International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10)-WHO Version for ;2019-covid-expanded

Chapter XVII
Congenital malformations, deformations and chromosomal abnormalities
(Q00-Q99)

Congenital malformations of the nervous system
(Q00-Q07)

Q00 Anencephaly and similar malformations

Q00.0 Anencephaly
Incl.:
Acephaly
Acrania
Amyelencephaly
Hemianencephaly
Hemicephaly
Q00.1 Craniorachischisis
Q00.2 Iniencephaly

Q01 Encephalocele

Incl.:
encephalomyelocele
hydroencephalocele
hydromeningocele, cranial
meningocele, cerebral
meningoencephalocele
Excl.:
acquired encephalocele (G93.5)
Meckel-Gruber syndrome (Q61.9)
Q01.0 Frontal encephalocele
Q01.1 Nasofrontal encephalocele
Q01.2 Occipital encephalocele
Q01.8 Encephalocele of other sites
Q01.9 Encephalocele, unspecified

Q02 Microcephaly

Incl.:
Hydromicrocephaly
Micrencephalon
Excl.:
Meckel-Gruber syndrome (Q61.9)

Q03 Congenital hydrocephalus

Incl.:
hydrocephalus in newborn
Excl.:
Arnold-Chiari syndrome (Q07.0)
hydrocephalus:
Q03.0 Malformations of aqueduct of Sylvius
Incl.:
Aqueduct of Sylvius:
  • anomaly
  • obstruction, congenital
  • stenosis
Q03.1 Atresia of foramina of Magendie and Luschka
Incl.:
Dandy-Walker syndrome
Q03.8 Other congenital hydrocephalus
Q03.9 Congenital hydrocephalus, unspecified

Q04 Other congenital malformations of brain

Excl.:
cyclopia (Q87.0)
macrocephaly (Q75.3)
Q04.0 Congenital malformations of corpus callosum
Incl.:
Agenesis of corpus callosum
Q04.1 Arhinencephaly
Q04.2 Holoprosencephaly
Q04.3 Other reduction deformities of brain
Incl.:
  • Absence
  • Agenesis
  • Aplasia
  • Hypoplasia
  • of part of brain
Agyria
Hydranencephaly
Lissencephaly
Microgyria
Pachygyria
Excl.:
congenital malformations of corpus callosum (Q04.0)
Q04.4 Septo-optic dysplasia
Q04.5 Megalencephaly
Q04.6 Congenital cerebral cysts
Incl.:
Porencephaly
Schizencephaly
Excl.:
acquired porencephalic cyst (G93.0)
Q04.8 Other specified congenital malformations of brain
Incl.:
Macrogyria
Q04.9 Congenital malformation of brain, unspecified
Incl.:
  • Congenital:
    • anomaly
    • deformity
    • disease or lesion
    • multiple anomalies
  • NOS of brain

Q05 Spina bifida

Incl.:
hydromeningocele (spinal)
meningocele (spinal)
meningomyelocele
myelocele
myelomeningocele
rachischisis
spina bifida (aperta)(cystica)
syringomyelocele
Excl.:
Arnold-Chiari syndrome (Q07.0)
spina bifida occulta (Q76.0)
Q05.0 Cervical spina bifida with hydrocephalus
Q05.1 Thoracic spina bifida with hydrocephalus
Incl.:
  • Spina bifida:
    • dorsal
    • thoracolumbar
  • with hydrocephalus
Q05.2 Lumbar spina bifida with hydrocephalus
Incl.:
Lumbosacral spina bifida with hydrocephalus
Q05.3 Sacral spina bifida with hydrocephalus
Q05.4 Unspecified spina bifida with hydrocephalus
Q05.5 Cervical spina bifida without hydrocephalus
Q05.6 Thoracic spina bifida without hydrocephalus
Incl.:
Spina bifida:
  • dorsal NOS
  • thoracolumbar NOS
Q05.7 Lumbar spina bifida without hydrocephalus
Incl.:
Lumbosacral spina bifida NOS
Q05.8 Sacral spina bifida without hydrocephalus
Q05.9 Spina bifida, unspecified

Q06 Other congenital malformations of spinal cord

Q06.0 Amyelia
Q06.1 Hypoplasia and dysplasia of spinal cord
Incl.:
Atelomyelia
Myelatelia
Myelodysplasia of spinal cord
Q06.2 Diastematomyelia
Q06.3 Other congenital cauda equina malformations
Q06.4 Hydromyelia
Incl.:
Hydrorachis
Q06.8 Other specified congenital malformations of spinal cord
Q06.9 Congenital malformation of spinal cord, unspecified
Incl.:
  • Congenital:
    • anomaly
    • deformity
    • disease or lesion
  • NOS of spinal cord or meninges

Q07 Other congenital malformations of nervous system

Excl.:
familial dysautonomia [Riley-Day] (G90.1)
neurofibromatosis (nonmalignant) (Q85.0)
Q07.0 Arnold-Chiari syndrome
Q07.8 Other specified congenital malformations of nervous system
Incl.:
Agenesis of nerve
Displacement of brachial plexus
Jaw-winking syndrome
Marcus Gunn syndrome
Q07.9 Congenital malformation of nervous system, unspecified
Incl.:
  • Congenital:
    • anomaly
    • deformity
    • disease or lesion
  • NOS of nervous system