Clinical and pathological challenges in the diagnosis of late-onset biliary atresia: four case studies

Biliary atresia (BA) is classically described at the neonatal age. However, rare cases of BA in older infants have also been reported. We report four cases of late-onset BA in infants older than 4 weeks (3 males, 1 female), and describe the diagnostic and management difficulties. One of the cases had a late-onset (29 weeks) presentation with a successful surgical procedure. We highlight the importance of this unusual differential diagnosis in infants with cholestatic syndrome, who may benefit from Kasai surgery, regardless of age.

Current thinking on the treatment of biliary atresia / 天津医药

This article made a historic review on the treatment and diagnosis process of biliary atresia at home and abroad, and analyzed the problems of diagnosis and treatment on biliary atresia. The author points out that there is no registered system in domestic, which results in screening job has been unable to conduct substantive work for many years. The author also puts forward his own views about the postoperative influence factors including the time of Kasai operation, postoperative cholangitis, biliary cirrhosis and other issues, and suggests that improving the prognosis of children with biliary atresia needs the cooperation among national colleagues to work together.

RESULT OF KASAI OPERATION, CHILDREN LIVER TRANSPLANTATION IN MONGOLIA / Мэс засал

Introduction: Biliary Atresia is a fibroobliterativedisorder of the intra andextrahepatic bile ducts in infancy, which isgoing progressively cholestatic liver disease.The failed Kasaiportoenterostomy requiresliver transplantation. The goal of this studyis to show the outcome of Kasai operation,recent improvement and correlation the datato overseas.Methods and Materials: This study wasconducted in the department of generalsurgery of National Center for Maternal andChild Health of Mongolia between 2010 and2016 on a total of 66 infancies with biliaryatresia.Results: Patient diagnosed with biliaryatresia, which performed Kasai operationwithin first 2 months the outcome is verygood early and late post-operation period.There were 3 patients with 10 year survival, 4patients with 5-10 year and 28 patients with5 year survival after Kasai operation. The mostcomport age for liver transplantation is 1 yearlater after Kasai operation in Mongolia. Livertransplantation programme is necessary forMongolian pediatric surgery, and we thoughtour team was assembled.Conclusion: The children with biliary atresiaperform the Kasai operation within 2 monthsthe outcome is very good. Children with biliaryatresia often experience long wait times fortransplant unless exception points are grantedto reflect severity of disease.In Mongolia livertransplantation done in 2 child.

The etiology and treatment of cholangitis after Kasai operation in biliary atresia patients / 天津医药

Cholangitis is one of the most common complications after Kasai operation in children with biliary atresia (BA), whose precise etiology is still unclear. The occurrence of cholangitis may be the results of concurrent effects of various factors such as the structural change of intrahepatic bile duct or insufficient volume of bile flow. The diagnosis for cholangitis is based on clinical manifestations nowadays, and combined therapies including antibiotics, steroids and hepatoprotectants have been used empirically. The prophylaxis and treatment of postoperative cholangitis is the key to improve the liver survival of biliary atresai patients. Only by figuring out the etiology of cholangitis that we can prevent it and make sure the long-term survival of BA patients.

Research progress on the survival of autologous liver in children with biliary atresia after Kasai operation / 天津医药

Biliary atresia (BA) is a kind of disease of unknown etiology, characterized by progressive inflammation and fibrosis of obstructive biliary diseases. Kasai portoenterostomy is the only method to treat BA. However, about 80% of the patients treated by Kasai operation still need liver transplantation in the future. Many factors affect the survival of autologous liver in children with BA after Kasai operation, including the types of BA, laparoscopic Kasai surgery or traditional open surgery, patient’s age at surgery, condition of liver function, occurrence of cholangitis, jaundice clearance, using steroids and central hospitalization. This article reviews the factors that affect the survival of autologous liver in patients with BA after Kasai surgery.

