MCC of neonatal jaundice requiring surgery

MC indication for liver TXP in children

Jaundice > 2 weeks after birth is suggestive of atresia (refractory to phototherapy)

• Acholic stools, palpable liver

Labs: elevated direct bili, GGT and LFT

Dx:

• 1st start with US → will not visualize a gallbladder
• HIDA (used to screen) if negative, rules out atresia – liver lights up by does not go into intestine = biliary atresia
  • Give phenobarbital as pretreatment prior to HIDA so liver excretes conjugated bili
  • r/o neonatal hepatitis (TORCH infections - Toxoplasmosis, Other infections, Rubella, Cytomegalovirus, Herpes) → Viral Hepatitis Panel
  • r/o other genetic causes: alpha-1-antitrypsin, cystic fibrosis
• Best test to confirm: Operative cholangiography. ALL patients need this to investigate biliary atresia
• liver biopsy (does not confirm) Periportal fibrosis, bile plugging → eventual cirrhosis
  • Percutaneous liver biopsy will differentiate between biliary atresia (Kasai) vs Alagille syndrome (c/i for Kasai procedure)

Tx:

If extra-hepatic ducts affected only Kasai procedure (Roux-en-y hepaticoportojejunostomy)

• Resect atretic extra hepatic bile duct segment to porta hepatis. Then anastomose jejunum to porta hepatis
• Need to perform before 3 months otherwise irreversible liver damage and need transplant
• Majority will still need Liver TXP or die from cirrhosis

If intra hepatic ducts involved (ductal hypoplasia) → Liver transplant

1/3 improve, 1/3 get TXP, 1/3 die