2019 ICD-10-CM Diagnosis Code Q04.2

Holoprosencephaly

    2016 2017 2018 2019 Billable/Specific Code POA Exempt
  • Q04.2 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 Q04.2 became effective on October 1, 2018.
  • This is the American ICD-10-CM version of Q04.2 - other international versions of ICD-10 Q04.2 may differ.
The following code(s) above Q04.2 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 Q04.2:
  • 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
  • Q04
    ICD-10-CM Diagnosis Code Q04

    Other congenital malformations of brain

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 1 Excludes
    Other congenital malformations of brain
Approximate Synonyms
  • Holoprosencephaly sequence
Clinical Information
  • A rare disorder characterized by the failure of the forebrain to divide into distinct hemispheres. It is associated with craniofacial abnormalities, developmental abnormalities, and seizures.
  • Anterior midline brain, cranial, and facial malformations resulting from the failure of the embryonic prosencephalon to undergo segmentation and cleavage. Alobar prosencephaly is the most severe form and features anophthalmia; cyclopia; severe intellectual disability; cleft lip; cleft palate; seizures; and microcephaly. Semilobar holoprosencepaly is characterized by hypotelorism, microphthalmia, coloboma, nasal malformations, and variable degrees of intellectual disability. Lobar holoprosencephaly is associated with mild (or absent) facial malformations and intellectual abilities that range from mild intellectual disability to normal. Holoprosencephaly is associated with chromosome abnormalities.
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.
  • Q04.2 is considered exempt from POA reporting.
ICD-10-CM Q04.2 is grouped within Diagnostic Related Group(s) (MS-DRG v36.0):
  • 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 Q04.2 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 Q04.2:
  • Holoprosencephaly Q04.2

ICD-10-CM Codes Adjacent To Q04.2
Q01.9 Encephalocele, unspecified
Q02 Microcephaly
Q03 Congenital hydrocephalus
Q03.0 Malformations of aqueduct of Sylvius
Q03.1 Atresia of foramina of Magendie and Luschka
Q03.8 Other congenital hydrocephalus
Q03.9 Congenital hydrocephalus, unspecified
Q04 Other congenital malformations of brain
Q04.0 Congenital malformations of corpus callosum
Q04.1 Arhinencephaly
Q04.2 Holoprosencephaly
Q04.3 Other reduction deformities of brain
Q04.4 Septo-optic dysplasia of brain
Q04.5 Megalencephaly
Q04.6 Congenital cerebral cysts
Q04.8 Other specified congenital malformations of brain
Q04.9 Congenital malformation of brain, unspecified
Q05 Spina bifida
Q05.0 Cervical spina bifida with hydrocephalus
Q05.1 Thoracic spina bifida with hydrocephalus
Q05.2 Lumbar spina bifida with hydrocephalus

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