- 1/10,000 live births.
- Unclear aetiology.
- Presentation – usually early neonatal w jaundice.
- Management – portoenterostomy (Kasai’s operation): anastomosis of Roux limb of jejunum to tissue of hilum. Restoration of bile flow in 86% treated before 8 weeks but only 36% in older children.
- 60% required liver transplant