Congenital malform of gallbladder, bile ducts and liver (Q44)

• ICD-10 Index

  • Congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)

    • Other congenital malformations of the digestive system (Q38-Q45)

      • Congenital malform of gallbladder, bile ducts and liver (Q44)

        • Q44 - Congenital malform of gallbladder, bile ducts and liver NON-BILLABLE CODE
        • Q44.0 - Agenesis, aplasia and hypoplasia of gallbladder BILLABLE CODE
        • Q44.1 - Other congenital malformations of gallbladder BILLABLE CODE
        • Q44.2 - Atresia of bile ducts BILLABLE CODE
        • Q44.3 - Congenital stenosis and stricture of bile ducts BILLABLE CODE
        • Q44.4 - Choledochal cyst BILLABLE CODE
        • Q44.5 - Other congenital malformations of bile ducts BILLABLE CODE
        • Q44.6 - Cystic disease of liver BILLABLE CODE
        • Q44.7 - Other congenital malformations of liver BILLABLE CODE

Clinical Information for Congenital malform of gallbladder, bile ducts and liver (Q44)

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.

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.

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).

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).

Cholestasis - Impairment of bile flow due to obstruction in small bile ducts (INTRAHEPATIC CHOLESTASIS) or obstruction in large bile ducts (EXTRAHEPATIC CHOLESTASIS).

Cholestasis, Extrahepatic - Impairment of bile flow in the large BILE DUCTS by mechanical obstruction or stricture due to benign or malignant processes.

Cholestasis, Intrahepatic - Impairment of bile flow due to injury to the HEPATOCYTES; BILE CANALICULI; or the intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC).

Bile Ducts - The channels that collect and transport the bile secretion from the BILE CANALICULI, the smallest branch of the BILIARY TRACT in the LIVER, through the bile ductules, the bile ducts out the liver, and to the GALLBLADDER for storage.