scholarly journals Management of Biliary Atresia: A Review

2016 ◽  
Vol 39 (1) ◽  
pp. 38-45
Author(s):  
Khan Lamia Nahid ◽  
Md Rokonuzzaman ◽  
ASM Bazlul Karim ◽  
Kaniz Fatema ◽  
Azmeri Sultana
Keyword(s):  
Liver Transplantation ◽  
Biliary Atresia ◽  
Biliary Tree ◽  
Unknown Etiology ◽  
Biliary Cirrhosis ◽  
Dark Urine ◽  
Kasai Operation ◽  
Age At Surgery ◽  
Life Threatening ◽  
Optimum Management

Biliary atresia (BA) is a rare neonatal disease of unknown etiology, where obstruction of the biliary tree causes severe cholestasis, leading to biliary cirrhosis and death in the first years of life, if the condition is left untreated. It is the most common cause of neonatal cholestasis and most common indication for liver transplantation in children. Biliary Atresia clinically presents with jaundice, pale stool, dark urine and hepatomegaly. The treatment of biliary atresia is surgical and is recommended as a sequence of, eventually, two interventions. During the first months of life a hepatoportoenterostomy (Kasai operation) should be performed, in order to restore the biliary flow to the intestine and lessen further damage to the liver. If this fails and/ or the disease progresses towards biliary cirrhosis and life-threatening complications, then liver transplantation is indicated. Age at surgery had a progressive and sustained deleterious effect on the results of the Kasai operation until adolescence. So early detection of biliary atresia is necessary for optimum management and overall survival of the patient.Bangladesh J Child Health 2015; VOL 39 (1) :38-45

scholarly journals Biliary Atresia: 50 Years after the First Kasai

ISRN Surgery ◽  
2012 ◽  
Vol 2012 ◽  
pp. 1-15 ◽  
Author(s):  
Barbara E. Wildhaber
Keyword(s):  
Liver Transplantation ◽  
Biliary Atresia ◽  
Clinical Sign ◽  
Great Degree ◽  
Biliary Tree ◽  
Unknown Etiology ◽  
Biliary Cirrhosis ◽  
Systematic Screening ◽  
Life Threatening ◽  
Pediatric Indication

Biliary atresia is a rare neonatal disease of unknown etiology, where obstruction of the biliary tree causes severe cholestasis, leading to biliary cirrhosis and death in the first years of life, if the condition is left untreated. Biliary atresia is the most frequent surgical cause of cholestatic jaundice in neonates and should be evoked whenever this clinical sign is associated with pale stools and hepatomegaly. The treatment of biliary atresia is surgical and currently recommended as a sequence of, eventually, two interventions. During the first months of life a hepatoportoenterostomy (a “Kasai,” modifications of which are discussed in this paper) should be performed, in order to restore the biliary flow to the intestine and lessen further damage to the liver. If this fails and/or the disease progresses towards biliary cirrhosis and life-threatening complications, then liver transplantation is indicated, for which biliary atresia represents the most frequent pediatric indication. Of importance, the earlier the Kasai is performed, the later a liver transplantation is usually needed. This warrants a great degree of awareness of biliary atresia, and the implementation of systematic screening for this life-threatening pathology.

scholarly journals Role of viruses in biliary atresia: news from mice and men

2018 ◽  
Vol 3 (2) ◽  
pp. 101-106 ◽  
Author(s):  
Claus Petersen ◽  
Omid Madadi-Sanjani
Keyword(s):  
Biliary Atresia ◽  
Inflammatory Process ◽  
Biliary Tree ◽  
Unknown Etiology ◽  
Symptomatic Therapy ◽  
Kasai Procedure ◽  
Current State ◽  
State Of Research ◽  
Oriented Approach

AbstractBiliary atresia (BA) is still an enigmatic disease of unknown etiology and cryptic pathomechanism. Despite the fact that BA is rated among rare diseases, it represents the most frequent indication for pediatric liver transplantation. Although every effort is made to elucidate the origin of the ongoing deterioration of liver function, no breakthrough has so far been achieved, which switches the surgical but symptomatic therapy to a cause-oriented approach. The nowadays leading hypothesis focuses on hepatotropic virus as a triggering agent for an autoimmunological self-limiting inflammatory process along the entire biliary tree. The present review highlights the current state of research on the factor “viruses in biliary atresia” in both patients undergoing the Kasai procedure and the virus-induced BA mouse model.

scholarly journals How to evaluate diagnosis and management of biliary atresia in the era of liver Transplantation in China

2018 ◽  
Vol 1 (1) ◽  
pp. e000002
Author(s):  
Jianghua Zhan ◽  
Yajun Chen ◽  
Kenneth K Y Wong
Keyword(s):  
Liver Transplantation ◽  
Survival Rate ◽  
Early Diagnosis ◽  
Biliary Atresia ◽  
Further Education ◽  
Monitoring Methods ◽  
Kasai Operation ◽  
First Choice ◽  
Choice Of Treatment ◽  
Hepatobiliary Diseases

