Specific code icon 2014 ICD-10-CM Diagnosis Code Q44.7

Other congenital malformations of liver

  • Q44.7 is a billable ICD-10-CM code that can be used to specify a diagnosis.
  • On October 1, 2015 ICD-10-CM will replace ICD-9-CM in the United States, therefore, Q44.7 and all other ICD-10-CM codes should only be used for training or planning purposes until then.
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).
Applicable To
  • Accessory liver
  • Alagille's syndrome
  • Congenital absence of liver
  • Congenital hepatomegaly
  • Congenital malformation of liver NOS
Description Synonyms
  • Congenital anomaly of liver
  • Congenital liver anomaly
Present On Admission
  • Q44.7 is considered exempt from POA reporting.
Mortality Data
  • Between 1999-2007 there were 88 deaths in the United States where ICD-10 Q44.7 was indicated as the underlying cause of death [source: cdc.govlink to cdc.gov]
  • ICD-10 Q44.7 as underlying cause of death data broken down by: gender, age, race, year
ICD-10-CM Q44.7 is grouped within Diagnostic Related Group(s) (MS-DRG v30.0):
  • 441 Disorders of liver except malig,cirr,alc hepa with mcc
  • 442 Disorders of liver except malig,cirr,alc hepa with cc
  • 443 Disorders of liver except malig,cirr,alc hepa without cc/mcc
Convert ICD-10-CM Q44.7 to ICD-9-CM
The following ICD-10-CM Index entries contain back-references to ICD-10-CM Q44.7: