Neonatal Cholestasis

Presentation

• Prolonged jaundice (persisting > 14 days in term infants or > 21 days in preterm)
• Acholic (pale or clay-colored) stools
• Dark urine standing out in diapers
• Hepatomegaly or splenomegaly on exam

Critical Initial Workup

• Fractionated bilirubin: Direct/Conjugated Bilirubin > 1.0 mg/dL (if TSB < 5) or > 20% of TSB (indicates cholestasis)
• Liver function enzymes: AST, ALT, Alkaline Phosphatase, GGT
• Coagulation profile (PT, aPTT, INR) - Vitamin K malabsorption is a critical risk
• Abdominal Ultrasound (fasting) to evaluate biliary anatomy, look for triangular cord sign or gallbladder absence (Biliary Atresia screening)

Further Evaluation & Management

• Biliary Atresia must be ruled out immediately (Time-sensitive: Kasai procedure best before 60 days of life)
• Workup for medical causes: Alpha-1 antitrypsin deficiency, Alagille syndrome, Galactosemia, TPN-associated cholestasis, CMV, Hypothyroidism
• Administer intramuscular Vitamin K to prevent hemorrhage if coagulopathic
• Provide fat-soluble vitamin supplementation (ADEK) and medium-chain triglyceride formula

Nutritional & GI Surgical Protocol

• NPO protocol: Replogle or Salem sump exactly at 10-14 cm mark hooked to low continuous suction (20-40 mmHg).
• TPN Initiation: Start amino acids at 2-3 g/kg/day, lipids at 1 g/kg/day within 24 hours of NPO status.
• X-Ray monitoring: Cross-table lateral and AP abdominal decubitus Q12h when concerned for perforation.
• Monitor daily abdominal girth measurements accurately marked by an indelible pen line at the umbilicus.
• Guaiac test all macroscopic bloody stools immediately.

Standardized Discharge & Follow-Up Criteria

• Adequate weight gain explicitly > 15-30g/day over a continuous 3-5 day period.
• Maintenance of temperature in an open crib with standard clothing for 48 hours.
• Successful completion of the 90-120 minute Infant Car Seat Challenge.
• Caregiver successfully completed CPR training, rooming-in, and independent medication administration.
• Referrals placed for High-Risk Infant Follow-Up Clinic, Early Intervention (PT/OT), and appropriate sub-specialists.

Diagnostic & Management Algorithm

• Neonatal Cholestasis Algorithm: [START] Direct Bili > 20% of Total -> [DECISION] Pale stools / hepatomegaly? -> [YES] Urgent Ultrasound to R/O Biliary Atresia -> [NO] Evaluate for TPN/Sepsis/Alpha-1 -> [ACTION] Start Ursodiol

References

1. Fawaz R, Baumann U, Ekong U, et al. Guideline for the Evaluation of Cholestatic Jaundice in Infants: Joint Recommendations of the NASPGHAN and ESPGHAN. J Pediatr Gastroenterol Nutr.
2. Feldman AG, Sokol RJ. Neonatal Cholestasis. Neoreviews.