Atresia of bile ducts

This code represents a congenital condition where the bile ducts, either inside or outside the liver, are abnormally narrow or completely absent. This malformation obstructs the flow of bile from the liver to the small intestine, leading to cholestasis and progressive liver damage. It is a serious condition typically diagnosed in infancy.

Use this code for documented cases of biliary atresia identified through imaging studies (e.g., ultrasound, cholangiogram) and/or liver biopsy. This diagnosis is often suspected in neonates presenting with persistent jaundice, pale stools, and dark urine. Documentation should clearly state "atresia of bile ducts" or "biliary atresia."

• Do not confuse with other congenital anomalies of the bile ducts, such as hypoplasia or cysts, which have different codes.
• Always sequence as the primary diagnosis when it is the reason for the encounter, especially for surgical interventions like a Kasai procedure.
• Look for documentation of associated conditions, such as congenital heart defects or polysplenia syndrome, and code them separately.