2024 ICD-10-CM Diagnosis Code Q44.7

by

Other congenital malformations of liver

  • Q44.7 should not be used for reimbursement purposes as there are multiple codes below it that contain a greater level of detail.
  • ICD-10-CM Q44.7 is a new 2024 ICD-10-CM code that became effective on October 1, 2023.
  • This is the American ICD-10-CM version of Q44.7 – other international versions of ICD-10 Q44.7 may differ.

Code Also

  • , if applicable, associated malformations affecting other systems

The following code(s) above Q44.7 contain annotation back-references

 that may be applicable to Q44.7:

  • Q00-Q99  Congenital malformations, deformations and chromosomal abnormalities

Approximate Synonyms

  • Alagille syndrome
  • Arteriohepatic dysplasia
  • Congenital anomaly of liver
  • Congenital focal nodular hyperplasia of liver
  • Congenital focal nodular hyperplasia, liver
  • Congenital liver anomaly

Clinical Information

  • 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).
  • An autosomal dominant genetic syndrome caused by mutations in the jag1 gene. It is characterized by cholestatic jaundice in infancy, hepatosplenomegaly, distinctive facial features (prominent forehead, elongated nose, and pointed chin), cardiac murmurs, bone malformations, and sometimes mild mental retardation.
  • Autosomal dominant mutation involving chromosome 20; characterized by the almost normal liver that has few or no intrahepatic bile ducts; other extrahepatic malformations include those in the heart, the eyes, the vertebral column, and the facies; major clinical features include jaundice, and congenital heart disease with peripheral pulmonary stenosis.
  • Intrahepatic cholestasis caused by paucity of interlobular bile ducts associated with cardiovascular and other anomalies. Major abnormalities include characteristic facies (prominent forehead, deeply set eyes, hypertelorism, straight nose, and pointed chin) sometimes referred to as cholestasis facies, hoarse voice, and a wide variety of other disorders. Moderate mental retardation occurs in about 15 %. Cerebrovascular complications may include the moyamoya syndrome (progressive obliteration of the intracranial carotid arteries and formation of an extensive vascular network of dilated small branches. The disorder was first reported in japanese children, and the angiographic appearance of thus formed fine network was described by the japanese expression “moyamoya,” meaning “something hazy, like a puff of smoke drifting in the air,” as seen on the radiograph).

Code History

  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
  • 2021 (effective 10/1/2020): No change
  • 2022 (effective 10/1/2021): No change
  • 2023 (effective 10/1/2022): No change
  • 2024 (effective 10/1/2023): Deleted code
  • 2024 (effective 10/1/2023): New code