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ICD-10-CM-Chapter 17
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Congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
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 +
Note
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Codes from this chapter are not for use on maternal records
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''Excludes2''
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inborn errors of metabolism (E70-E88)
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 +
This chapter contains the following blocks
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 +
*'''[[Q00-Q07]]'''
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 +
Congenital malformations of the nervous system
 +
 +
*'''[[Q10-Q18]]'''
 +
 +
Congenital malformations of eye, ear, face and neck
 +
 +
*'''[[Q20-Q28]]'''
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 +
Congenital malformations of the circulatory system
 +
 +
*'''[[Q30-Q34]]'''
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 +
Congenital malformations of the respiratory system
 +
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*'''[[Q35-Q37]]'''
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Cleft lip and cleft palate
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*'''[[Q38-Q45]]'''
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Other congenital malformations of the digestive system
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*'''[[Q50-Q56]]'''
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Congenital malformations of genital organs
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*'''[[Q60-Q64]]'''
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 +
Congenital malformations of the urinary system
 +
 +
*'''[[Q65-Q79]]'''
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 +
Congenital malformations and deformations of the musculoskeletal system
 +
 +
*'''[[Q80-Q89]]'''
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 +
Other congenital malformations
 +
 +
*'''[[Q90-Q99]]'''
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 +
Chromosomal abnormalities, not elsewhere classified
 +
 +
Congenital malformations of the nervous system (Q00-Q07)
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*'''[[Q00]]''' Anencephaly and similar malformations
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 +
*'''[[Q00.0]]''' Anencephaly
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Acephaly
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Acrania
 +
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Amyelencephaly
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Hemianencephaly
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Hemicephaly
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*'''[[Q00.1]]''' Craniorachischisis
 +
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*'''[[Q00.2]]''' Iniencephaly
 +
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*'''[[Q01]]''' Encephalocele
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''Includes''
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 +
Arnold-Chiari syndrome, type III
 +
 +
encephalocystocele
 +
 +
encephalomyelocele
 +
 +
hydroencephalocele
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hydromeningocele, cranial
 +
 +
meningocele, cerebral
 +
 +
meningoencephalocele
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''Excludes1''
