- Etiology: two types:
— Perinatal (Acquired) is 80% of cases and is due to inflammation causing destruction of fully formed biliary ducts
— Embryonic (Fetal) is 20% of cases - Imaging: absence of biliary tree
- US: gallbladder is small to absent (<15-19 mm in length), triangular cord sign is obliterated fibrous ductal remnant with thickening near bifurcation of portal vein that is positive if > 3-4 mm but is difficult to identify, nonvisualization of common bile duct, hypertrophied hepatic artery
- HIDA scan: pretreat with phenobarbital for 1 week to optimize liver uptake, decreased uptake of radiotracer in liver, no excretion of radiotracer into biliary tree or bowel
- DDX: neonatal hepatitis
- Treatment: Kasai hepatic portoenterostomy – resect biliary remnant and creat Roux-en-Y intestinal anastomosis within 60 days to prevent liver fibrosis and failure
- Clinical: most common cause of neonatal cholestasis, clinical triad is jaundice / acholic stool / dark urine, Embryonic (Fetal) type associated with heterotaxy (50%) and thus polysplenia / asplenia and malrotation, preduodenal portal vein (60%), interrupted inferior vena cava (40%), cardiac anomalies (50%)
Radiology Cases of Biliary Atresia

