A 6-week-old term neonate brought for persistent yellowish discoloration of eyes since day 10 has total bilirubin of 11 mg/dL with direct fraction of 6 mg/dL. Stools are pale clay coloured, urine stains the diaper dark yellow, and the liver is firm and palpable 4 cm below the costal margin. Ultrasound shows an absent or small gallbladder. What is the most appropriate next step?
- A Urgent evaluation for biliary atresia with HIDA scan and intraoperative cholangiography, aiming for Kasai portoenterostomy before 60 days of age ✓
- B Stop breastfeeding for 48 hours and observe
- C Phototherapy and reassessment after 2 weeks
- D Phenobarbital therapy for suspected Crigler-Najjar syndrome
Correct answer: A. Urgent evaluation for biliary atresia with HIDA scan and intraoperative cholangiography, aiming for Kasai portoenterostomy before 60 days of age
Explanation
Conjugated hyperbilirubinemia with acholic stools, dark urine and hepatomegaly defines obstructive cholestasis, and biliary atresia is the leading cause. Outcome depends critically on Kasai portoenterostomy performed before 60 days of life, so any infant with conjugated jaundice beyond 14 days needs urgent surgical evaluation. Phototherapy is ineffective for conjugated bilirubin, and Crigler-Najjar causes severe unconjugated jaundice without acholic stools.
Reference: Nelson Textbook of Pediatrics, 21st ed.
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