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
		Acephaly Acrania Amyelencephaly Hemianencephaly Hemicephaly
Q00.1		Craniorachischisis
Q00.2		Iniencephaly


Q01	Encephalocele
	Includes:	encephalomyelocele hydroencephalocele hydromeningocele, cranial meningocele, cerebral meningoencephalocele
	Excludes:	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
	Hydromicrocephaly Micrencephalon
	Excludes:	Meckel-Gruber syndrome ( Q61.9 )


Q03	Congenital hydrocephalus
	Includes:	hydrocephalus in newborn
	Excludes:	Arnold-Chiari syndrome ( Q07.0 ) hydrocephalus: · acquired ( G91.- ) · due to congenital toxoplasmosis ( P37.1 ) · with spina bifida ( Q05.0-Q05.4 )
Q03.0	Malformations of aqueduct of Sylvius
	Aqueduct of Sylvius: · anomaly · obstruction, congenital · stenosis
Q03.1	Atresia of foramina of Magendie and Luschka
	Dandy-Walker syndrome
Q03.8	Other congenital hydrocephalus
Q03.9	Congenital hydrocephalus, unspecified


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


Q05	Spina bifida
	Includes:	hydromeningocele (spinal) meningocele (spinal) meningomyelocele myelocele myelomeningocele rachischisis spina bifida (aperta)(cystica) syringomyelocele
	Excludes:	Arnold-Chiari syndrome ( Q07.0 ) spina bifida occulta ( Q76.0 )
Q05.0	Cervical spina bifida with hydrocephalus
Q05.1	Thoracic spina bifida with hydrocephalus
	Spina bifida:
	· dorsal · thoracolumbar		} }	with hydrocephalus
Q05.2	Lumbar spina bifida with hydrocephalus
	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
	Spina bifida: · dorsal NOS · thoracolumbar NOS
Q05.7	Lumbar spina bifida without hydrocephalus
	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
	Atelomyelia Myelatelia Myelodysplasia of spinal cord
Q06.2	Diastematomyelia
Q06.3	Other congenital cauda equina malformations
Q06.4	Hydromyelia
	Hydrorachis
Q06.8	Other specified congenital malformations of spinal cord
Q06.9	Congenital malformation of spinal cord, unspecified
	Congenital:
	· anomaly · deformity · disease or lesion	} } }	NOS of spinal cord or meninges


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