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 +
Meckel-Gruber syndrome (Q61.9)
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 +
*'''[[Q01.0]]''' Frontal encephalocele
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 +
*'''[[Q01.1]]''' Nasofrontal encephalocele
 +
 +
*'''[[Q01.2]]''' Occipital encephalocele
 +
 +
*'''[[Q01.8]]''' Encephalocele of other sites
 +
 +
*'''[[Q01.9]]''' Encephalocele, unspecified
 +
 +
*'''[[Q02]]''' Microcephaly
 +
 +
''Includes''
 +
 +
hydromicrocephaly
 +
 +
micrencephalon ''Code first''
 +
 +
, if applicable, congenital Zika virus disease
 +
 +
''Excludes1''
 +
 +
Meckel-Gruber syndrome (Q61.9)
 +
 +
*'''[[Q03]]''' Congenital hydrocephalus
 +
 +
''Includes''
 +
 +
hydrocephalus in newborn
 +
 +
''Excludes1''
 +
 +
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)
 +
 +
*'''[[Q03.0]]''' Malformations of aqueduct of Sylvius
 +
 +
Anomaly of aqueduct of Sylvius
 +
 +
Obstruction of aqueduct of Sylvius, congenital
 +
 +
Stenosis of aqueduct of Sylvius
 +
 +
*'''[[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
 +
 +
''Excludes1''
 +
 +
cyclopia (Q87.0)
 +
 +
macrocephaly (Q75.3)
 +
 +
*'''[[Q04.0]]''' Congenital malformations of corpus callosum
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 +
Agenesis of corpus callosum
 +
 +
*'''[[Q04.1]]''' Arhinencephaly
 +
 +
*'''[[Q04.2]]''' Holoprosencephaly
 +
 +
*'''[[Q04.3]]''' Other reduction deformities of brain
 +
 +
Absence of part of brain
 +
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Agenesis of part of brain
 +
 +
Agyria
 +
 +
Aplasia of part of brain
 +
 +
Hydranencephaly
 +
 +
Hypoplasia of part of brain
 +
 +
Lissencephaly
 +
 +
Microgyria
 +
 +
Pachygyria
 +
 +
''Excludes1''
 +
 +
congenital malformations of corpus callosum (Q04.0)
 +
 +
*'''[[Q04.4]]''' Septo-optic dysplasia of brain
 +
 +
*'''[[Q04.5]]''' Megalencephaly
 +
 +
*'''[[Q04.6]]''' Congenital cerebral cysts
 +
 +
Porencephaly
 +
 +
Schizencephaly
 +
 +
''Excludes1''
 +
 +
acquired porencephalic cyst (G93.0)
 +
 +
*'''[[Q04.8]]''' Other specified congenital malformations of brain
 +
 +
Arnold-Chiari syndrome, type IV
 +
 +
Macrogyria
 +
 +
*'''[[Q04.9]]''' Congenital 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
 +
 +
*'''[[Q05]]''' Spina bifida
 +
 +
''Includes''
 +
 +
hydromeningocele (spinal)
 +
 +
meningocele (spinal)
 +
 +
meningomyelocele
 +
 +
myelocele
 +
 +
myelomeningocele
 +
 +
rachischisis
 +
 +
spina bifida (aperta)(cystica)
 +
 +
syringomyelocele ''Use additional'' code for any associated
 +
 +
paraplegia (paraparesis) (G82.2-)
 +
 +
''Excludes1''
 +
 +
Arnold-Chiari syndrome, type II (Q07.0-)
 +
 +
spina bifida occulta (Q76.0)
 +
 +
*'''[[Q05.0]]''' Cervical spina bifida with hydrocephalus
 +
 +
*'''[[Q05.1]]''' Thoracic spina bifida with hydrocephalus
 +
 +
Dorsal spina bifida with hydrocephalus
 +
 +
Thoracolumbar spina bifida with hydrocephalus
 +
 +
*'''[[Q05.2]]''' Lumbar spina bifida with hydrocephalus
 +
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Lumbosacral spina bifida with hydrocephalus
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*'''[[Q05.3]]''' Sacral spina bifida with hydrocephalus
 +
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*'''[[Q05.4]]''' Unspecified spina bifida with hydrocephalus
 +
 +
*'''[[Q05.5]]''' Cervical spina bifida without hydrocephalus
 +
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*'''[[Q05.6]]''' Thoracic spina bifida without hydrocephalus
 +
 +
Dorsal spina bifida NOS
 +
 +
Thoracolumbar spina bifida NOS
 +
 +
*'''[[Q05.7]]''' Lumbar spina bifida without hydrocephalus
 +
 +
Lumbosacral spina bifida NOS
 +
 +
*'''[[Q05.8]]''' Sacral spina bifida without hydrocephalus
 +
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*'''[[Q05.9]]''' Spina bifida, unspecified
 +
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*'''[[Q06]]''' Other congenital malformations of spinal cord
 +
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*'''[[Q06.0]]''' Amyelia
 +
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*'''[[Q06.1]]''' Hypoplasia and dysplasia of spinal cord
 +
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Atelomyelia
 +
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Myelatelia
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Myelodysplasia of spinal cord
 +
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*'''[[Q06.2]]''' Diastematomyelia
 +
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*'''[[Q06.3]]''' Other congenital cauda equina malformations
 +
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*'''[[Q06.4]]''' Hydromyelia
 +
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Hydrorachis
 +
 +
*'''[[Q06.8]]''' Other specified congenital malformations of spinal cord
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*'''[[Q06.9]]''' Congenital malformation of spinal cord, unspecified
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 +
Congenital anomaly NOS of spinal cord
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Congenital deformity NOS of spinal cord
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 +
Congenital disease or lesion NOS of spinal cord
 +
 +
*'''[[Q07]]''' Other congenital malformations of nervous system
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''Excludes2''
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 +
congenital central alveolar hypoventilation syndrome (G47.35)
 +
 +
familial dysautonomia (Riley-Day) (G90.1)
 +
 +
neurofibromatosis (nonmalignant) (Q85.0-)
 +
 +
*'''[[Q07.0]]''' Arnold-Chiari syndrome
 +
 +
Arnold-Chiari syndrome, type II
 +
 +
''Excludes1''
 +
 +
Arnold-Chiari syndrome, type III (Q01.-)
 +
 +
Arnold-Chiari syndrome, type IV (Q04.8)
 +
 +
*'''[[Q07.00]]''' Arnold-Chiari syndrome without spina bifida or hydrocephalus
 +
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*'''[[Q07.01]]''' Arnold-Chiari syndrome with spina bifida
 +
 +
*'''[[Q07.02]]''' Arnold-Chiari syndrome with hydrocephalus
 +
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*'''[[Q07.03]]''' Arnold-Chiari syndrome with spina bifida and hydrocephalus
 +
 +
*'''[[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
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 +
Congenital anomaly NOS of nervous system
 +
 +
Congenital deformity NOS of nervous system
 +
 +
Congenital disease or lesion NOS of nervous system
 +
 +
Congenital malformations of eye, ear, face and neck (Q10-Q18)
 +
 +
''Excludes2''
 +
 +
cleft lip and cleft palate (Q35-Q37)
 +
 +
congenital malformation of cervical spine (Q05.0, Q05.5, Q67.5, Q76.0-Q76.4)
 +
 +
congenital malformation of larynx (Q31.-)
 +
 +
congenital malformation of lip NEC (Q38.0)
 +
 +
congenital malformation of nose (Q30.-)
 +
 +
congenital malformation of parathyroid gland (Q89.2)
 +
 +
congenital malformation of thyroid gland (Q89.2)
 +
 +
*'''[[Q10]]''' Congenital malformations of eyelid, lacrimal apparatus and orbit
 +
 +
''Excludes1''
