Prognosis of extrahepatic biliary atresia.

We carried out a retrospective investigation of the 89 patients with extrahepatic biliary atresia born in The Netherlands during a 10 year period. Of these 89 patients 10 had a diagnostic laparotomy only. Eight patients had an anastomosis between the proximal bile duct and the intestine, and the remaining 71 had hepatic portoenterostomies. Bile drainage was re-established in 46 (65%). After successful hepatic portoenterostomy the development of cholangitis was the most important determinant of long term survival; five year survival was 54% in the 19 patients who had cholangitis and 91% in the 27 who did not. In the whole group of 71 patients the five year survival was 47%. Seventeen patients were at least 5 years of age at the time of writing, three of whom had had liver transplantation. Three patients have cirrhosis and hyperbilirubinaemia, and the other 11 have normal bilirubin concentrations and normal or slightly raised transaminase activities. To improve these results early surgical intervention in all children with extrahepatic biliary atresia is necessary, as are better methods of prophylaxis and treatment of cholangitis.     

Short term histological liver changes in extrahepatic biliary atresia with good postoperative bile drainage.

Short term histological liver changes were studied in 13 patients with congenital biliary atresia, who showed good bile drainage after radical operation. Biopsies obtained at the time of the corrective surgery and at the second operation to convert from external total to partial bile drainage (undertaken once the bilirubin concentration was less than 30.8 mumol/l) were compared. A significant correlation was found between histological findings and the clinical course. In particular, at the second biopsy an increase in hepatic fibrosis and cell infiltration was observed mainly in patients whose bilirubin concentration decreased slowly or who had cholangitis, or both. Hepatic fibrosis and cell infiltration had decreased, however, in an appreciable number of patients whose postoperative course had been characterised by a rapid reduction in the bilirubin concentration and no cholangitis.     

Congenital dilatation of extrahepatic bile ducts in children. Experience in the central hospital of Hue, Vietnam.

BACKGROUND/PURPOSE: The authors present a study of a series of cases in children with congenital dilatation of the extrahepatic bile ducts (CDEBD). METHODS: Between November 1998 and October 2002, 38 children aged between 50 days and 15 years suffering from CDEBD, admitted to the Central Hospital in Hue, Vietnam, were treated surgically with a minimum follow-up of one year. RESULTS: Diagnosis was based only on ultrasonography which was 100% accurate. According to Miyano's classification, 26 cases presented as cystic dilatation of the main bile duct (MBD) associated with dilatation of the intrahepatic bile ducts, while in the other 12 cases the dilatation of the MBD was of the fusiform type. The surgical treatment of choice was extensive excision of the dilatated extrahepatic bile ducts and biliary drainage according to the Roux-en-Y method in 36/38 patients. The other two patients underwent surgery for internal biliary drainage without removing the cysts. During follow-up we observed one case of pancreatitis out of the 36 patients who underwent excision of the dilatated extrahepatic bile ducts, while the two patients who had internal biliary drainage without removal of the cysts suffered from numerous attacks of cholangitis. CONCLUSIONS: The reflux of bile in the dilated biliary tree plays an important role in the etiopathogenesis of CDEBD. Excision of the extrahepatic bile ducts and internal biliary drainage by Roux-en-Y has proved a satisfactory surgical method.     

Percutaneous management of tumoral biliary obstruction in children

Background There is limited experience of percutaneous biliary interventions in children although they are safe and effective procedures. Objective To evaluate the efficacy and safety of percutaneous management of tumoral biliary obstruction in children. Materials and methods Percutaneous biliary interventions were performed in eight children (six boys, two girls) with a mean age of 10.5 years (range 4–17 years). The interventions included percutaneous biliary drainage (five patients), percutaneous biliary drainage and placement of a self-expanding metallic stent (two patients), and percutaneous cholecystostomy (one patient). All patients had signs of obstructive jaundice and two had cholangitis. Results All procedures were successful. No procedure-related mortality was observed. Bilirubin levels returned to normal in four of the eight patients. Findings of cholangitis resolved in the two affected patients after the procedure and antibiotic treatment. Two patients underwent surgery after percutaneous biliary drainage procedures. A self-expanding metallic stent was placed in two patients with malignancy and the stents remained patent until death. Conclusion Percutaneous biliary interventions can be performed safely for the management of tumoral biliary obstruction in children.     

