2019 ICD-10-CM Diagnosis Code Q44.7

Other congenital malformations of liver

    2016 2017 2018 2019 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 2019 edition of ICD-10-CM Q44.7 became effective on October 1, 2018.
  • This is the American ICD-10-CM version of Q44.7 - other international versions of ICD-10 Q44.7 may differ.
Applicable To
  • Accessory liver
  • Alagille's syndrome
  • Congenital absence of liver
  • Congenital hepatomegaly
  • Congenital malformation of liver NOS
The following code(s) above Q44.7 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to Q44.7:
  • Q00-Q99
    2019 ICD-10-CM Range Q00-Q99

    Congenital malformations, deformations and chromosomal abnormalities

    Note
    • Codes from this chapter are not for use on maternal records
    Type 2 Excludes
    • inborn errors of metabolism (E70-E88)
    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).
Present On Admission
POA Help
"Present On Admission" is defined as present at the time the order for inpatient admission occurs — conditions that develop during an outpatient encounter, including emergency department, observation, or outpatient surgery, are considered POA.
  • Q44.7 is considered exempt from POA reporting.
ICD-10-CM Q44.7 is grouped within Diagnostic Related Group(s) (MS-DRG v36.0):
  • 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

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

Diagnosis Index entries containing back-references to Q44.7:

ICD-10-CM Codes Adjacent To Q44.7
Q43.8 Other specified congenital malformations of intestine
Q43.9 Congenital malformation of intestine, unspecified
Q44 Congenital malformations of gallbladder, bile ducts and liver
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 liver
Q45 Other congenital malformations of digestive system
Q45.0 Agenesis, aplasia and hypoplasia of pancreas
Q45.1 Annular pancreas
Q45.2 Congenital pancreatic cyst
Q45.3 Other congenital malformations of pancreas and pancreatic duct
Q45.8 Other specified congenital malformations of digestive system
Q45.9 Congenital malformation of digestive system, unspecified
Q50 Congenital malformations of ovaries, fallopian tubes and broad ligaments
Q50.0 Congenital absence of ovary
Q50.01 …… unilateral

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