Specific code icon 2014 ICD-10-CM Diagnosis Code Q00.0

Anencephaly

  • Q00.0 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, Q00.0 and all other ICD-10-CM codes should only be used for training or planning purposes until then.
Clinical Information
  • A malformation of the nervous system caused by failure of the anterior neuropore to close. Infants are born with intact spinal cords, cerebellums, and brainstems, but lack formation of neural structures above this level. The skull is only partially formed but the eyes are usually normal. This condition may be associated with folate deficiency. Affected infants are only capable of primitive (brain stem) reflexes and usually do not survive for more than two weeks. (from menkes, textbook of child neurology, 5th ed, p247)
  • A rare neural tube defect during pregnancy, resulting in the absence of a large portion of the brain and skull in the fetus.
  • Congenital absence of the spinal cord and brain
  • Congenital malformation of the nervous system caused by failure of the anterior neuropore to close; infants are born with intact spinal cords, cerebellums, and brainstems, but lack formation of neural structures above this level; the skull is only partially formed but the eyes are usually normal; affected infants are only capable of primitive reflexes and usually do not survive for more than two weeks; complete absence of the brain is rare.
Applicable To
  • Acephaly
  • Acrania
  • Amyelencephaly
  • Hemianencephaly
  • Hemicephaly
Description Synonyms
  • Amyelencephalus
  • Anencephalus
  • Brain aplasia, congenital
  • Congenital brain aplasia
Present On Admission
  • Q00.0 is considered exempt from POA reporting.
Mortality Data
  • Between 1999-2007 there were 2,836 deaths in the United States where ICD-10 Q00.0 was indicated as the underlying cause of death [source: cdc.govlink to cdc.gov]
  • ICD-10 Q00.0 as underlying cause of death data broken down by: gender, age, race, year
ICD-10-CM Q00.0 is grouped within Diagnostic Related Group(s) (MS-DRG v30.0):
  • 091 Other disorders of nervous system with mcc
  • 092 Other disorders of nervous system with cc
  • 093 Other disorders of nervous system without cc/mcc
  • 793 Full term neonate with major problems
Convert ICD-10-CM Q00.0 to ICD-9-CM
The following ICD-10-CM Index entries contain back-references to ICD-10-CM Q00.0: