Biliary atresia: Outcome and management

Untreated, biliary atresia remains a fatal condition of the newborn. Most present within four to six weeks of conjugated jaundice and acholic stools, and, although still a challenging diagnosis to make, therein lies the opportunity of changing the course of this otherwise inexorable disease. The aim of surgery is to restore bile flow, alleviate jaundice and abbreviate the cholangiodestructive process within the liver. The Kasai portoenterostomy, introduced almost 50 years ago in Japan, aims to expose microscopic biliary ductules within the fibroinflammatory mass at the porta hepatis, and restore bile drainage into a mobilised Roux loop. About 50% of infants with BA will be able to clear their jaundice following Kasai alone, given appropriately experienced surgeons and if performed prior to the onset of overt cirrhosis. They have a reasonable expectation of long-term survival to adulthood with a good quality-of-life. The remainder may be candidates for liver transplantation (where available) although donor organ shortage and immunosuppresion-related complications remain significant problems.     

Long-term survival after Kasai's operation for biliary atresia

Long-term survival of biliary atresia patients after Kasai hepaticoportoenterostomy is being increasingly reported. Prognostic factors indicative of a favorable long-term outcome consist of: (1) early age at operation; (2) bilirubin excretion of at least 6 mg daily 1 month after operation; (3) favorable hepatic histology at the time of operation; and (4) low incidence of postoperative cholangitis. Jaundice-free long-term survival is now attained in from one-fourth to one-third of patients undergoing Kasai hepaticoportoenterostomy by experienced surgeons in the Western hemisphere. In many long-term survivors there is normalization of liver function, improvement in hepatic histology, and resolution of the complications of portal hypertension. Most patients in this category have made a normal adjustment to their disease and lead active adolescent and adult lives. Offprint requests to: B. A. Stewart     

Improved weight gain following closure of exteriorized conduit for biliary atresia

Accelerated weight-gain velocity was observed in five infants with extraheptic biliary atresia following closure of the exteriorized conduit. Despite excellent bile drainage (daily volumes 150–900 ml) and refeeding of this fluid to the efferent limb, steatorrhea occurred in three patients and was suspected in the other two. Total bile-acid concentrations in the bile collected prior to refeeding into the jejunum were below the critical micellar concentration in most of the specimens tesred. The nutritional consequences of bile diversion need to be balanced against the beneficial effects in diminishing the incidence of postoperative cholangitis. We postulate that fat malabsorption occured in these children because of inadequate bile-acid concentrations and inefficient fat emulsification. Bile acids were normally conjugated with glycine and taurine. Offprint requests to: J. Levy     

Long-term follow-up of patients with biliary atresia successfully treated with hepatic portoenterostomy. The importance of sequential treatment

The outcome of 18 biliary atresia (BA) patients (5 male, 13 female; age range 10.7–22.5 years; mean 15.4±0.7 years) treated with hepatic portoenterostomy (HPE) and jaundice-free for more than 10 years without liver transplantation (LT) is analyzed retrospectively. Eight of these patients subsequently required LT (age at LT 12.8±0.5 years, range 10.5–15.2 years); 3 children (aged 11.6, 13.2 and 14.1 years, respectively) had episodes of gastrointestinal variceal bleeding associated with other signs of severe disease and are now candidates for LT; and among the 7 asymptomatic patients (age range 11.2–22.5 years; mean 15.9±2.1 years), 5 had sonographic and biochemical signs of moderate portal hypertension (PH). In order to analyze whether the age at transplantation influences the survival of children transplanted for BA, we also reviewed the outcome of 71 BA patients transplanted at our hospital between 1986 and 1996. All the children older than 10 years at the time of LT were alive; only patients younger than 10 years died following LT (n= 15). We conclude that the natural outcome of extrahepatic BA is toward PH, fibrosis, and cirrhosis, even in those cases successfully treated with HPE. In our experience, the results of sequential treatment with HPE and LT were excellent.     

