🫀 Chapter 413: Cystic Diseases of the Biliary Tract and Liver

Nelson Textbook of Pediatrics 22nd Edition — Cystic diseases of the liver and biliary tract result from ductal plate malformation (ciliopathies). Key entities: Choledochal malformations (Todani types I-V, risk of cholangiocarcinoma), Caroli disease (intrahepatic ductal dilatation, recurrent cholangitis), Congenital hepatic fibrosis (preserved synthetic function, portal hypertension), ARPKD (PKHD1, congenital hepatic fibrosis + renal cysts), ADPKD (PKD1/PKD2, later onset).

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📋 30 Clinical Scenarios — Cystic Liver & Biliary Diseases

📇 High‑Yield Review Cards (Cystic Diseases)

🩺 Clinical Presentations: Cystic Liver & Biliary Diseases

Select a presentation for diagnostic clues and management.

📋 Stepwise Evaluation & Management of Cystic Liver Diseases

🔑 Key Principles — Cystic Diseases (Nelson Ch.413)
• Choledochal malformation: Most common type I (fusiform/saccular). Triad: abdominal pain, jaundice, mass (rarely all three). Malignancy risk (cholangiocarcinoma). Treatment: complete excision + Roux-en-Y hepaticojejunostomy.
• Caroli disease: Non-obstructive saccular dilation of intrahepatic bile ducts. Presents with recurrent cholangitis, cholelithiasis. Treatment: antibiotics, ursodiol, partial hepatectomy if localized, liver transplant for diffuse.
• Congenital hepatic fibrosis (CHF): Ductal plate malformation. Preserved synthetic function, portal hypertension (varices, splenomegaly). Treat complications of portal hypertension.
• ARPKD (PKHD1): Enlarged echogenic kidneys + CHF. Respiratory distress in neonates; portal hypertension later.
• ADPKD (PKD1/PKD2): Later onset, hepatic cysts rare in childhood.

    ⚡ Reflex Prompts — Cystic Liver Diseases Clinical Decisions

    📖 Cystic Diseases of Biliary Tract & Liver — Core Summary