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 +
cryptophthalmos NOS (Q11.2)
 +
 +
cryptophthalmos syndrome (Q87.0)
 +
 +
*'''[[Q10.0]]''' Congenital ptosis
 +
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*'''[[Q10.1]]''' Congenital ectropion
 +
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*'''[[Q10.2]]''' Congenital entropion
 +
 +
*'''[[Q10.3]]''' Other congenital malformations of eyelid
 +
 +
Ablepharon
 +
 +
Blepharophimosis, congenital
 +
 +
Coloboma of eyelid
 +
 +
Congenital absence or agenesis of cilia
 +
 +
Congenital absence or agenesis of eyelid
 +
 +
Congenital accessory eyelid
 +
 +
Congenital accessory eye muscle
 +
 +
Congenital malformation of eyelid NOS
 +
 +
*'''[[Q10.4]]''' Absence and agenesis of lacrimal apparatus
 +
 +
Congenital absence of punctum lacrimale
 +
 +
*'''[[Q10.5]]''' Congenital stenosis and stricture of lacrimal duct
 +
 +
*'''[[Q10.6]]''' Other congenital malformations of lacrimal apparatus
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 +
Congenital malformation of lacrimal apparatus NOS
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 +
*'''[[Q10.7]]''' Congenital malformation of orbit
 +
 +
*'''[[Q11]]''' Anophthalmos, microphthalmos and macrophthalmos
 +
 +
*'''[[Q11.0]]''' Cystic eyeball
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 +
*'''[[Q11.1]]''' Other anophthalmos
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 +
Anophthalmos NOS
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 +
Agenesis of eye
 +
 +
Aplasia of eye
 +
 +
*'''[[Q11.2]]''' Microphthalmos
 +
 +
Cryptophthalmos NOS
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 +
Dysplasia of eye
 +
 +
Hypoplasia of eye
 +
 +
Rudimentary eye
 +
 +
''Excludes1''
 +
 +
cryptophthalmos syndrome (Q87.0)
 +
 +
*'''[[Q11.3]]''' Macrophthalmos
 +
 +
''Excludes1''
 +
 +
macrophthalmos in congenital glaucoma (Q15.0)
 +
 +
*'''[[Q12]]''' Congenital lens malformations
 +
 +
*'''[[Q12.0]]''' Congenital cataract
 +
 +
*'''[[Q12.1]]''' Congenital displaced lens
 +
 +
*'''[[Q12.2]]''' Coloboma of lens
 +
 +
*'''[[Q12.3]]''' Congenital aphakia
 +
 +
*'''[[Q12.4]]''' Spherophakia
 +
 +
*'''[[Q12.8]]''' Other congenital lens malformations
 +
 +
Microphakia
 +
 +
*'''[[Q12.9]]''' Congenital lens malformation, unspecified
 +
 +
*'''[[Q13]]''' Congenital malformations of anterior segment of eye
 +
 +
*'''[[Q13.0]]''' Coloboma of iris
 +
 +
Coloboma NOS
 +
 +
*'''[[Q13.1]]''' Absence of iris
 +
 +
Aniridia ''Use additional''
 +
 +
code for associated glaucoma (H42)
 +
 +
*'''[[Q13.2]]''' Other congenital malformations of iris
 +
 +
Anisocoria, congenital
 +
 +
Atresia of pupil
 +
 +
Congenital malformation of iris NOS
 +
 +
Corectopia
 +
 +
*'''[[Q13.3]]''' Congenital corneal opacity
 +
 +
*'''[[Q13.4]]''' Other congenital corneal malformations
 +
 +
Congenital malformation of cornea NOS
 +
 +
Microcornea
 +
 +
Peter's anomaly
 +
 +
*'''[[Q13.5]]''' Blue sclera
 +
 +
*'''[[Q13.8]]''' Other congenital malformations of anterior segment of eye
 +
 +
*'''[[Q13.81]]''' Rieger's anomaly ''Use additional''
 +
 +
code for associated glaucoma (H42)
 +
 +
*'''[[Q13.89]]''' Other congenital malformations of anterior segment of eye
 +
 +
*'''[[Q13.9]]''' Congenital malformation of anterior segment of eye, unspecified
 +
 +
*'''[[Q14]]''' Congenital malformations of posterior segment of eye
 +
 +
''Excludes2''
 +
 +
optic nerve hypoplasia (H47.03-)
 +
 +
*'''[[Q14.0]]''' Congenital malformation of vitreous humor
