Overview

• Biliary Atresia
  • Congenital condition where a section of the bile duct is either narrowed or absent.
  • Presents shortly after birth with significant prolonged jaundice (>14 days) due to high conjugated bilirubin levels.
    • Jaundice will extend beyond the physiological two weeks.
    • Dark urine and pale stools.
    • Appetite and growth disturbance.
  • Ix → cholangiography (definitive diagnosis - will fail to show biliary tree) + percutaneous liver biopsy.
  • Mx → surgical intervention (kasai procedure - hepatoportoenterostomy) is only definitive treatment. Involves removing the dysfunctional biliary tree and creating a direct anastomosis between the small intestine and the liver to allow biliary outflow (ie. restores bile flow from the liver to the proximal small bowel).
    • Liver transplantation may be required.

Making Diagnosis

Clinical Features

Investigations

Management Plan

Complications →

Prognosis →