A 5-week-old infant has persistent jaundice since the second week of life, acholic pale stools, and dark urine. Total bilirubin is 9 mg/dl with a direct fraction of 7 mg/dl. Ultrasound shows a contracted or absent gallbladder. What is the correct definitive surgical intervention and its optimal timing?

• A Cholecystojejunostomy performed after 6 months of age
• B Hepatoportoenterostomy (Kasai procedure), ideally before 60 days of life ✓
• C Primary liver transplantation as the first procedure at any age
• D Hepatoportoenterostomy, which gives equally good results whenever it is done

Explanation

The picture is biliary atresia: conjugated jaundice with acholic stools in a young infant. Definitive treatment is the Kasai hepatoportoenterostomy, and its success falls sharply with delay; drainage rates are best when the operation is done before about 60 days of age, and outcomes deteriorate markedly after 90 days. Cholecystojejunostomy fails because the entire extrahepatic duct system is obliterated. Transplantation is reserved for failed Kasai or late presentation.

Reference: Nelson Textbook of Pediatrics, 21st ed.

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Written and medically reviewed by the StethoPrep medical team.