A scoring system to predict the need for liver transplantation for biliary atresia after Kasai portoenterostomy

A retrospective analysis was performed of the records of 133 patients with extrahepatic biliary atresia (EHBA) who had undergone a Kasai portoenterostomy. The patients were divided into a non-transplantation group who survived but did not receive liver transplantation after the procedure and a failure group of those who died or received liver transplantation. A score was calculated that assessed nine factors, including laboratory values and complications. The data were assessed at the time complications occurred. The scores were analysed by a trend analysis to see if serial scores predicted the evolution of liver disease. A receiver operating characteristic (ROC) curve was plotted to assess the optimal cut-point for the scoring system. There were 98 patients in the non-transplantation group and 35 in the failure group. The latter group had significantly higher post-operative bilirubin (9.3±7.2 mg/dl versus 3.5±3.1 mg/dl), ALT (136±89 U/l versus 92±88 U/l), prothrombin time, and incidence of cirrhosis, ascites, oesophageal varices, portal hypertension, cholangitis and sepsis than the non-transplantation group ( P <0.05). A score of 8 had a high sensitivity (96.9%) and specificity (89.5%) for predicting the need for liver transplant. Conclusion:based on easily available clinical information, our scoring system can predict which patients with biliary atresia who have already undergone a Kasai procedure should be considered for liver transplantation.Abbreviations EHBA extrahepatic biliary atresia - ROC receiver operating characteristic     

Answered and unanswered controversies in the surgical management of extra hepatic biliary atresia

Almost a half-century since Kasai described the portoentersotomy for extrahepatic biliary atresia (EHBA), some questions about the management of this condition have been resolved and many are unanswered. The most useful diagnostic steps to aid in the diagnosis are debated. Sonography can be helpful but its sensitivity and selectivity are arguable with strong advocates for its effectiveness. Likewise, the magnetic resonance imaging has forceful advocates but also has not been universalized. The liver biopsy, done commonly before an operation for cholestasis, is often not discriminating. The radionuclide scan hepatobiliary iminodiacetic acid (HIDA) scan after phenobarbital stimulation is helpful if negative, but false positive results are common. It is agreed to proceed expeditiously to the operation in the cholestatic infant after a prompt investigation. The proposal to avoid this step and provide liver transplantation as initial management for EHBA has been suppressed by several clinical findings. The Kasai procedure has not worsened the outcome of eventual liver transplantation. The Kasai, even if it eventually fails, will often buy time and allow the child to grow before transplantation is needed. Multiple reoperations prior to the transplant are discouraged. Revisions to improve bile flow have not gained wide popularity. Use of a stoma to divert the bile has been largely abandoned. The need for frozen section examination of the liver at the site chosen for the portoenterostomy is no longer demanded. The preferred type of intestinal conduit is argued. Unanswered questions about the post-operative management include the role of steroids and of prophylactic antibiotics. The Biliary Atresia Research Consortium, a multi-institutional National Institutes of Health (NIH)-supported project, will address many of the unanswered issues.     

胆道闭锁Kasai手术效果影响因素的研究进展

胆道闭锁(biliary atresia,BA)是肝内外胆管进行性纤维化引起肝内外胆管硬化和闭锁的一种常见疾病,Kasai手术(肝门空肠吻合术)改善了患儿预后.手术时肝脏的病理状态,包括肝脏纤维化程度、胆管板畸形、肝门部纤维斑块、胆道闭锁类型都对Kasai术后的效果有影响.单纯用年龄评价手术预后有欠妥当.建立胆道闭锁治疗中心以提高外科医师实施Kasai手术的经验有助于改善手术预后,而胆道闭锁脾脏畸形综合征、早产儿、CMV感染对预后有不利影响.激素、熊去氧胆酸及抗生素的预防性使用其有效性目前尚有争议.     

Clinical significance of plasma endothelin levels in patients with biliary atresia

Biliary atresia (BA) is the end-result of a destructive inflammatory process that affects intra- and extrahepatic bile ducts, leading to fibrosis and obliteration of the biliary tracts with the development of biliary cirrhosis and portal hypertension (PH). Endothelins (ET) are 21-amino-acid peptides of endothelial origin with potent vasoconstrictor activity that bind to various cells of the liver. Nothing is presently known about plasma ET levels in BA. The aim of this study was to determine the clinical significance of plasma ET levels in patients with BA after hepatic portoenterostomy (Kasai's procedure) and to correlate these with liver function tests (LFT) and PH. We measured plasma concentrations of ET in 19 patients with BA (5 boys and 14 girls; mean age 11.6 ± 5.5 years) after portoenterostomy and 10 age-matched controls. Patients were grouped according to outcome based on LFT: group A consisted of 9 patients with an ‘‘unfavorable outcome” and Group B 10 patients with a “favorable outcome”. The plasma ET levels were measured using a highly sensitive and specific enzyme immunometeric assay (EIA). No patient had ascites or hepatorenal syndrome. Plasma ET levels were significantly higher in patients with BA than in controls (3.42 ± 0.42 vs 1.75 ± 0.39 pg/ml, respectively; P < 0.01) and in patients in group A than in group B. (3.75 ± 0.25 vs 3.06 ± 0.23 pg/ml, respectively; P < 0.01). In group A, plasma ET levels were higher in patients with PH (n = 4) than in those without PH (n = 5) (3.99 ± 0.06 vs 3.64 ± 0.22 pg/ml, respectively; P < 0.05). We conclude that plasma ET levels are high in patients with BA, especially those with severe biliary cirrhosis, and that ET may partially contribute to development of PH in BA. The results of the present study also suggest that plasma ET concentrations may be a useful marker in the follow-up of patients with BA. Accepted: 12 September 1997     