Cholangitis after hepatic portoenterostomy for biliary atresia: a multivariate analysis of risk factors

Cholangitis occurred in 59% of 77 patients who underwent the portoenterostomy procedure for biliary atresia between 1980 and 2000. Good postoperative bile drainage was associated with a lower risk of cholangitis than partial (odds ratio, 5.72; 95% CI, 2.89-11.3) or poor (odds ratio, 3.29; 95% CI, 1.89-5.7) bile drainage. Cholangitis was not an independent risk factor for death or liver transplantation.     

Endoscopic biliary drainage management for children with serious cholangitis caused by congenital biliary dilatation

Congenital biliary dilatation (CBD) is usually associated with complications such as recurrent cholangitis, manifested as abdominal pain, vomiting, and jaundice. If cholangitis cannot be controlled by conservative treatment, a good therapeutic effect can be obtained through percutaneous biliary drainage or open T-tube drainage. We aimed to evaluate our experiences in biliary drainage through endoscopic retrograde cholangiopancreatography in children with cholangitis caused by CBD. From January 2014 to December 2017, 167 children with CBD were treated in our hospital. 17 patients (10.18%) with serious cholangitis caused by CBD underwent ERCP. There were 4 males and 13 females with an age range of 10–120 months (average 56.4 months). Placement of a biliary stent was attempted for biliary drainage through endoscopic retrograde cholangiopancreatography. Of the 17 patients studied, 13 children had jaundice and 15 had elevated aminotransferases. ERCP showed CBD in all patients and a common biliopancreatic duct in 12 of 17 patients (70.6%). Five patients underwent nasobiliary drainage and 12 patients underwent biliary drainage through double pigtail tubes. All patients achieved successful biliary drainage. Postoperative pancreatitis occurred in one patient. Biochemical indicators decreased significantly in 12 patients (70.6%) on the second postoperative day. The average length of hospital stay after surgery was 4.5 (range 3–7) days. No major complications related to ERCP were observed and all children had a good prognosis so far. Endoscopic biliary drainage is a safe, simple, and reliable technique. It can be used to resolve CBD-associated cholangitis, evaluate the biliary tract and pancreatobiliary duct junction, and guide pediatric surgeons to choose the right time and the correct procedure for CBD.     

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     

胆道闭锁术后早期胆管炎风险因素分析

目的 探讨影响胆道闭锁术后早期胆管炎发作的风险因素. 方法 对本院2007年1月至2011年12月收治胆道闭锁患儿临床资料进行回顾性分析.所有患儿均采用统一治疗方案,包括手术方式为标准Kasai手术或肝管空肠吻合术,术中胆支引流肠襻45 cm,术后使用激素、抗菌素和利胆药物.将病例以手术年龄、术后黄疸清除效果、Ohi分型和肝纤维化病理分级进行分组,分析影响早期胆管炎发作的原因. 结果 共有139例BA患儿进行手术,124例获得随访.术后35例早期胆管炎发作,发生率为28.3％.术后黄疸完全清除率早期胆管炎发作组低于未发作组（28.2％∶71.8％,P=0.004）,术后1年、2年自体肝生存率早期胆管炎发作组低于未发作组（分别为44.5％±8.5％∶86.5％±3.6％,44.5％±8.5％∶76.3％±4.5％,P=0.003）.Logistic分析显示黄疸清除速度（P=0.000）,肝纤维化病理分级（P =0.029）和Ohi分型基本型（P =0.042）影响早期胆管炎发作而与手术年龄（P=0.579）,Ohi分型亚型（P=0.511）和肝外胆管详细分型（P =0.224）无关.结论 良好的肝门部病变类型,术后较好的胆汁引流和较轻的肝纤维化有利于减少胆道闭锁术后早期胆管炎的发生.     

