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 2026, 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).
Caroli Disease
Congenital cystic dilatation of the intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC). It consists of 2 types: simple Caroli disease is characterized by bile duct dilatation (ectasia) alone; and complex Caroli disease is characterized by bile duct dilatation with extensive hepatic fibrosis and portal hypertension (HYPERTENSION, PORTAL). Benign renal tubular ectasia is associated with both types of Caroli disease.
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.
Cone Dystrophy
A general term which describes a group of rare eye disorders that affect the cone cells of the RETINA. Cone dystrophy can cause a variety of symptoms including decreased visual clarity or acuity when looking straight ahead (central vision), a reduced ability to see colors, and an increased sensitivity to light (PHOTOPHOBIA).
Jagged-1 Protein
A serrate-jagged protein that functions as a ligand for NOTCH RECEPTORS. It may regulate CELL DIFFERENTIATION in HEMATOPOIESIS and PHYSIOLOGIC ANGIOGENESIS. Mutations in the Jagged-1 gene are associated with ALAGILLE SYNDROME 1.
About the Q44 Code Range
The ICD-10 code section Q44 covers a range of congenital malformations affecting the gallbladder, bile ducts, and liver. These codes are specifically used to identify inherited or present-at-birth structural abnormalities of these digestive organs, including absence, underdevelopment, abnormal shape, or cystic conditions.
For instance, Q44.0 addresses agenesis, aplasia, and hypoplasia of the gallbladder, conditions also known as congenital absence or underdevelopment of the gallbladder. The code Q44.1 covers other unusual gallbladder malformations such as duplication or septation, while Q44.2 identifies various forms of bile duct atresia, a serious condition where bile ducts are blocked or absent at birth. Additional codes like Q44.3 and Q44.4 are used for congenital stenosis of bile ducts and choledochal cysts, respectively, which cause bile flow obstructions. Liver malformations, including cystic disease (Q44.6) and syndromes like Alagille syndrome (Q44.71), are also classified here. These distinct codes help medical coders accurately report and diagnose congenital defects related to liver and biliary system abnormalities, facilitating specialized patient care and research.
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, 2025 through September 30, 2026. 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 2026 ICD-10-CM Tabular List and order file, effective October 1, 2025 through September 30, 2026.