Portal hypertensive enteropathy in biliary atresia

This paper describes a rare case of biliary atresia (BA) in which massive postoperative bleeding developed due to portal hypertensive enteropathy. A 15-year-old boy had been doing well after Kasai's operation for BA. At around the age of 6 years he developed recurrent episodes of esophageal variceal bleeding and underwent esophageal transection, splenectomy, sclerotherapy, and embolization of the left gastric vein. At the age of 15 years he suddenly developed abdominal pain, hematemesis, and massive tarry stools. His hemoglobin level was 6.3 g/dl. Endoscopy showed several small, polypoid lesions in the jejunum beyond Treitz' ligament. The source of the bleeding was one of these lesions located in the proximal jejunojejunostomy of the Roux-en-Y loop. Because he had repeated episodes of melena, he underwent partial resection of the jejunum under endoscopic guidance. He has since been free of gastrointestinal (GI) bleeding. From this experience, we conclude that the polypoid lesions of an enteropathy may be a cause of massive GI bleeding in the postoperative portal hypertension of BA. Accepted: 17 April 1997     

Is age really critical when performing portoenterostomy for biliary atresia?

We report on two infants with biliary atresia who underwent a successful Kasai operation at the age of 125 and 109 days, respectively. These cases support the hypothesis that in some cases the biliary tract may be normal or near-normal at birth, but secondary changes may later lead to an abnormality. Under such circumstances the prognosis is influenced by the length of time since the onset of biliary tract obstruction, and not by the absolute age of the patient. This implies that the Kasai operation should still be considered after the age of 100 days of life.     

Adjuvant steroid treatment following Kasai portoenterostomy and clinical outcomes of biliary atresia patients: an updated meta-analysis

Background

It is controversial whether adjuvant steroid treatment should be given to biliary atresia (BA) patients following a Kasai portoenterostomy (KPE). This study aimed to quantitatively and systematically evaluate the effect of adjuvant steroid therapy post-KPE in relation to major clinical outcomes of BA patients.

Methods

We systematically reviewed the literature in PubMed, Embase, the Cochrane Library, China Knowledge Resource Integrated Database, Wanfang Database, Scholarly and Academic Information Navigator and manually searched for relevant papers published before August, 2015. We extracted data on the effects of steroid treatment following KPE on clinical outcome, including jaundice free rate and native liver survival rate at 6 months, 1 or 2 years after KPE. The weighted overall relative risk (RR) and 95% confidence intervals (CIs) were calculated by using a random-effects model.

Results

Eight cohort studies and two randomized controlled trials (RCTs) were identified (n=998). Of them, 6 cohort studies and 2 trials investigated the effect of steroid treatment as compared to non-users or placebo (n=566), and 2 cohort studies compared the effects of high-dose to low-dose steroid treatment (n=432). Steroid usage increased the clearance rates of jaundice at 6 months (pooled RR: 1.32; 95% CI: 0.995-1.76; I²=72.6%) and 1 year (pooled RR: 1.35; 95% CI: 1.12-1.61; I²=0.0%), but not 2 years (pooled RR: 0.82; 95% CI: 0.55-1.22; I²=0.0%) after KPE. There was no solid evidence supporting that steroid treatment would improve native liver survival rate at 6 months (pooled RR: 1.02; 95% CI: 0.90-1.15; I²=0.0%), 1 year (pooled RR: 1.10; 95% CI: 0.91-1.34; I²=35.2%) or 2 years (pooled RR: 1.00; 95% CI: 0.73-1.35; I²=57.4%) after KPE.