BackgroundBiliary atresia (BA) is one of the most challenging hepatobiliary diseases in children. Notwithstanding the reasonable outcome of liver transplantation (LT), portoenteral anastomosis (Kasai operation) is still the first choice of treatment for patients with BA. How to improve the survival rate of the patients with BA in the era of LT is a new challenge.Data sourcesBased on recent original publications and the experience with the BA in China, we review many factors that influence BA survival situation, including early diagnosis and screen plan, defects of early treatment, Kasai operation, and LT and indicate present questions about BA diagnosis and treatment in China.ResultsBA diagnosis may also be delayed due to insufficient understanding of BA and lack of jaundice monitoring methods at different levels of the hospitals in China. Further education of the physicians at smaller city hospitals about BA, neonatal jaundice and cholestasis would be helpful in improving early diagnosis of BA. Early surgical intervention is still the only guarantee to improve the survival rate of BA with native liver.ConclusionsIn the era of LT, especially in China, Kasai operation can provide waiting time for living donor LT and improve the success rate of LT and minimize the rapid deterioration of liver function of the children with BA and decrease the mortality in patients with BA.

scholarly journals Sequential Treatment of Biliary Atresia With Kasai Hepatoportoenterostomy and Liver Transplantation: Benefits, Risks, and Outcome in 393 Children

2021 ◽  
Vol 9 ◽  
Author(s):  
Roberto Tambucci ◽  
Catherine de Magnée ◽  
Margot Szabo ◽  
Aniss Channaoui ◽  
Aurore Pire ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Biliary Atresia ◽  
Graft Survival ◽  
Bowel Perforation ◽  
Survival Rates ◽  
Sequential Treatment ◽  
Biliary Cirrhosis ◽  
End Stage ◽  
Sequential Strategy ◽  
Early Reoperation

Introduction: Surgical treatment of biliary atresia (BA) is still based on sequential strategy with Kasai hepatoportoenterostomy (KP) followed by liver transplantation (LT), in case of complicated secondary biliary cirrhosis. Concerns have been expressed regarding the risks of LT related to previous KP, suggesting primary LT as an exclusive treatment of BA.Methods: Single-center retrospective analysis including 393 pediatric patients who underwent LT for BA from 1993 to 2018, categorized into two groups: with (KP) or without (NoKP) previous KP. Pre-LT clinical condition was estimated considering age at LT, time on waiting list, pediatric end-stage liver disease score (PELD), and presence of portal vein hypoplasia. Post-LT outcome was evaluated considering patient and graft survival rates, and need for early reoperation due to abdominal or graft-related complications (<45 days after LT).Results: Two-hundred ninety-six patients (75.3%) were categorized in the KP group, and 97 (24.7%) in the NoKP group. Median age at LT was 1.14 years in the KP group and 0.85 years in the NoKP group (p < 0.0001). PELD score was significantly less severe in KP patients (p < 0.05). One-year patient survival rates were 96.9 and 96.8% in the KP and NoKP groups, respectively (p = 0.43), and the corresponding graft survival was 92.5 and 94.8% (p = 0.97). The need for early reoperation was more frequent in the KP group (29.8%) vs. NoKP group (12.4%, p = 0.01). The rate of bowel perforation was non-significantly higher in the KP group (8.1%) vs. NoKP group (3.1%, p = 0.11).Conclusions: The sequential strategy including KP and LT allowed performing LT in patients with significant older age and better clinical conditions, when compared to those transplanted without previous KP. Patient and graft survivals were not impacted by previous KP. Although previous KP was associated with an increased rate of post-LT surgical complications, bowel perforation and bleeding did not occur significantly more frequently. Such results support the current strategy based on sequential treatment.

scholarly journals Biliary Atresia Associated with Polysplenia Syndrome, Dextrocardia, Situs Inversus Totalis and Malrotation of Intestines

2014 ◽  
Vol 3 (1) ◽  
pp. 9
Author(s):  
Praveen Mathur ◽  
Rahul Gupta ◽  
Varsha Soni ◽  
Reyaz Ahmed ◽  
Ram Babu Goyal
Keyword(s):  
Biliary Atresia ◽  
Rare Disease ◽  
Situs Inversus ◽  
Congenital Anomalies ◽  
Female Child ◽  
Situs Inversus Totalis ◽  
Biliary Cirrhosis ◽  
Polysplenia Syndrome ◽  
Kasai Operation

Biliary atresia (BA) is a rare disease and the end result of a destructive, inflammatory cholangiopathy, leading to fibrosis and biliary cirrhosis. It is classified into syndromic variety with various congenital anomalies and non-syndromic (isolated anomaly). We present here a 1-month-old female child with the syndromic variety of BA associated with polysplenia syndrome, dextrocardia, situs inversus totalis and malrotation of intestines. She developed jaundice in the first week of life. Kasai operation was performed but she developed cholangitis and septicemia 2.5 months after surgery and succumbed later.

