Congenital malformations of the nervous system (Q00-Q07)
chapter 17: Congenital Malformations, Deformations and Chromosomal Abnormalities
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Official coding guidelines
From the ICD-10-CM Official Guidelines for Coding and Reporting, FY 2027 (CMS and NCHS). Links open the PDF at the right page.
Assign Q00-QA1 codes for documented congenital anomalies or genetic disorders at any age, coding non-inherent manifestations separately and sequencing Z38 first on the birth admission.
All codes under Q00-Q07
Q00-Q07 can't be used on a claim. Pick a billable code below; grey headings group them.
- Q00Anencephaly and similar malformations
- Q00.0Anencephaly
- Q00.1Craniorachischisis
- Q00.2Iniencephaly
- Q01Encephalocele
- Q01.0Frontal encephalocele
- Q01.1Nasofrontal encephalocele
- Q01.2Occipital encephalocele
- Q01.8Encephalocele of other sites
- Q01.9Encephalocele, unspecified
- Q02Microcephaly
- Q03Congenital hydrocephalus
- Q03.0Malformations of aqueduct of Sylvius
- Q03.1Atresia of foramina of Magendie and Luschka
- Q03.8Other congenital hydrocephalus
- Q03.9Congenital hydrocephalus, unspecified
- Q04Other congenital malformations of brain
- Q04.0Congenital malformations of corpus callosum
- Q04.1Arhinencephaly
- Q04.2Holoprosencephaly
- Q04.3Other reduction deformities of brain
- Q04.4Septo-optic dysplasia of brain
- Q04.5Megalencephaly
- Q04.6Congenital cerebral cysts
- Q04.8Other specified congenital malformations of brain
- Q04.9Congenital malformation of brain, unspecified
- Q05Spina bifida
- Q05.0Cervical spina bifida with hydrocephalus
- Q05.1Thoracic spina bifida with hydrocephalus
- Q05.2Lumbar spina bifida with hydrocephalus
- Q05.3Sacral spina bifida with hydrocephalus
- Q05.4Unspecified spina bifida with hydrocephalus
- Q05.5Cervical spina bifida without hydrocephalus
- Q05.6Thoracic spina bifida without hydrocephalus
- Q05.7Lumbar spina bifida without hydrocephalus
- Q05.8Sacral spina bifida without hydrocephalus
- Q05.9Spina bifida, unspecified
- Q06Other congenital malformations of spinal cord
- Q06.0Amyelia
- Q06.1Hypoplasia and dysplasia of spinal cord
- Q06.2Diastematomyelia
- Q06.3Other congenital cauda equina malformations
- Q06.4Hydromyelia
- Q06.8Other specified congenital malformations of spinal cord
- Q06.9Congenital malformation of spinal cord, unspecified
- Q07Other congenital malformations of nervous system
- Q07.0Arnold-Chiari syndrome
- Q07.00Arnold-Chiari syndrome without spina bifida or hydrocephalus
- Q07.01Arnold-Chiari syndrome with spina bifida
- Q07.02Arnold-Chiari syndrome with hydrocephalus
- Q07.03Arnold-Chiari syndrome with spina bifida and hydrocephalus
- Q07.8Other specified congenital malformations of nervous system
- Q07.9Congenital malformation of nervous system, unspecified
Q00-Q07 MS-DRGs
This code does not group to an MS-DRG as a principal diagnosis.
About the condition
Plain-language background from MedlinePlus (National Library of Medicine), not coding guidance.
Neural Tube Defects
Neural tube defects are birth defects of the brain, spine, or spinal cord. They happen in the first month of pregnancy, often before a woman even knows that she is pregnant. The two most common neural tube defects are spina bifida and anencephaly. In spina bifida, the fetal spinal column doesn't close completely. There is usually nerve damage that causes at least some paralysis of the legs. In anencephaly, most of the brain and skull do not develop. Babies with anencephaly are usually either stillborn or die shortly after birth. Another type of defect, Chiari malformation, causes the brain tissue to extend into the spinal canal.
Read more about neural tube defects on MedlinePlus