biliary atresia Flashcards
define biliary atresia
paediatric condtion involving either the obliteration or discontinunity within the extrahepatic biliary system-> obstrcution of the flow of bile
what does biliary atresia cause
obstrtuction in flow of bile
results in neonatal presenation of cholestasisi for the first few weeks of life
aetiology of biliary atresia 4
pathogensis is unclear but:
-infectious agents
-congential malformations
-retained toxins within the bile
these are contirbuting factors
more common in females than males
types of biliary atresia and definition 3
type 1- proximal ducts are patent but the common duct is obliterated
type 2- atresia of cystic duct and cystic strcutres are found in the porta hepatis
type 3 atresia of the left and right ducts to the level of the porta hepatis - ACCOUNTS FOR 90% OF BILIARY ATRESIA
signs of biliary atresia 4
jaundice
HSM
abnormal growth
caridac murmurs if associted caridac abnormalites present
symptoms of biliary atresia 3
jaundice extending beyond 2 weeks
-dark urine -pale stools
appetite and growth disturbances (unreliable)
investigatoins for biliary atresia 6
both conjugated and total bilirubin levels
-total may be normal wherease conjugaeted bilirubin is abnormally high
LFTs- bile acid and aminiotransferesases aare raised
serum alpha1-antitryhsin - edficency may be the cause of neonatal cholestasis
sweat chloride test -cystic fibrosis may be the cause
US of biliary treee and liver
percutaneous liver biopsy with intraoperative cholagioscopy
managemnt of biliary atreisa 3
surgical intervention - only defintive treatment
- dissection of abnormalities into distinct ducts and anastomosis creation
medical intervention includes antibiotic coverage and bile acid enhances following surgery
complications of biliary atresia 3
unsuccessful anastomsis formation
progressive liver disease
cirrhosis w evenual hepatocelluaar carcinoma
prognosis of biliary atresia 2
good if surgery successful
if surgery fials - liver transplant within first two years of life
