GI - Biliary Atresia Flashcards
Definition
progressive, idiopathic, fibro-obliterative disease of intra- and extra-hepatic biliary tree that causes biliary obstruction
Epidemiology
Female
Higher prevalence in East Asia + Pacific Islander populations
Pathophysiology
It is unclear exactly why biliary atresia occurs, however it is thought that one or more of immunologic, infectious and genetic factors contribute to initiation of epithelial damage and lead to subsequent sclerosis of the bile ducts.
Aetiology
CMV
Rotavirus
Two types of biliary atresia
- Perinatal (late-onset) form: the more common type, presenting several weeks after birth
- Embryonic (fetal, early-onset) form: less common (16% of cases) and often associated with other major birth defects
Signs
Hepatomegaly
Scleral icterus
Failure to thrive
Abdominal distension
Signs of portal hypertension (if severe)
Symptoms
Jaundice
Pale stools
Dark urine
Irritability
Diagnosis
FIRST LINE =
- LFTs = cholestatic picture = high conjugated bilirubin, GGT and ALP
- Abdo USS = absence or abnormality of the gallbladder and bile ducts
GOLD STANDARD = Intraoperative cholangiogram = narrow bile ducts
Management
FIRST LINE:
- Kasai procedure: the initial treatment is a surgical procedure (hepatoportoenterostomy), which involves removing the fibrotic bile ducts and creating a pathway for bile drainage using a loop of the small intestine.
- Ursoseoxycholic acid: given postoperatively to promote bile flow
SECOND LINE:
Liver transplant : This is often necessary if the Kasai procedure is unsuccessful or if cirrhosis develops. It is estimated that up to half of children will require this.
Complications
Cholangitis: this is a common postoperative complication
Cirrhosis and liver failure: despite treatment, many children with biliary atresia develop cirrhosis over time
Nutritional deficiencies: due to malabsorption of fat-soluble vitamins.