Outcome of Alagille Syndrome Patients Who Had Previously Received Kasai Operation during Infancy: A Single Center Study / 대한소아소화기영양학회지

PURPOSE: Infants with Alagille syndrome (AGS) are occasionally misdiagnosed as biliary atresia and subsequently undergo Kasai operation. The purpose of this study was to investigate the outcome of patients with AGS who had previously received Kasai operation during infancy. METHODS: This retrospective study was conducted at the Department of Pediatrics, Samsung Medical Center. We compared the prognosis and mortality between those who had undergone Kasai operation during infancy (Kasai group) and those who had not (non-Kasai group). RESULTS: Among the 15 children with AGS, five had received Kasai operation, while 10 had not. All subjects in the Kasai group revealed neonatal cholestasis, while 70% of the non-Kasai group showed neonatal cholestasis. Liver transplantation was performed in 100% (5/5) among the Kasai group, and 20.0% (2/10) among the non-Kasai group (p=0.007). Mortality was observed in 60.0% (3/5) among the Kasai group, and 10.0% (1/10) among the non-Kasai group (p=0.077). CONCLUSION: Although overall mortality rate did not significantly differ between the two groups, the proportion of patients receiving liver transplantation was significantly higher in the non-Kasai group. The relatively worse outcome in AGS patients who had received Kasai operation may be due to the unfavorable influences of Kasai operation on the clinical course of AGS, or maybe due to neonatal cholestasis, irrespective of the Kasai operation.

Atresia biliar: una enfermedad grave / Biliary atresia: a severe illness

La atresia biliar es una grave enfermedad que se manifiesta en los recién nacidos, y se desconoce su causa. La inflamación y destrucción progresiva de los conductos biliares conducen a la aparición de ictericia, coluria y acolia entre la segunda y sexta semana de vida. Como existen múltiples causas de colestasis neonatal en esta etapa de la vida, es necesario realizar un diagnóstico y derivación precoz para ofrecer un tratamiento quirúrgico, con el fin de restablecer el flujo biliar. Alrededor del 80% de los pacientes normalizan la bilirrubina luego de la portoenterostomía (operación de Kasai), realizada antes de los 45 días de vida. Si la operación fracasa, el trasplante hepático surge como única alternativa. La atresia biliar debe diagnosticarse durante el primer mes de vida y ser considerada una urgencia quirúrgica.

Biliary atresia is a serious disease of unknown cause, affecting newborns. An inflammation and progressive destruction of the bile ducts lead to jaundice, dark urines, and acholia, between the second and sixth weeks of life. Neonatal cholestasis could be due to several different diseases, thus a diagnosis of biliary atresia and early derivation for surgical treatment are necessary to allow a restoration of the bile flow. Eighty percent of the children normalize serum bilirubin after the portoenterostomy (Kasai operation), if they are operated before their 45 days of life. When Kasai operation fails, a liver transplantation is the only possibility. Biliary atresia must be diagnosed before the first month of life and must be considered as a surgical emergency.

Effect of fibrotic plates of porta hepatis on the prognosis of Kasai procedure with biliary atresia / 中华实用儿科临床杂志

Objective To investigate the correlation between the remnants of extrahepatic bilie duct with the prognosis of biliary atresia after Kasai operation.Methods From Sep.2005 to Sep.2011,93 cases of biliary atresia [51 boys and 42 girls,ages at operation:61 days(40-121 days)] in Tongji Hospital Affiliated of Tongji Medical College of Huazhong University of Science and Technology were operated with Kasai procedure,and the diagnosis of type Ⅲ biliary atresia was based on the cholangiography and the intraoperative findings.According to whether there was fibrotic remnant plates of porta hepatis or not,type Ⅲ biliary atresia cases were grouped into 2 subtypes:subtype A,in which there was fibrotic remnant plates of porta hepatis (n =80) ; subtype B,in which there was no fibrotic remnant plates,but solid cords structure like hepatic duct was observed(n =13).Specimens of the remnants were colleted in operation and pathologic section detected the degree of bile ductular proliferation in the remnants.Postoperatively,all of the patients were followed up.The contents included the cholangitis occurrence rate,the jaundice disappearance rate and survival rate during postoperative 2 years.Results The patients had more bile ductular proliferation in subtype A (x2 =18.49,P ＜0.05).There was no significant difference in incidence of cholangitis in postoperative 1 month between the 2 groups (x2 =0.01,P ＞ 0.05).The jaundice disappearance rate in postoperative 6 months of subtype A was higher than the rate of subtype B(x2 =9.19,P ＜ 0.05).The survival rate during postoperative 2 years of subtype A was higher than the rate of subtype B(x2 =4.49,P ＜ 0.05).Conclusions There are higher jaundice disappearance rate and 2 years survival rate in type Ⅲ biliary atresia with the fibrotic remnant plates of porta hepatis,which suggest that there is more bile ductular proliferation in the extrahepatic remnants which is good for biliary drainage in subtype A after Kasai operation,and it can slow down hepatic injury and have a long-term good life quality.