Outcome of minimal surgery for hydatid cysts of the liver in children with reference to post-operative biliary leakage

Analysis of 30 children with hydatid cysts of the liver who were treated by partial pericystectomy and external tube drainage showed that five (21%) of 24 cases in whom clear hydatid fluid was observed during surgery developed biliary leakage. The cysts in the remaining six contained bile-stained fluid, indicating the presence of cystobiliary communications, and five of these children continued to drain bile post-operatively, in spite of appropriate precautions taken during surgery. Histological examination of the pericyst wall confirmed the presence of openings of small bile ducts in the cyst which probably caused the biliary leak. It is possible that some larger openings were overlooked during surgery. It is concluded from this study that biliary leakage during the post-operative period should be expected in a significant number of patients subjected to surgery for hydatid cysts of the liver. However, the leak is likely to cease spontaneously, providing distal biliary duct obstruction is ruled out and external tube drainage is used to prevent accumulation of bile in the pericyst cavity.     

Long-term prognosis in biliary atresia after hepatic portoenterostomy: analysis of 35 patients who survived beyond 5 years of age

The long-term prognosis in 35 patients with biliary atresia, who had undergone successful hepatic portoenterostomy and survived beyond 5 years of age, was studied. Eighteen (51%) patients developed ascending cholangitis after surgery. Jaundice recurred or increased in half of the patients, mainly as a result of ascending cholangitis. Another serious problem was the development of portal hypertension. Esophageal varices developed in 16 (46%) patients, with rupture and massive hemorrhage in seven. Leukopenia resulting from hypersplenism was common. Eighteen patients had WBC counts less than 5000/microliter. Thrombocytopenia was less common than leukopenia. Height was slightly below normal, although weight was within the normal range. Three patients died of hepatic failure; all had previously had ascending cholangitis. These observations indicate that ascending cholangitis is critical in the long-term and short-term prognoses in patients who have undergone successful repair of biliary atresia.     

Intrahepatic biliary tract abnormalities in children with corrected extrahepatic biliary atresia

Three children with surgically corrected extrahepatic biliary atresia developed recurrent cholangitis associated with bile lakes that failed to drain via the hepatoportoenterostomy. Surgical or percutaneous drainage of these cysts was followed by both resolution of the infection and spontaneous internal drainage. We postulate that the ongoing inflammatory process resulted in intrahepatic biliary obstruction, which caused cholangitis and bile cysts. Successful treatment required not only antibiotics but drainage of the bile lakes. Development of bile cysts is a new cause of recurrent cholangitis seen in extrahepatic biliary atresia.     

Simultaneous surgical and interventional radiological approach to treat complicated biliary strictures after pediatric liver transplantation

Post-transplantation biliary strictures occur in 5-15% of the pediatric liver transplant patients and are conventionally managed by interventional radiological techniques. Failure of this treatment leads to reoperation and sometimes to retransplantation. Herein, we describe a surgical approach and interventional radiologic approach to manage biliary strictures that failed the conventional radiologic treatment, in order to avoid retransplantation. Included in the study were eight children who underwent liver transplantation at our center or referred to our institution for evaluation of the biliary strictures that failed radiological treatment. Biliary strictures were confirmed by a narrowing of the biliary anastomosis on the percutaneous transhepatic cholangiogram. At surgery, a guide wire was introduced into the distal bile system through the use of an enterotomy in Roux limb. Over the guide wire, the stricture was ballooned and the diameter of the biliary tree was determined. A pigtail catheter was introduced on the biliary tree across the abdominal wall, the liver, the stricture and the anastomosis into the enterotomy. A final cholangiogram confirmed the positioning of the catheter. Mean follow-up was 39.8 +/- 20.8 months. All patients had their strictures successfully treated and survived the procedure. Three patients were readmitted to the hospital with fever. It was necessary to revise the hepaticojejunostomy in three patients because of cholangitis and/or recurrence of biliary stricture. Of the eight patients of this study, two required retransplantation and one died. We conclude that an aggressive combined surgical and radiologic approach can avoid retransplantation in patients with complicated post-transplant biliary strictures.     