Biliary Atresia Revisited

2004 ◽  
Vol 7 (2) ◽  
pp. 109-124 ◽  
Author(s):  
Ellen Kahn
Keyword(s):  
Biliary Atresia ◽  
Congenital Anomalies ◽  
Cell Transformation ◽  
Biliary Tree ◽  
Bile Acid Metabolism ◽  
Biliary Cirrhosis ◽  
Intrahepatic Bile Ducts ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin ◽  
Extrahepatic Biliary Tree

Extrahepatic biliary atresia (EHBA) is an inflammatory fibrosing process affecting the extrahepatic and intrahepatic biliary tree resulting in fibrous obliteration of the extrahepatic biliary tract, ductopenia of intrahepatic bile ducts, and biliary cirrhosis. EHBA is divided into a correctable and a noncorrectable type with focal patency of the otherwise atretic biliary tree in the former and no patency of the biliary tree in the noncorrectable type. EHBA is divided in a fetal, prenatal or embryonic, and a more common, perinatal, acquired form. The symptoms of the former start shortly after birth and there is frequently an association with a variety of congenital anomalies. Children with the perinatal form become jaundiced several weeks after birth; no associated congenital anomalies are present. Morphologically, an inflammatory and fibrosing process of the extrahepatic biliary tree leads to complete lumenal obliteration. The liver is characterized by a nonspecific giant cell transformation, and portal expansion by fibrous connective tissue with marked ductular proliferation. With time, ductopenia and biliary cirrhosis develop. The diffential diagnosis with other conditions with similar microscopic patterns such as alpha-1 antitrypsin deficiency, total parental nutrition, obstruction by a choledochal cyst, arteriohepatic dysplasia, familial progressive intra-hepatic cholestasis, and alteration of the bile acid metabolism is discussed. In the fetal group, abnormalities in different genes seem to play a role; ductal plate malformation is another possibility. Different etiologies have been postulated in the perinatal form of EHBA: genetic susceptilibility, vascular factors, toxins, and infections mainly by rotavirus and reovirus. The pathogenesis is complex. EHBA is a heterogenous disease, resulting from a combination of genetic factors, insults, and activation of different genetic and immunologic pathways. The treatment of EHBA is surgical, with anastomosis between the biliary tree and the intestine in the correctable type and a hepatic portoenterostomy (HPE) for the noncorrectable group. HPE is a temporizing treatment allowing the infant to develop and grow, followed in the majority of the patients by liver transplantation.

scholarly journals Long-term Outcome of Kasai Operation on Children with Biliary Atresia between 2001-2010 in Namazi Hospital, Shiraz, Iran

2014 ◽  
Vol 3 (2) ◽  
pp. 109-114
Author(s):  
Seyed Mohsen Dehghani ◽  
Fatemeh Ghorban nejad ◽  
Mahmoud Haghighat ◽  
Hamid Reza Forootan ◽  
Ali Bahador
Keyword(s):  
Risk Factors ◽  
Liver Transplantation ◽  
Biliary Atresia ◽  
Operation Time ◽  
Influential Factors ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Kasai Operation ◽  
Kaplan Meier ◽  
Time Of Operation

Background: Biliary atresia is a 100% fatal disorder without any treatment in infants as a leading cause of cirrhosis and Kasai operation, as the only operative choice, which plays a crucial role in increasing their rate of survival. Nonetheless, many patients end up with liver transplantation in the future owing to various inevitable hepatic and biliary problems which do remain after the Kasai operation. In this study, we aimed to assess the prognosis and influential factors on the outcome of this procedure on children attending Shiraz Namazi Hospital.Patients and Methods: A database of medical records of 61 patients (F=41, M=20) with biliary atresia, aged between 30 to 100 days (65.27±18 days) at the time of operation, who had undergone the Kasai operation in Shiraz Namazi Hospital from 2001 to 2010 was examined in a retrospective review. Risk factors of failure (death and liver transplantation) were analyzed by Kaplan-Meier test.Results: Age at the operation time, type of atresia, and dilatation of the bile ducts were identified as the risk factors; 29 patients had signs of hepatomegaly, 6 patients had liver transplantation. Among them, 3 survived.Conclusion: Of the patients, 61% with biliary atresia survived for more than 5 years with their native liver while the overall survival rate among 52 patients was 85.30±5.96 months. Postoperative care and regular checkups should be considered for all survivors as a lifelong procedure to prevent any possibilities of future hepatic deterioration.

Sign in / Sign up

Export Citation Format

Share Document