MRI Findings of Long-term Survivals after Kasai Portoenterostomy

The purpose of this study is to analyse clinical impact of specific MRI findings in liver in patients of long-term survivors after Kasai portoenterostomy (KPE). Twenty-eight patients who were underwent KPE were followed up more than 5 years. Macro-regenerative nodule (MRN) and beaded-duct dilatation (BDD) were considered as important findings in liver MRI. The association between these findings in MRI and clinical indicator, serum bilirubin level and history of cholangitis were evaluated. Sixteen patients (57.1%) were shown MRN in liver MRI. There were 14 patients(50%) whose MRI showed BDD. Serum total and direct bilirubin were 3.6mg/dL and 1.8mg/dL respectively in positive MRN group whereas 1.4mg/dL and 0.7mg/dL in negative MRN group (p 0.427). Serum total and direct bilirubin level were 4.2mg/dL and 2.1mg/dL in patients with BDD negative group compare to 1.1mg/dL and 0.5mg/dL in BDD positive group (p 0.281). The odds ratio to have cholangitis in the patient with MRN was 2.3 and 0.53 in patient with BDD in their MRI findings. MRN in liver MRI may suggest high bilirubin level and more chance to have cholangitis, but the findings of BDD may related to low bilirubin level and less change to have cholangitis.

Experience of Biliary Atresia-Long-term Survival

Biliary atresia (BA) is an uncommon neonatal surgical disease that has a fatal outcome if not properly treated. The survival rates of the patients with native liver after Kasai's operation in countries outside Japan are not so good. We reviewed the results of 22 cases of biliary atresia treated in Kosin University Hospital between October 1987 and March 2001. There were 13 males and 9 females aged from 21 to 106 days (mean 52 days). There were 3 cases of Type I (13.6%), and 3 of Type II (13.6%), and 16 Type III (72.7%). The operative methods were resection of the common bile duct remnant and cyst followed by Roux-en-Y hepaticojejunostomy in 3 cases for Type I BA; Kasai I in 15 cases, Kasai II in 1 case, and Ueda's operation in 3 cases for Types II and III BA. There was no death within the first 30 days after operation. We were able to follow 21 of the 22 patients (95.4%) for more than 5 years. The actual 5 year survival rate (YSR) was 40.9%. One Type I case received a living-related liver transplantation at 6 years of age because of the multiple intrahepatic stones and liver cirrhosis. Five YSR after biliostomy group (Kasai II and Ueda op.) was 75% (3/4) while that of Kasai I was 20% (3/15). One case had no bile duct in the resected fibrotic plaque on microscopic review and died 8 months after Kasai I operation, would have been a strong candidate for early liver transplantation. From the above result, our conclusions are as follows; (1) early liver transplantation should be considered for cases of no bile duct after pathologic examination of the resected specimen, (2) measures to prevent postoperative cholangitis and prevention of postoperative liver cirrhosis are needed, (3) liver transplantation program should be available for failed cases.

Long-term Outcome after Surgery for Biliary Atresia (Study of 10 patients surviving more than 10 years)

Biliary atresia (BA) is the result of fibrosing destructive inflammatory process affecting intrahepatic and extrahepatic bile ducts, which lead to cirrhosis and portal hypertension. Kasai portoenterostomy has been the standard operative procedure in biliary atresia. Recently, there has been remarkable increase in the survival rate in cases of BA. However, long-term survivors are not clearly evaluated in Korea. To define long-term prognosis factors of patients who underwent surgery for BA, a retrospective study was undertaken of 10 (37 %) patients surviving more than 10 years among 27 patients who underwent one of Kasai procedures between 1981 and 1995. Hepatomegaly was present in 4 and splenomegaly in 7 patients. Serum bilirubin was normalized at 1 year after operation. Aspartate aminotransferase (AST, GOT), Alanine aminotransferase(ALT, GPT) were normalized at 12 years and alkaline phosphatase(ALP) was normalized at 13.5 years. Cholangitis developed mainly within 5 years after operation so close follow up is needed. Life long follow-up is needed because of progressive deterioration of liver function even after 10 years.