Summer school: pediatric hepatologyBiliary atresia
Section snippets
Definition
Biliary atresia is characterized by an ascending inflammatory and destructive process of the biliary tree. Its onset is at birth and leads to obliterative scarring of the extrahepatic and later in the course intrahepatic bile ducts. Typically untreated patients succumb within the first year of life to complications of biliary cirrhosis and end-stage liver disease.
Etiology and pathogenesis
Current knowledge of the origins of biliary atresia is still limited. It is a heterogeneous entity with likely a variety of causes that share a final phenotype of inflammatory biliary disease of the neonate. A still unclear predisposition of the neonate in combination with parental factors (age, maternal diabetes, INVS, gene mutated mouse model), infectious triggers (predominant onset in winter months, Rota- or Reovirus induced animal models) and genetics (association with HLA B 12 or trisomy
Clinical symptoms
Children with biliary atresia may present with isolated jaundice as their only symptom. Apart from children with the syndromic form who may display clinical signs like polysplenia, the majority of children with biliary atresia have few suspicious clinical findings. They are healthy looking, thriving neonates with a pleasant aspect and no splenomegaly. Other than children with i.e. alpha-1-antitrypsin deficiency who are underweight and miserable following long-standing intrauterine illness,
Diagnostic approach
In any neonate with conjugated hyperbilirubinaemia (> 20% conjugated of total bilirubin) after the 14th day of life, biliary atresia has to be excluded by proving patency of the extrahepatic bile ducts. This can be achieved by endoscopic retrograde cholangiography, by hepatobiliary sequence scintigraphy or by direct visualization in diagnostic laparoscopy/-tomy. Indirect investigations such as liver histology or high-resolution magnetic resonance imaging can be useful for staging and grading of
Therapy
Definitive though usually palliative treatment for biliary atresia is a surgically reconstructed extrahepatic bile draining structure. The principles of Kasai hepatoportoenterostomy include resection of the choledochal remnants, gallbladder and portal plate. A jejunal Roux-en-Y anastomosis is created and the jejunal loop is brought to the porta hepatis. Finally, the portal region is connected to the loop to allow biliary drainage. The initial surgical approach was to anastomose ductal
Prognosis
Children with successful Kasai hepatoportoenterostomy often achieve good long-term survival of 75–90% at good quality of life. Some reports even document long-term survival of about 10% of these patients without liver disease, a finding that has not always been confirmed in large series. Patients who are diagnosed before day 30 of life often have aggressive disease and about 60% of these require transplantation in the first year of life. Children operated before day 60 achieve biliary drainage
Disclosure of interest
The authors declare that they have no conflicts of interest concerning this article.
References (0)
Cited by (31)
Biliary atresia in Vietnam: Management and the burden of disease
2017, Surgery (United States)Management of children with early onset of neonatal cholestasis: How can we do better?
2015, Journal de Pediatrie et de PuericulturePreoperative hepatic hemodynamics in the prediction of early portal vein thrombosis after liver transplantation in pediatric patients with biliary atresia
2015, Hepatobiliary and Pancreatic Diseases InternationalDiseases of the Pediatric Gallbladder and Biliary Tract
2014, Textbook of Gastrointestinal Radiology: Volumes 1-2, Fourth Edition