Q44.2

Billable

Atresia of bile ducts

HCC Category Mapping

V28HCC 68 — Cholangitis and Biliary Cirrhosis

0.000

What This Code Means

A birth defect where the bile ducts are completely closed or fail to develop, preventing bile from flowing to the intestines.

Coding Tips

•Atresia of bile ducts is a serious condition often requiring surgical intervention
•This code is distinct from stenosis (Q44.3) which involves narrowing rather than complete closure

Clinical Significance

Biliary atresia is a serious congenital condition requiring urgent surgical intervention (Kasai procedure) and often liver transplantation, making it one of the most common indications for pediatric liver transplant. Early recognition and treatment are crucial for survival, and these patients require lifelong specialized hepatology care with significant resource utilization.

Documentation Requirements

✓Confirmation of complete absence or closure of bile ducts
✓Type of biliary atresia (extrahepatic, intrahepatic, or mixed)
✓Liver biopsy findings showing bile duct absence
✓Associated complications (cirrhosis, portal hypertension)
✓Surgical interventions (Kasai portoenterostomy, liver transplant)
✓Current liver function status and laboratory values
✓Jaundice progression and bilirubin levels

Commonly Confused Codes

Q44.3 — Stenosis/stricture of bile ducts (narrowed, not completely absent)K83.1 — Obstruction of bile duct (acquired, not congenital)P59.29 — Neonatal jaundice (symptom, not underlying cause)K76.89 — Other specified diseases of liver (acquired liver disease)

Code Hierarchy

└Q44Congenital malformations of gallbladder, bile ducts and liver └Q44.2Atresia of bile ducts

└Q44.2Atresia of bile ducts