Prophylactic antibiotics in pediatric surgery

The frequency and appropriateness of prophylactic antibiotic use in children less than 6 years of age who received surgery were examined. Antibiotics were prescribed for 62% of children who had surgery, and prophylaxis was the sole reason for antibiotic use in 73% of the patients. Prophylactic antibiotics were administered inappropriately with respect to timing or duration to 42% of the children receiving preoperative prophylaxis, 67% receiving intraoperative prophylaxis, and 55% receiving postoperative prophylaxis. Thus, prophylaxis alone is the major indication for antibiotic use in pediatric surgical patients, and prophylactic antibiotics are frequently administered inappropriately.     

Biliary atresia,an analysis of 30 cases

Of 30 cases of extrahepatic biliary atresia, treated during 1973–81, twenty five were males and five were females. Two thirds of these were admitted after 90 days of age. A correct pre operative diagnosis was made in 28 patients. Wedge liver biopsy and operative cholangiograms were done in all cases. Various types of drainage procedures done in 20 patients included hepatic porto- enterostomy 13, hepatic porto- cholecystostomy 4, choledochojejunostomy 2 and choledochoduodenostomy 1. Since 1980, an operating microscope had been used for the dissection at porta hepatis. Free flow of bile from dissected porta hepatis was observed n 14 patients (70%); 8 of them were more than three months of age. In 2 cases, in whom hepatic porto- cholecystostomy was done, bile flow stopped after some time. There were three post operative deaths due to Klebsella sepsis. At 6 to 24 months follow up, symptoms improved in 12 patients (60%). Seven of them (35%) being completely free from jaundice while 2 of these developed portal hypertension and died of haemetemesis. Mild attacks of cholangitis have been managed conservatively in 10 patients (50%).     

Biliary atresia and the Kasai operation: continuing care

Surgical intervention utilizing the Kasai hepatic portoenterostomy has improved the outcome of patients with biliary atresia and provided a population of patients with unique health problems. The clinical course of 21 children followed for three years or longer was reviewed, focusing on their medical management. Ten (47.6%) had successful bile drainage following surgery and experienced a number of specific problems including recurrent cholangitis, nutritional and growth deficiencies, delayed developmental landmarks, portal hypertension, osteomalacia and osteoporosis, and social and psychiatric difficulties. These complications responded to aggressive medical therapy and support. Although the overall three-year survival of this series was 38.1%, in children who were operated upon prior to 2 months of age and in whom the enteric conduit was externalized the three-year survival rate was 66.7%.     

Intrahepatic biliary cysts after hepatic portoenterostomy in four children with biliary atresia

We report our experience with 4 cases of cystic dilatation of intrahepatic bile ducts following hepatic portoenterostomy for biliary atresia. Two of the cases did not achieve satisfactory bile excretion and all four cases developed recurrent cholangitis after hepatic portoenterostomy. The attacks of cholangitis seemed to be associated with the presence of intrahepatic cysts. Although one case resulted in death from hepatic failure, three other cases are now outpatients. Patients who develop recurrent cholangitis following hepatic portoenterostomy, should be examined to exclude the presence of intrahepatic biliary cysts. Ultrasonography, computed tomography and percutaneous transhepatic cholangiography were all effective in detecting cysts and provided valuable information for planning treatment. Percutaneous transhepatic or surgical drainage of the bile ducts was effective in reducing jaundice, and recurrent cholangitis.     

Hepatic duct stone associated with chlamydia sepsis: a rare condition in childhood

Stone formation in the biliary system is a rare condition in infants. A few cases of bile stones in the biliary tree have been reported with underlying predisposing factors, such as sepsis and antibiotic usage. This article describes a surgically treated 16-week-old infant with recurrent cholangitis who had a bile stone in the hepatic duct after chlamydia sepsis. Accepted: 4 February 1997     

Hepatic porto-enterostomy or cholecystostomy in the treatment of extrahepatic biliary atresia. A study of 49 cases.