Conclusions

Adjuvant steroid treatment following KPE may improve short-term (≤1 year) clearance rate of jaundice, but no significant effects on long-term (≥2 years) clearance rate of jaundice and native liver survival rate. Studies on doses and duration of steroids, and long-term follow-up studies are warranted.

     

胆道闭锁Kasai术后早期并发症的分析

目的 分析胆道闭锁Kasai术后早期并发症与激素抗生素治疗方案、生存预后的关系.方法 回顾性分析1994年8月至2008年8月Ⅲ型胆道闭锁Kasai术后281例临床资料.根据治疗方案分为一般治疗A组和术后大剂量激素和抗生素治疗B组.随访术后2年生存,分析早期并发症与手术日龄、术前胆红素、术后激素抗生素治疗方案、生存预后之间的关系.结果 术后共93例(33.1%)发生早期并发症,急性胆管炎最常见.无并发症与并发症相比,术前总胆红素及手术日龄无差异;危险因素分析,急性胆管炎、上消化道出血分别与术前胆红素及手术日龄无明显相关.B组并发症较A组明显降低(25.7%比63.6%,P＜0.01),其中反流性胆管炎较A组明显降低(23.9%比56.4%,P＜0.01),但术后早期消化道出血无统计学差异.2年随访率92.5%,术后2年以上存活率51.2%,早期并发症2年生存率较无早期并发症明显降低(34.4%比59.6%,P＜0.01).急性胆管炎2年生存率亦明显降低(32.9%比59.2%,P＜0.01).B组2年生存率高于A组(54.0%比40.0%,P=0.043＜0.05).结论 胆道闭锁Kasai术后早期急性胆管炎是影响术后生存的危险因素;术后大剂量应用激素和抗生素疗法明显降低早期急性胆管炎发生率,提高术后2年生存率.

Abstract：

Objective The purpose of this study was to evaluate the relation between early complications of Kasai operation and high-dose steroids and antibiotics and to demonstrate the prognosis of biliary atresia(BA). Methods 281 patients diagnosed with biliary atresia(BA) type Ⅲ,admitted from Aug. 1994 to Aug 2008 were retrospectively reviewed and followed up for two years. The patients were divided into two groups. Patients in Group A were controls while patients in Group B were treated with high-dose steroids and antibiotics. We evaluated the relation between early complications and age at operation (days), baseline bilirubin, different treatments and 2 year survival. Results 93 patients had early complications,and cholangitis is the most common, There is no statistics difference of complications in age at operation and baseline bilirubin. The complication rate was 63. 6% in group A, and 25. 7% in group B(P＜0. 01). The cholangitis rate was 56. 4% in group A and 23. 9% in group B (P＜0. 01). Two groups showed no significant difference in digestive tract bleeding. The visit frequency rate was 92. 5%, while 2 year survival rate was 51. 2%. Patients with early complications or cholangitis have a 34. 4% or 32. 9% 2 year survival rate, while those without have a 59. 6% or 59. 2%(P＜ 0. 01). Patients with high-dose treatment in group B have a better 2 year survival rate (54. 0% vs 40. 0% ,P＜0. 05). Conclusions Early cholangitis was the risk factor of outcome after Kasai operation. These data implied that the use of high-dose steroids and antibiotics can lower the occurrence of cholangitis and elevate 2 year survival rate.     

术前营养状态对胆道闭锁术后胆管炎的影响研究

目的 探讨胆道闭锁患儿术前营养状态对术后胆管炎发生的影响.方法 对2014年1月至2014年12月底收入我院的66例经手术探查确诊为Ⅲ型胆道闭锁的患儿采用STRONGKid方法进行营养不良风险评估并分组:低度营养不良风险组,中度营养不良风险组及重度营养不良风险组.通过门诊及电话进行随访,随访内容为首次发生胆管炎的时间,早期胆管炎发生情况及术后3个月内发生胆管炎的次数,术后6个月内黄疸消退情况.结果 低度营养不良风险患儿0例,中度营养不良风险患儿30例(45.5％),重度营养不良患儿36例(54.5％).中度营养不良组首次发生胆管炎的时间(44.31±18.75)d明显晚于重度营养不良组(26.75±7.96)d,P=0.002;中度营养不良组早期胆管炎的发生率13.3％明显低于重度营养不良组66.7％,P=0.04;重度营养不良组患儿术后3个月内胆管炎的平均发生次数(2.11次/人)明显高于中度营养不良组(1次/人),中度营养不良组术后6个月黄疸清除率60.0％高于重度营养不良组16.7％,P=0.014.结论 胆道闭锁患儿术前合并重度营养不良易于发生早期胆管炎且反复发生,中度营养不良组的患儿首次发生胆管炎的时间明显晚于重度营养不良组,重度营养不良组术后黄疸清除率明显低于中度营养不良组.     

