1. ICD-10-CM Codes
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2. Q00-Q99
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3. Q38-Q45
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4. Q44-
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5. 2023 ICD-10-CM Diagnosis Code Q44.7

2023 ICD-10-CM Diagnosis Code Q44.7

Other congenital malformations of liver

2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code POA Exempt

• Q44.7 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
• The 2023 edition of ICD-10-CM Q44.7 became effective on October 1, 2022.
• This is the American ICD-10-CM version of Q44.7 - other international versions of ICD-10 Q44.7 may differ.

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

• 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).

• Q44.7 is considered exempt from POA reporting.

• 441 Disorders of liver except malignancy, cirrhosis or alcoholic hepatitis with mcc
• 442 Disorders of liver except malignancy, cirrhosis or alcoholic hepatitis with cc
• 443 Disorders of liver except malignancy, cirrhosis or alcoholic hepatitis without cc/mcc

Convert Q44.7 to ICD-9-CM

• 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

• Absence (of) (organ or part) (complete or partial)
  • liver Q44.7 (congenital)
• Accessory (congenital)
  • liver Q44.7
• Agenesis
  • hepatic Q44.7
  • liver Q44.7
• Alagille's syndrome Q44.7
• Anomaly, anomalous (congenital) (unspecified type) Q89.9
  ICD-10-CM Diagnosis Code Q89.9

  Congenital malformation, unspecified

  2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code POA Exempt

  Applicable To

  • Congenital anomaly NOS
  • Congenital deformity NOS
  • liver Q44.7
• Deformity Q89.9
  ICD-10-CM Diagnosis Code Q89.9

  Congenital malformation, unspecified

  2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code POA Exempt

  Applicable To

  • Congenital anomaly NOS
  • Congenital deformity NOS
  • liver (congenital) Q44.7
• Distortion(s) (congenital)
  • liver Q44.7
• Duplication, duplex - see also Accessory
  • liver Q44.7
• Hepatomegaly - see also Hypertrophy, liver
  • congenital Q44.7
• Hyperplasia, hyperplastic
  • liver (congenital) Q44.7
• Hypertrophy, hypertrophic
  • liver R16.0
    ICD-10-CM Diagnosis Code R16.0

    Hepatomegaly, not elsewhere classified

    2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code

    Applicable To

    • Hepatomegaly NOS
    • congenital Q44.7
• Hypoplasia, hypoplastic
  • liver Q44.7
• Lobulation (congenital) - see also Anomaly, by site
  • liver, abnormal Q44.7
• Malformation (congenital) - see also Anomaly
  • liver Q44.7
• Malposition
  • congenital
    • liver Q44.7
• Riedel's
  • lobe, liver Q44.7
• Syndrome - see also Disease
  • Alagille's Q44.7

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.