2020 ICD-10-CM Diagnosis Code Q00.0

Anencephaly

    2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
  • Q00.0 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2020 edition of ICD-10-CM Q00.0 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of Q00.0 - other international versions of ICD-10 Q00.0 may differ.
Applicable To
  • Acephaly
  • Acrania
  • Amyelencephaly
  • Hemianencephaly
  • Hemicephaly
The following code(s) above Q00.0 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 Q00.0:
  • Q00-Q99
    2020 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
  • Amyelencephalus
  • Anencephalus
  • Congenital brain aplasia
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.
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.
  • Q00.0 is considered exempt from POA reporting.
ICD-10-CM Q00.0 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 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
  • 791 Prematurity with major problems
  • 793 Full term neonate with major problems

Convert Q00.0 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
  • 2020 (effective 10/1/2019): No change
Code annotations containing back-references to Q00.0:
  • Type 1 Excludes: Q75
    ICD-10-CM Diagnosis Code Q75

    Other congenital malformations of skull and face bones

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • congenital malformation of face NOS (Q18.-)
    • congenital malformation syndromes classified to Q87.-
    • dentofacial anomalies [including malocclusion] (M26.-)
    • musculoskeletal deformities of head and face (Q67.0-Q67.4)
    • skull defects associated with congenital anomalies of brain such as:
    • anencephaly (Q00.0)
    • encephalocele (Q01.-)
    • hydrocephalus (Q03.-)
    • microcephaly (Q02)

Diagnosis Index entries containing back-references to Q00.0:
  • Absence (of) (organ or part) (complete or partial)
    • brain Q00.0
    • skull bone (congenital) Q75.8
      ICD-10-CM Diagnosis Code Q75.8

      Other specified congenital malformations of skull and face bones

        2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
      Applicable To
      • Absence of skull bone, congenital
      • Congenital deformity of forehead
      • Platybasia
      • with
        • anencephaly Q00.0
  • Acephalia, acephalism, acephalus, acephaly Q00.0
  • Acrania Q00.0
  • Agenesis
    • brain Q00.0
    • skull (bone) Q75.8
      ICD-10-CM Diagnosis Code Q75.8

      Other specified congenital malformations of skull and face bones

        2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
      Applicable To
      • Absence of skull bone, congenital
      • Congenital deformity of forehead
      • Platybasia
      • with
        • anencephaly Q00.0
  • Amyelencephalus, amyelencephaly Q00.0
  • Anencephalus, anencephaly Q00.0
  • Anomaly, anomalous (congenital) (unspecified type) Q89.9
    ICD-10-CM Diagnosis Code Q89.9

    Congenital malformation, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
    Applicable To
    • Congenital anomaly NOS
    • Congenital deformity NOS
    • bone Q79.9
      ICD-10-CM Diagnosis Code Q79.9

      Congenital malformation of musculoskeletal system, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
      Applicable To
      • Congenital anomaly of musculoskeletal system NOS
      • Congenital deformity of musculoskeletal system NOS
      • skull Q75.9
        ICD-10-CM Diagnosis Code Q75.9

        Congenital malformation of skull and face bones, unspecified

          2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
        Applicable To
        • Congenital anomaly of face bones NOS
        • Congenital anomaly of skull NOS
        • with
          • anencephaly Q00.0
    • skull Q75.9
      ICD-10-CM Diagnosis Code Q75.9

      Congenital malformation of skull and face bones, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
      Applicable To
      • Congenital anomaly of face bones NOS
      • Congenital anomaly of skull NOS
      • with
        • anencephaly Q00.0
    • hemianencephaly Q00.0
    • hemicephaly Q00.0
    • hemicrania Q00.0
  • Aplasia - see also Agenesis
  • Deformity Q89.9
    ICD-10-CM Diagnosis Code Q89.9

    Congenital malformation, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
    Applicable To
    • Congenital anomaly NOS
    • Congenital deformity NOS
    • skull (acquired) M95.2
      ICD-10-CM Diagnosis Code M95.2

      Other acquired deformity of head

        2016 2017 2018 2019 2020 Billable/Specific Code
      • congenital Q75.8
        ICD-10-CM Diagnosis Code Q75.8

        Other specified congenital malformations of skull and face bones

          2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
        Applicable To
        • Absence of skull bone, congenital
        • Congenital deformity of forehead
        • Platybasia
        • with
          • anencephaly Q00.0
  • Distortion(s) (congenital)
    • skull bone(s) Q75.8
      ICD-10-CM Diagnosis Code Q75.8

      Other specified congenital malformations of skull and face bones

        2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
      Applicable To
      • Absence of skull bone, congenital
      • Congenital deformity of forehead
      • Platybasia
      • with
        • anencephalus Q00.0
  • Hemianencephaly Q00.0
  • Hemicephalus, hemicephaly Q00.0
  • Hemicrania
    • congenital malformation Q00.0
  • Hypoplasia, hypoplastic
    • skull (bone) Q75.8
      ICD-10-CM Diagnosis Code Q75.8

      Other specified congenital malformations of skull and face bones

        2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
      Applicable To
      • Absence of skull bone, congenital
      • Congenital deformity of forehead
      • Platybasia
      • with
        • anencephaly Q00.0
  • Imperfect
    • closure (congenital)
      • skull Q75.0
        ICD-10-CM Diagnosis Code Q75.0

        Craniosynostosis

          2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
        Applicable To
        • Acrocephaly
        • Imperfect fusion of skull
        • Oxycephaly
        • Trigonocephaly
        • with
          • anencephaly Q00.0
  • Monster, monstrosity (single) Q89.7
    ICD-10-CM Diagnosis Code Q89.7

    Multiple congenital malformations, not elsewhere classified

      2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
    Applicable To
    • Multiple congenital anomalies NOS
    • Multiple congenital deformities NOS
    Type 1 Excludes
    • congenital malformation syndromes affecting multiple systems (Q87.-)
    • acephalic Q00.0

ICD-10-CM Codes Adjacent To Q00.0
P96.2 Withdrawal symptoms from therapeutic use of drugs in newborn
P96.3 Wide cranial sutures of newborn
P96.5 Complication to newborn due to (fetal) intrauterine procedure
P96.8 Other specified conditions originating in the perinatal period
P96.81 Exposure to (parental) (environmental) tobacco smoke in the perinatal period
P96.82 Delayed separation of umbilical cord
P96.83 Meconium staining
P96.89 Other specified conditions originating in the perinatal period
P96.9 Condition originating in the perinatal period, unspecified
Q00 Anencephaly and similar malformations
Q00.0 Anencephaly
Q00.1 Craniorachischisis
Q00.2 Iniencephaly
Q01 Encephalocele
Q01.0 Frontal encephalocele
Q01.1 Nasofrontal encephalocele
Q01.2 Occipital encephalocele
Q01.8 Encephalocele of other sites
Q01.9 Encephalocele, unspecified
Q02 Microcephaly
Q03 Congenital hydrocephalus

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