Child health
AKT · Child health/Neonatal & surgical
Biliary atresia
Progressive fibro-obliteration of bile ducts
Overview
Progressive obliteration of the extrahepatic biliary tree presenting as CONJUGATED neonatal jaundice with pale stools and dark urine. A surgical emergency — outcome depends on Kasai portoenterostomy before ~60 days.
Recognise
- Prolonged jaundice (>2 weeks) that is CONJUGATED
- PALE/clay stools and DARK urine
- Hepatomegaly, faltering growth
- Otherwise initially well baby
Red flags
- Any conjugated neonatal jaundice — refer urgently; the window for Kasai is narrow
Differentials & how to tell them apart
Physiological/breast-milk jaundiceUNCONJUGATED, normal stool/urine, thriving
Neonatal hepatitisconjugated too — distinguished on biopsy/HIDA
Choledochal cystcystic dilatation on USS
Alagille syndromepaucity of bile ducts + cardiac/vertebral/facial features
Investigations
Split bilirubin (conjugated raised), LFTs, USS (absent/abnormal gallbladder), HIDA scan, liver biopsy; intraoperative cholangiogram confirms.
Management
Kasai portoenterostomy (ideally before 60 days of life)
- 1Urgent referral. Kasai portoenterostomy, ideally before 60 days of age.Gate: Outcome falls sharply if Kasai is done after ~60–80 days → treat conjugated jaundice as an emergency
- 2Many ultimately need liver transplantation; nutritional + fat-soluble vitamin support.
Surgery (Kasai portoenterostomy) — definitive; before ~60 days for best outcome
Fat-soluble vitamin supplementation — ADEK; ursodeoxycholic acid
Key points
The single most important paediatric reason to check a SPLIT bilirubin in any prolonged jaundice. Pale stools are the red flag.
Monitor & prognosis
Growth, LFTs, signs of portal hypertension; transplant assessment.
Kasai restores drainage in many; a proportion still need transplant.
Source: NICE; BSPGHAN