Inspissated bile syndrome

Inspissated bile syndrome, also known as bile plug syndrome, is a condition primarily affecting neonates where thick, viscous bile obstructs the intrahepatic or extrahepatic bile ducts. This obstruction leads to cholestasis, characterized by conjugated hyperbilirubinemia and jaundice.

This code is used for neonates presenting with signs of cholestasis and documented evidence of inspissated bile obstructing the biliary system. Documentation should clearly indicate the presence of thick bile plugs identified through imaging studies (e.g., ultrasound) or direct visualization during surgical exploration.

• Differentiate from other causes of neonatal cholestasis, such as biliary atresia (Q44.2) or neonatal hepatitis (P59.2).
• Ensure documentation specifies "inspissated bile" or "bile plug syndrome" to support this specific diagnosis.
• Consider sequencing this code after any underlying conditions contributing to the inspissated bile, if applicable.