- 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, triangular cord sign = bile ductal remnant in porta hepatis, 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
- Clinical: 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