早期胆管炎对Kasai's术远期疗效的影响

目的 探讨Kasai's术后胆管炎对胆道闭锁(BA)长期牛存的影响.方法 通过对92例BA行Kasai's术时的手术年龄、术式和术后胆管炎发生等临床资料进行收集和分析,特别是重点分析早期胆管炎对患儿的影响.结果 对1989年3月至1997年3月共92例BA行Kasai's术,手术年龄30～137 d.其中男55例,女37例.失访3例,死亡66例,存活23例巾男14例,女9例,存活患儿年龄最大者现已满19岁.92例中,在出生后60 d内手术25例,现存活5例;61～90 d手术53例.现存活15例.91 d以上手术14例,现存活3例.本组发生早期胆管炎58例,现仍存活7例.19例因肝门部胆管梗阻再次手术,现存活4例.结论 ①胆管炎的处理是BA治疗过程中最困难的一环.胆管炎是BA术后最常见又是最难处理的并发症;②防止早期胆管炎的发生是手术成功的关键,早期胆管炎的危害大.在适宜的年龄进行Kasai's术时,有无胆管炎发生与生存率关系更密切;③对因胆管炎引起的肝门部胆管梗阻应积极治疗;④早期胆管炎不但影响手术后的牛存率,还对远期生活质量有影响.     

Biliary atresia in Turkish children

AIM: To evaluate surgical success, survival rate and relationship of outcome parameters with time at diagnosis and operation of extrahepatic biliary atresia (EHBA) patients in Izmir, Turkey. METHODS: Clinical and laboratory data were reviewed from case reports of 27 EHBA patients. Twenty-five patients were operated on using Kasai procedure and two cases received liver transplants without portoenterostomy due to decompansated liver cirrhosis on diagnosis. Post operational success was defined as clearance of jaundice (bilirubine level <2 mg/dL). Patients were studied in two groups: I (jaundice free and/or compensated liver disease with liver transplant if needed after 3 years of age) and II (progressive liver disease with death or liver transplant if needed before 3 years of age). Kasai success, age at diagnosis and operation, survival and correlation of outcome with age, preoperative liver functions were evaluated. Eight patients received liver transplants. RESULTS: Median age at diagnosis was 63.5 days (21-212) and portoenterostomy was performed at median age of 67.5 days (25-220). Kasai operation was successful in two cases (8%) and the operation was performed at 35 and 42 days. Age at diagnosis (P = 0.13) and operation (P = 0.2) was not different between the two groups. Group I and II consisted of seven (28%) and 18 (72%) infants. Pre-existing ascites, serum alanine aminotransferase (ALT) and globulin levels were significantly higher in group II patients (P = 0.008, P = 0.04, P = 0.017, respectively). CONCLUSIONS: The results of the present study indicate that general practitioners should pay close attention to the evaluation of infants with prolonged jaundice in Turkey. Because of frequent late diagnosis presenting with cirrhosis at admission and also because of the low organ donation rates in Turkey, living related liver transplantation is an option and is currently undergoing detailed ethical consideration.     

Early cytomegalovirus infection and the long-term outcome of biliary atresia

Aim:  To study the impact of ongoing cytomegalovirus (CMV) infection at presentation of biliary atresia (BA) on the long-term outcome after Kasai procedure.
Methods:  Twenty-eight patients with BA born 1988–1997 were included and followed-up until 2007. Eleven patients (group A) had ongoing CMV infection at presentation and were compared to the remaining 17 patients (group B). Median age at Kasai procedure was 75 days in group A and 70 days in group B (p = 0.12).
Results:  Including all patients, survival with native liver was 50% and 36% at 4 and 10 years of follow-up respectively. At the end of follow-up, it was 25% and overall survival was 68%. When comparing groups A and B, neither difference in survival with native liver (p = 0.67, log-rank test) nor in survival after liver transplantation was detected.
Conclusion:  Survival with native liver after Kasai procedure is comparable to that of other centres. CMV positive patients may present with a later onset, alternatively the detection of CMV infection could delay the referral of BA patients. No significant differences in long-term outcome were detected with regard to early CMV infection.     

