What is biliary atresia?
Biliary atresia is a rare condition that affects the liver and bile ducts in infants.
It is characterized by the abnormal narrowing or closure of the extrahepatic biliary tree, which can prevent bile from flowing properly from the liver to the intestines. This can lead to a buildup of bile in the liver, scarring, and eventually liver failure.
Biliary atresia can be difficult to diagnose and typically requires surgery(Kasai op.)to correct.
CLINICAL FINDINGS
– Neonatal jaundice with conjugated (direct) hyperbilirubinemia
; Affects 1 in 10,000-13,000 newborn infants
– Kasai portoenterostomy temporarily effective in 90% if performed < 2 months of age; ↓ to < 50% if > 3 months
– Liver transplant ultimately required in most by adulthood, even with prompt Kasai
; Due to gradual fibrosis and portal hypertension
IMAGING
– Ultrasonography
; Absent or abnormal small irregular gallbladder in vast majority (gallbladder ghost sign)
; Echogenic fibrous tissue (triangular cord sign) anterior to portal vein at site of obliterated extrahepatic biliary duct
; No biliary ductal dilatation
; Liver echotexture – typically normal
– Hepatobiliary scan (DISIDA scan)
; No radiotracer excretion into intestines ; gall bladder visible in up to 25%
– Intraoperative cholangiogram : No biliary enteric channel
Image from Diagnostic imaging, pediatrics
Image from Diagnostic imaging, pediatrics
