Congenital malformations of the nervous system (Q00-Q07)
Q00Anencephaly and similar malformations
Q00.0Anencephaly
› Acephaly
› Acrania
› Amyelencephaly
› Hemianencephaly
› Hemicephaly
Q00.1Craniorachischisis
Q00.2Iniencephaly
Q01Encephalocele
Includes: Arnold-Chiari syndrome, type III
› encephalocystocele
› encephalomyelocele
› hydroencephalocele
› hydromeningocele, cranial
› meningocele, cerebral
› meningoencephalocele
Excludes 1: Meckel-Gruber syndrome (Q61.9)
Q01.0Frontal encephalocele
Q01.1Nasofrontal encephalocele
Q01.2Occipital encephalocele
Q01.8Encephalocele of other sites
Q01.9Encephalocele, unspecified
Q02Microcephaly
Includes: hydromicrocephaly
› micrencephalon
Code First: , if applicable, congenital Zika virus disease
Excludes 1: Meckel-Gruber syndrome (Q61.9)
Q03Congenital hydrocephalus
Includes: hydrocephalus in newborn
Excludes 1: Arnold-Chiari syndrome, type II (Q07.0-)
› acquired hydrocephalus (G91.-)
› hydrocephalus due to congenital toxoplasmosis (P37.1)
› hydrocephalus with spina bifida (Q05.0-Q05.4)
Q03.0Malformations of aqueduct of Sylvius
› Anomaly of aqueduct of Sylvius
› Obstruction of aqueduct of Sylvius, congenital
› Stenosis of aqueduct of Sylvius
Q03.1Atresia of foramina of Magendie and Luschka
› Dandy-Walker syndrome
Q03.8Other congenital hydrocephalus
Q03.9Congenital hydrocephalus, unspecified
Q04Other congenital malformations of brain
Excludes 1: cyclopia (Q87.0)
› macrocephaly (Q75.3)
Q04.0Congenital malformations of corpus callosum
› Agenesis of corpus callosum
Q04.1Arhinencephaly
Q04.2Holoprosencephaly
Q04.3Other reduction deformities of brain
› Absence of part of brain
› Agenesis of part of brain
› Agyria
› Aplasia of part of brain
› Hydranencephaly
› Hypoplasia of part of brain
› Lissencephaly
› Microgyria
› Pachygyria
Excludes 1: congenital malformations of corpus callosum (Q04.0)
Q04.4Septo-optic dysplasia of brain
Q04.5Megalencephaly
Q04.6Congenital cerebral cysts
› Porencephaly
› Schizencephaly
Excludes 1: acquired porencephalic cyst (G93.0)
Q04.8Other specified congenital malformations of brain
› Arnold-Chiari syndrome, type IV
› Macrogyria
Q04.9Congenital malformation of brain, unspecified
› Congenital anomaly NOS of brain
› Congenital deformity NOS of brain
› Congenital disease or lesion NOS of brain
› Multiple anomalies NOS of brain, congenital
Q05Spina bifida
Includes: hydromeningocele (spinal)
› meningocele (spinal)
› meningomyelocele
› myelocele
› myelomeningocele
› rachischisis
› spina bifida (aperta)(cystica)
› syringomyelocele
Use Additional Code: code for any associated paraplegia (paraparesis) (G82.2-)
Excludes 1: Arnold-Chiari syndrome, type II (Q07.0-)
› spina bifida occulta (Q76.0)
Q05.0Cervical spina bifida with hydrocephalus
Q05.1Thoracic spina bifida with hydrocephalus
› Dorsal spina bifida with hydrocephalus
› Thoracolumbar spina bifida with hydrocephalus
Q05.2Lumbar spina bifida with hydrocephalus
› Lumbosacral 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
› Dorsal spina bifida NOS
› Thoracolumbar spina bifida NOS
Q05.7Lumbar spina bifida without hydrocephalus
› Lumbosacral spina bifida NOS
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
› Atelomyelia
› Myelatelia
› Myelodysplasia of spinal cord
Q06.2Diastematomyelia
Q06.3Other congenital cauda equina malformations
Q06.4Hydromyelia
› Hydrorachis
Q06.8Other specified congenital malformations of spinal cord
Q06.9Congenital malformation of spinal cord, unspecified
› Congenital anomaly NOS of spinal cord
› Congenital deformity NOS of spinal cord
› Congenital disease or lesion NOS of spinal cord
Q07Other congenital malformations of nervous system
Excludes 2: congenital central alveolar hypoventilation syndrome (G47.35)
› familial dysautonomia [Riley-Day] (G90.1)
› neurofibromatosis (nonmalignant) (Q85.0-)
Q07.0Arnold-Chiari syndrome
› Arnold-Chiari syndrome, type II
Excludes 1: Arnold-Chiari syndrome, type III (Q01.-)
› Arnold-Chiari syndrome, type IV (Q04.8)
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
› Agenesis of nerve
› Displacement of brachial plexus
› Jaw-winking syndrome
› Marcus Gunn's syndrome
Q07.9Congenital malformation of nervous system, unspecified
› Congenital anomaly NOS of nervous system
› Congenital deformity NOS of nervous system
› Congenital disease or lesion NOS of nervous system