Long-term outcome of hepatic portoduodenostomy with interposition of the ileocecoappendix for biliary atresia

Hepatic portoduodenostomy with interposition of the ileocecoappendix (HPI) can be used for the surgical treatment of biliary atresia (BA). The purpose of this study was to evaluate the long-term outcome of patients with HPI. The records of nine patients who had HPI performed for BA were reviewed. Mean age at portoenterostomy was 68.1 days (range 26–113 days). At the end of 2006, seven of the nine subjects were alive, although two required liver transplantation (LT). The two deaths occurred 94 days and 2 years after HPI due to varicella infection and variceal bleeding, respectively. Length of follow-up for the seven survivors ranged from 17 to 19 years (mean 18 years). Three subjects achieved normal liver function after HPI and have remained jaundice-free to date. Another three who were initially jaundice-free required Roux-en-Y jejunostomy (RYJ) to the ileocecum for severe obstructive cholestasis 6 months, 3 years, and 19 years after HPI secondary to stones in the cecum. RYJ was successful in these three cases, and liver function returned to normal within a few months. Two of these three have continued to have almost normal liver function, but one required LT 5 years after RYJ. The remaining case had LT because of liver dysfunction 14 months after HPI. There is a high risk for stone formation and obstruction with the HPI procedure because bile can stagnate in the ileocecum.     

Serum hyaluronan as a marker reflecting the severity of cirrhosis and portal hypertension in postoperative biliary atresia

The majority of patients with biliary atresia (BA) develop progressive liver disease and serious complications of portal hypertension, including esophageal varices and ascites, despite successful Kasai operation. The aim of this study was to evaluate whether serum hyaluronan (HA) reflects the severity of cirrhosis and its complications in patients with postoperative BA. Fifty-four postoperative BA patients aged 1 to 19 years were recruited into this study. Serum HA was determined by ELISA-based assay, and serum HA levels were compared among groups of patients according to Childs classification as well as the presence of esophageal varices and ascites. Mean serum HA concentration was significantly increased in patients with Child C (534.35±573.02 ng/ml) in comparison to patients with Child B (97.67±104.22 ng/ml, p=0.002) and Child A (36.27±71.41 ng/ml, p<0.001). Mean serum HA concentration was also significantly higher in patients who had ascites or esophageal varices than in those who did not suffer from these complications. Furthermore, patients who had variceal hemorrhage had significantly higher mean serum HA levels than those without bleeding. Thus, serum HA levels may reflect the severity of cirrhosis and its complications, and may be useful in monitoring the clinical outcomes of patients with postoperative BA.     

胆道闭锁患儿Kasai术后早期胆管炎相关危险因素分析

目的 探讨影响胆道闭锁患儿(biliary atresia,BA)经典Kasai术后早期胆管炎发生的相关因素.方法 对本组中35例BA患儿的临床资料应用二项分类logistic同归分析方法作回顾性分析.结果 胆道闭锁患儿术后近期胆管炎发生与术后胆汁引流效果、术后辅以激素治疗及术中预留胆支长度有显著相关性,而与患儿性别、手术日龄、术前总胆红素及肝功能、手术前后辅以熊去氧胆酸和苯巴比妥利胆退黄治疗、术中设置防反流瓣及术后抗感染力度等因素无关.结论 胆道闭锁患儿Kasai术后胆汁引流效果好,辅以激素治疗,同时术中预留胆支长度充分的BA患儿,其术后不易发生早期胆管炎;反之,患儿发生早期胆管炎风险增加.

Abstract：

Objective To evaluate the factors affecting the early post-operative cholangeitis after classical Kasai operation for biliary atresia (BA) in children. Methods The clinical data of thirty-five BA children from pediatric surgery department of Nanjing Childrens Hospital was retrospectively analyzed using binary logistic regression analysis. Results The occurrence of early cholangitis after classical Kasai operation was found to correlate with post-op biliary drainage effect, hormone therapy and the length of Roux-loop in operation. No correlation could be established between early cholangeitis and sex,age, total bilirubin and pre-op liver function, ursodeoxycholic and luminal therapy, the installation of anti-refluxing valve in operation and the dosage of antibiotics. Conclusions Good biliary drainage,hormone therapy and sufficient length of Roux-loop were associated with decreased risk of early cholangitis.