and reduction in the number of interlobular bile ducts, has a bad prognosis. It has been claimed that other treatment methods apart from liver transplantation are not effective. Seven patients with BH underwent tube cholecystostomy, decompression and saline irrigation of the biliary tree via tube cholecystostomy. We present our treatment method, together with the early and late biochemical and histopathological results of these patients. PATIENTS AND METHODS: The records of seven patients with BH were reviewed retrospectively. BH was proved by operative cholangiography. Irrigation was performed intermittently with warm saline to the biliary tract via tube cholecystostomy over two to three months. SGOT, SGPT, alkaline phosphatase (AP) and bilirubin levels were evaluated preoperatively and postoperatively. Histopathological findings were also evaluated. RESULTS: Median age at operation was 46 days (range 20 - 90 days). There were six males and one female. Five patients recovered completely. There was a statistical difference between preoperative and postoperative SGOT, SGPT, AP, and bilirubin levels of patients who recovered (p < 0.05). CONCLUSION: Similar to biliary atresia, many factors such as the patient's age, postoperative bilirubin level, histopathology of liver, treatment method, and cholangitis as a complication of the surgical procedure affect the prognosis of BH. Operation before the age of 70 days, normal bilirubin levels after operation, no cholangitis attack, intracellular and intracanalicular cholestasis, and mild mixed cellular infiltration are favourable factors. We believe that decompression and irrigation of the biliary tract is an effective treatment method for suitable cases of BH.     

Anatomy of the porta hepatis as a basis for extended hepatoporto-enterostomy for extrahepatic biliary atresia--a new surgical technique.

Reports on long-term survival rates after Kasai's HPE reveal that 23% of patients are healthy ten and more years after surgery. Therefore we studied again the anatomy and histology of the porta hepatis and the portal tracts in order to improve this surgical procedure. METHODS: Autopsied livers of 6 children with and 6 without EHBA were investigated with regard to the topographical and histological anatomy of the major intra- and extrahepatic bile ducts. RESULTS: On the basis of anatomical findings we have modified the conventional Kasai's HPE and developed an extended exploration of the porta hepatis. The procedure and surgical technique is described in this paper.     

Surgical modifications,additions, and alternatives to Kasai hepato-portoenterostomy to improve the outcome in biliary atresia

Kasai hepato-portoenterostomy (HPE) is the most widely used surgical technique to restore bile flow in biliary atresia (BA). We aimed to review literature on HPE substitutes and additions to Kasai especially in advanced BA (ABA). A PubMed search was done for surgical procedures apart from or along with Kasai HPE for BA. Additional procedures to prevent cholangitis were also reviewed. Procedures and outcome were analysed. Alternative procedures done by the authors have also been described briefly. Results have been compiled in this review article. In ABA, with portal hypertension and liver cirrhosis, Kasai HPE is associated with poor outcome, increased morbidity, and even mortality. Most require liver transplant (LT). Some alternatives to HPE include exploration at porta hepatis to assess the bile flow yet avoid the major surgery (HPE) as a bridge to LT. Conduit diversion may help to combat cholangitis resistant to steroid therapy. Stoma formation is not preferred in cases listed for LT due to high risk of bleeding. Hepatocyte infusion, stem cell therapy, and synthetic liver are the future options to meet the challenges in BA. Various alternative procedures may become handy in the future especially in ABA.     

先天性胆道闭锁与巨细胞病毒感染的临床研究

为了探讨先天性胆道闭锁（ＣＢＡ）的原因，分析肝脏病理变化、临床预后与巨细胞病毒（ＣＭＶ）感染的关系。取１４例ＣＢＡ患儿术中肝脏及肝门淋巴结标本，一份进行多聚酶链反应巨细胞病毒基因（ＰＣＲ－ＣＭＶ－ＤＮＡ）检测，另一份肝脏标本做ＨＥ染色光镜检查。结果：１４例中ＣＭＶ阳性占８例。肝脏病理检查表明随手术时日龄增加，胆管增生明显。比较ＣＭＶ阳性组与ＣＭＶ阴性组肝细胞病变、淤胆无差异，肝纤维化ＣＭＶ阴性组重于阳性组；肝细胞坏死ＣＭＶ阳性组重于阴性组。随访８～２０个月，生存６例，其中ＣＭＶ阳性５例，死亡８例。ＣＭＶ感染可能是ＣＢＡ的病因之一，肝纤维化程度、术后胆管炎发生的频率和程度与预后关系密切。从临床归转看ＣＭＶ感染阳性组预后较好。     

小儿胆道急症腹腔镜下经皮胆囊造瘘13例临床分析

目的探讨小儿胆道急症的临床特点,总结腹腔镜下经皮胆囊造瘘在小儿胆道急症中的应用价值。方法回顾性分析2006年6月至2011年6月作者收治的13例胆道急症患儿临床资料,其中自发性胆道穿孔5例,胆总管囊肿并发急性胆管炎5例,结石或蛔虫梗阻并发急性胆管炎3例。患儿均在腹腔镜下行经皮胆囊造瘘。结果手术均成功实施,术后临床症状缓解,无胆道出血和胆瘘发生。自发性胆道穿孔5例,随访3个月至1年,胆道无异常发现,1例有胆总管扩张改变,目前仍在随访中,均未行二期手术。胆总管囊肿并发急性胆囊炎5例,4例在胆囊造瘘术1～3个月后行胆总管囊肿根治术,1例胆总管扩张回缩明显,目前仍在随访中。结石或蛔虫致胆道梗阻3例,术后13超复查,2例未见明显结石或蛔虫影像,胆道未见明显扩张。1例胆囊内仍有较大结石存在,于胆囊造瘘术后3个月行胆囊切除术。结论小儿胆道急症多在先天性胆道疾病的基础上并发。腹腔镜下经皮胆囊造瘘术治疗小儿胆道急症,可有效缓解胆道梗阻,术后根据情况行二期胆道处理,部分患儿甚至可以免除二期手术,是治疗小儿胆道急症的有效方法。     

Congenital solitary liver cysts.

AIM: The aim of the study was to describe clinical and pathological findings in children with rare congenital solitary liver cyst. MATERIAL AND METHODS: Six children with congenital liver cyst were treated at the authors' institution from 1995 to 2002. Clinical records and operative findings of these patients were reviewed retrospectively. RESULTS: Age at presentation ranged from neonatal to 14 years, and there were four girls and two boys. Hepatomegaly was the most common symptom in neonates while the older children presented with abdominal pain. Ultrasound was conclusive for diagnosis in all children, prenatal ultrasound in three. Intrahepatic cyst was found in five children. Partially extrahepatic cyst in the porta hepatis was found in one. The cyst diameters varied from 8 to 10 cm. Cysts were excised via an open abdominal approach in all patients. Histological findings confirmed the diagnosis of congenital liver cyst. Columnar epithelium was found in newborns while atrophic changes of epithelial lining were seen in older children. In a case of a 13-year-old girl the cyst was lined by metaplastic squamous epithelium with foci of slight dysplasia. Immunohistochemical studies were available in three recent children with positive findings in all of them. The postoperative course was uneventful in all patients. The follow-up period ranged from 36-120 months and no recurrence was observed. CONCLUSION: With appropriate selection of symptomatic patients, total excision of a congenital liver cyst is a safe procedure with excellent long-term results. Given the possibility of malignant degeneration of a congenital liver cyst we recommend its surgical excision in most cases.