Differential diagnosis of neonatal cholestasis

• Obstruction
  — Biliary atresia
  — Choledochal cyst / Caroli disease
  — Cholelithiasis
  — Bile plug syndrome
  — Progressive familial intrahepatic cholestasis
  — Alagille syndrome
• Infectious
  — Neonatal hepatitis
  — Sepsis
  — TORCH
• Metabolic
  — A1AT deficiency
  — Cystic fibrosis
  — Gaucher disease
  — Mitochondrial
• Miscellaneous
  — TPN
  — Thyroid
  — Shunts

Approach to the differential diagnosis of neonatal cholestasis:

• Begin with an ultrasound to look at the gallbladder and biliary tree and to assess for a choledochal cyst
• Consider hepatobiliary scintigraphy to distinguish between neonatal hepatitis and biliary atresia