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 Large-duct cholangiopathies: aetiology, diagnosis and treatment

2019 ◽  
Vol 10 (3) ◽  
pp. 284-291 ◽  
Author(s):  
Shyam Menon ◽  
Andrew Holt
Keyword(s):  
Bile Flow ◽  
Biliary Tree ◽  
Biliary Cirrhosis ◽  
Caroli Disease ◽  
End Stage Liver Disease ◽  
Recurrent Pyogenic Cholangitis ◽  
Chronic Cholestasis ◽  
End Stage ◽  
Extrahepatic Biliary Tree ◽  
Portal Biliopathy

Cholangiopathies describe a group of conditions affecting the intrahepatic and extrahepatic biliary tree. Impairment to bile flow and chronic cholestasis cause biliary inflammation, which leads to more permanent damage such as destruction of the small bile ducts (ductopaenia) and biliary cirrhosis. Most cholangiopathies are progressive and cause end-stage liver disease unless the physical obstruction to biliary flow can be reversed. This review considers large-duct cholangiopathies, such as primary sclerosing cholangitis, ischaemic cholangiopathy, portal biliopathy, recurrent pyogenic cholangitis and Caroli disease.

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.

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 Recent advances in understanding biliary atresia

F1000Research ◽  
2019 ◽  
Vol 8 ◽  
pp. 218 ◽  
Author(s):  
Andrew Wehrman ◽  
Orith Waisbourd-Zinman ◽  
Rebecca G Wells
Keyword(s):  
Biliary Atresia ◽  
Extrahepatic Bile Duct ◽  
Biliary Tree ◽  
Liver Parenchyma ◽  
Early Diagnostics ◽  
The Past ◽  
Recent Advances ◽  
Neonatal Liver ◽  
Extrahepatic Biliary Tree ◽  
Extrahepatic Bile Duct Obstruction

Biliary atresia (BA) is a neonatal liver disease characterized by progressive obstruction and fibrosis of the extrahepatic biliary tree as well as fibrosis and inflammation of the liver parenchyma. Recent studies found that infants who will go on to develop BA have elevated direct bilirubin levels in the first few days of life, suggesting that the disease starts in utero. The etiology and pathogenesis of BA, however, remain unknown. Here, we discuss recent studies examining potential pathogenetic mechanisms of BA, including genetic susceptibility, involvement of the immune system, and environmental insults such as viruses and toxins, although it is possible that there is not a single etiological agent but rather a large group of injurious insults that result in a final common pathway of extrahepatic bile duct obstruction and liver fibrosis. The management and diagnosis of BA have not advanced significantly in the past decade, but given recent advances in understanding the timing and potential pathogenesis of BA, we are hopeful that the next decade will bring early diagnostics and novel therapeutics.

scholarly journals Paucity of intrahepatic bile ducts in infancy: experience of a tertiary center

2004 ◽  
Vol 41 (3) ◽  
pp. 190-192 ◽  
Author(s):  
Adriana Maria Alves De Tommaso ◽  
Agnes Sumi Kawasaki ◽  
Gabriel Hessel
Keyword(s):  
Bile Duct ◽  
Bile Ducts ◽  
Portal Tract ◽  
Intrahepatic Bile Duct ◽  
University Hospital ◽  
Intrahepatic Bile Ducts ◽  
Tertiary Center ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

BACKGROUND: Intrahepatic cholestasis secondary to paucity of bile duct is an alteration of the anatomic integrity of the biliary tract. Can be defined only histologically and, clinically, two categories are recognized: syndromic and non-syndromic, where the prognosis is generally more severe. AIM: To evaluate the history, clinical and biochemical characteristics, etiology and improvement of children who have paucity of intrahepatic bile duct followed at tertiary center. PATIENTS AND METHODS: Eleven children with paucity of intrahepatic bile duct, followed at the Pediatric Hepatology Service of the University Hospital, Campinas, SP, Brazil, were evaluated in the period from 1986 to 2001. RESULTS: Among the patients, three presented the syndromic and eight the non-syndromic form (two with alpha-1-antitrypsin deficiency, one with lues, one secondary to sepsis, three with probable etiology by cytomegalovirus and one without a definite etiology). Referral ranged from 31 to 1185 days. Birth weights ranged from 1920 g to 3590 g. Most of the patients presented pale stools. The median bile duct/portal tract ratio was 0.14. The majority of the children presented a favorable follow-up, regardless of the form of presentation. CONCLUSION: Paucity of intrahepatic bile ducts should be considered in children with cholestasis and its differentiation from extrahepatic causes of neonatal cholestasis is important in order to avoid surgery. Diagnosis of non-syndromic form should not be regarded as unfavorable prognosis, as the evolution is probably related to the etiology in this form of presentation.

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: evaluation on two distinct periods at a reference pediatric service

2014 ◽  
Vol 51 (1) ◽  
pp. 53-58 ◽  
Author(s):  
Thais Costa Nascentes QUEIROZ ◽  
Alexandre Rodrigues FERREIRA ◽  
Eleonora Druve Tavares FAGUNDES ◽  
Mariza Leitão Valadares ROQUETE ◽  
Francisco José PENNA
Keyword(s):  
Biliary Atresia ◽  
Waiting Time ◽  
Neonatal Period ◽  
Biliary Tree ◽  
Probability Of Survival ◽  
Age At Surgery ◽  
Pediatric Service ◽  
Extrahepatic Biliary Tree ◽  
Observation Period

Context Biliary atresia is a progressive, idiopathic, fibro-obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction exclusively in the neonatal period. Objectives To assess the differences regarding age at referral, age at surgery, duration of propaedeutics and waiting time for surgery between two groups of infants in different periods. Methods Retrospective study of infants diagnosed with biliary atresia on two periods: 1983-1993 and 1998-2011. Results Biliary atresia was diagnosed in 129 infants, being 48 in casuistic I and 81 in casuistic II. The median age at admission was 94 and 60 days, respectively (P = 0.0001). On evaluating patients who had undergone portoenterostomy before 120 days of age, no difference was observed regarding the duration of propaedeutics or waiting time for surgery (P = 0.15), but difference was found when comparing the age at surgery (P = 0.002). Among those infants with no biliary flow and without liver transplantation or death after 18 post-operative months, the estimated probability of survival was 44.6% and 38.7% in casuistics I and II, respectively. In casuistic I, all infants who showed biliary flow were alive during the observation period and, in casuistic II, 80.3% were alive after 7 years of follow-up. Conclusions Even though patients were admitted and treated earlier, it is clear that surgery could be done sooner. Delay in referral and timely propaedeutics were the main contributors.

Sign in / Sign up

Export Citation Format

Share Document