Congenital malformations of gallbladder, bile ducts and liver (Q44) ICD-10-CM
The Q44 code range covers congenital malformations of gallbladder, bile ducts and liver with 12 ICD-10-CM diagnosis codes. 10 of them are billable and valid for claim submission in fiscal year 2027, and the category headers group them but cannot themselves be billed.
Codes in the Q44 Range 12 codes · 10 billable
- Q44 Congenital malformations of gallbladder, bile ducts and liverNon-billable
- Q44.0 Agenesis, aplasia and hypoplasia of gallbladder
- Q44.1 Other congenital malformations of gallbladder
- Q44.2 Atresia of bile ducts
- Q44.3 Congenital stenosis and stricture of bile ducts
- Q44.4 Choledochal cyst
- Q44.5 Other congenital malformations of bile ducts
- Q44.6 Cystic disease of liver
- Q44.7 Other congenital malformations of liverNon-billable
- Q44.70 Other congenital malformation of liver, unspecified
- Q44.71 Alagille syndrome
- Q44.79 Other congenital malformations of liver
Clinical Terms in This Code Range
Definitions from the National Library of Medicine for conditions coded in the Q44 range.
Alagille Syndrome
A multisystem disorder that is characterized by aplasia of intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC), and malformations in the cardiovascular system, the eyes, the vertebral column, and the facies. Major clinical features include JAUNDICE, and congenital heart disease with peripheral PULMONARY STENOSIS. Alagille syndrome may result from heterogeneous gene mutations, including mutations in JAG1 on CHROMOSOME 20 (Type 1) and NOTCH2 on CHROMOSOME 1 (Type 2).
Choledochal Cyst
A congenital anatomic malformation of a bile duct, including cystic dilatation of the extrahepatic bile duct or the large intrahepatic bile duct. Classification is based on the site and type of dilatation. Type I is most common.
About the Q44 Code Range
These conditions are present at birth and affect the gallbladder, bile ducts, or liver.
Gallbladder codes distinguish an absent or underdeveloped gallbladder (Q44.0) from other malformations (Q44.1). Bile duct codes separate closed ducts (Q44.2), narrowed ducts (Q44.3), a bile duct cyst (Q44.4), and other malformations (Q44.5).
Liver codes distinguish cystic disease, involving cysts (Q44.6), from other malformations (Q44.7). The latter separates unspecified malformations (Q44.70), Alagille syndrome (Q44.71), and other liver malformations (Q44.79).
Questions About This Page
How many billable codes are in the Q44 range?
Of the 12 codes in this range, 10 are billable and valid for claim submission from October 1, 2026 through September 30, 2027. Category header codes group them but cannot be reported on claims.
What does the Q44 range classify?
The range classifies congenital malformations of gallbladder, bile ducts and liver. Each code links to its own reference page with billing status, MS-DRG grouping, coding notes, and clinical information.
Related References
Source: CMS FY 2027 ICD-10-CM Tabular List and order file, effective October 1, 2026 through September 30, 2027.