Hepatic porto-enterostomy or cholecystostomy (Kasai's procedure) was successful in restoring bile flow in 31 of 49 patients with "noncorrectable" extrahepatic biliary atresia. However, all but one of the 31 developed acute or chronic complications such as cholangitis, bile peritonitis, or portal hypertension. During a five-year follow-up period, 26 (53%) died while 9 of the 23 survivors continue to manifest chronic or recurrent cholangitis. Thirteen of the 19 survivors who are more than one year of age have developed portal hypertension. These complications limit the prognosis of infants with "noncorrectable" biliary malformations.     

Biliary atresia with a “cyst at porta”: management and outcome as per the cholangiographic anatomy

The purpose of this study is to classify biliary atresia (BA) with a "cyst at porta" according to the cholangiographic anatomy and to define management strategy and outcome in each group. A cyst at porta was identified in 13 of 58 babies (22.4%) with BA at first presentation. The cholangiographic anatomy was classified as; Group A (n = 7), type III BA with extrahepatic cyst; Group B (n = 2), type I or II BA with extrahepatic biliary cyst; and Group C (n = 4), type I or II BA with both extrahepatic and intrahepatic biliary cysts. The remaining 45 patients were comprised of type III BA without a cyst. A Kasai's portoenterostomy (PE) was performed for all Group A patients. Groups B and C were treated by hepaticojejunostomy (n = 5) or portoenterostomy (n = 1). All 45 patients with type III BA without a cyst were treated by a Kasai's PE. The median age at surgery was 92 days (ranges 28-342 days). There were three early post-operative deaths, all in patients with type III BA without cyst. Overall 18/55 (32.7%) patients achieved a jaundice free state. In Group A, 5/7 (71.4%) patients had bile flow, 2/7 (28.6%) are anicteric and 2/7(28.6%) had 1-2 episodes of post-operative cholangitis. In Group B, both patients are anicteric and none had post-operative cholangitis. In Group C, all four babies had bile flow but, significant morbidity because of recurrent severe cholangitis. Only one patient reached a jaundice free state. Of the remaining 42 patients with type III BA without a cyst, 27 (64.3%) had bile flow, 13 (31%) became jaundice free and 14 (33.3%) have had 1-2 episodes of post-operative cholangitis. In conclusion, thirteen of 58 (22.4%) babies with BA had a "cyst at porta" at first presentation in this series. The outcome was most satisfactory in type I BA without intrahepatic cystic dilatation (Group B) in terms of achieving a jaundice free state and freedom from recurrent cholangitis. However, intrahepatic biliary cysts (Group C) were associated with recurrent severe cholangitis and a poor eventual outcome despite a good initial bile flow. The outcome in type III BA with extrahepatic cyst was comparable to type III BA without cyst.     

胆总管未闭锁型胆道闭锁的手术治疗及疗效分析

目的 分析24例胆总管未闭锁型胆道闭锁的临床疗效,旨在指导临床对此类胆道闭锁的手术方式进行选择.方法 回顾分析我院1995至2006年间收治的24例胆总管未闭锁型胆道闭锁患儿手术、术后病理及随访资料.对空肠肝门吻合(A组)与胆囊肝门吻合(B组)在自体肝生存率、黄疸清除率、反流性胆管炎发生率及临床疗效等方面比较分析;并对胆道闭锁胆囊病理标本及正常对照组胆囊进行胶原纤维Van Gieson染色和CD68免疫组化染色,比较其纤维化及炎性反应的差异.结果 平均(51±29.02)个月随访期内,自体肝生存率为66.7%.A组自体肝1年生存率为78.6%,3年生存率78.6%;B组1年生存率为66.7%,3年生存率33.2%,两组3年生存率差异有统计学意义(P＜0.05);A组术后6个月黄疸清除率明显高于B组(86.7%比33.3%,P＜0.05);术后1年胆管炎发生率,A组为60.0%,B组为44.4%(P＞0.05).胆道闭锁患儿胆囊表现为明显纤维化和CD68阳性细胞浸润.结论 胆囊肝门吻合治疗胆总管未闭锁型胆道闭锁的疗效较肝门空肠吻合差,可能与胆囊肝门吻合后残余胆道存在纤维化及炎症细胞浸润有关,对其治疗推荐采用肝门空肠吻合.