 +
 +
Congenital vitreous opacity
 +
 +
*'''[[Q14.1]]''' Congenital malformation of retina
 +
 +
Congenital retinal aneurysm
 +
 +
*'''[[Q14.2]]''' Congenital malformation of optic disc
 +
 +
Coloboma of optic disc
 +
 +
*'''[[Q14.3]]''' Congenital malformation of choroid
 +
 +
*'''[[Q14.8]]''' Other congenital malformations of posterior segment of eye
 +
 +
Coloboma of the fundus
 +
 +
*'''[[Q14.9]]''' Congenital malformation of posterior segment of eye, unspecified
 +
 +
*'''[[Q15]]''' Other congenital malformations of eye
 +
 +
''Excludes1''
 +
 +
congenital nystagmus (H55.01)
 +
 +
ocular albinism (E70.31-)
 +
 +
optic nerve hypoplasia (H47.03-)
 +
 +
retinitis pigmentosa (H35.52)
 +
 +
*'''[[Q15.0]]''' Congenital glaucoma
 +
 +
Axenfeld's anomaly
 +
 +
Buphthalmos
 +
 +
Glaucoma of childhood
 +
 +
Glaucoma of newborn
 +
 +
Hydrophthalmos
 +
 +
Keratoglobus, congenital, with glaucoma
 +
 +
Macrocornea with glaucoma
 +
 +
Macrophthalmos in congenital glaucoma
 +
 +
Megalocornea with glaucoma
 +
 +
*'''[[Q15.8]]''' Other specified congenital malformations of eye
 +
 +
*'''[[Q15.9]]''' Congenital malformation of eye, unspecified
 +
 +
Congenital anomaly of eye
 +
 +
Congenital deformity of eye
 +
 +
*'''[[Q16]]''' Congenital malformations of ear causing impairment of hearing
 +
 +
''Excludes1''
 +
 +
congenital deafness (H90.-)
 +
 +
*'''[[Q16.0]]''' Congenital absence of (ear) auricle
 +
 +
*'''[[Q16.1]]''' Congenital absence, atresia and stricture of auditory canal (external)
 +
 +
Congenital atresia or stricture of osseous meatus
 +
 +
*'''[[Q16.2]]''' Absence of eustachian tube
 +
 +
*'''[[Q16.3]]''' Congenital malformation of ear ossicles
 +
 +
Congenital fusion of ear ossicles
 +
 +
*'''[[Q16.4]]''' Other congenital malformations of middle ear
 +
 +
Congenital malformation of middle ear NOS
 +
 +
*'''[[Q16.5]]''' Congenital malformation of inner ear
 +
 +
Congenital anomaly of membranous labyrinth
 +
 +
Congenital anomaly of organ of Corti
 +
 +
*'''[[Q16.9]]''' Congenital malformation of ear causing impairment of hearing, unspecified
 +
 +
Congenital absence of ear NOS
 +
 +
*'''[[Q17]]''' Other congenital malformations of ear
 +
 +
''Excludes1''
 +
 +
congenital malformations of ear with impairment of hearing (Q16.0-Q16.9)
 +
 +
preauricular sinus (Q18.1)
 +
 +
*'''[[Q17.0]]''' Accessory auricle
 +
 +
Accessory tragus
 +
 +
Polyotia
 +
 +
Preauricular appendage or tag
 +
 +
Supernumerary ear
 +
 +
Supernumerary lobule
 +
 +
*'''[[Q17.1]]''' Macrotia
 +
 +
*'''[[Q17.2]]''' Microtia
 +
 +
*'''[[Q17.3]]''' Other misshapen ear
 +
 +
Pointed ear
 +
 +
*'''[[Q17.4]]''' Misplaced ear
 +
 +
Low-set ears
 +
 +
''Excludes1''
 +
 +
cervical auricle (Q18.2)
 +
 +
*'''[[Q17.5]]''' Prominent ear
 +
 +
Bat ear
 +
 +
*'''[[Q17.8]]''' Other specified congenital malformations of ear
 +
 +
Congenital absence of lobe of ear
 +
 +
*'''[[Q17.9]]''' Congenital malformation of ear, unspecified
 +
 +
Congenital anomaly of ear NOS
 +
 +
*'''[[Q18]]''' Other congenital malformations of face and neck
 +
 +
''Excludes1''
 +
 +
cleft lip and cleft palate (Q35-Q37)
 +
 +
conditions classified to Q67.0-Q67.4
 +
 +
congenital malformations of skull and face bones (Q75.-)
 +
 +
cyclopia (Q87.0)
 +
 +
dentofacial anomalies (including malocclusion) (M26.-)
 +
 +
malformation syndromes affecting facial appearance (Q87.0)
 +
 +
persistent thyroglossal duct (Q89.2)
 +
 +
*'''[[Q18.0]]''' Sinus, fistula and cyst of branchial cleft
 +
 +
Branchial vestige
 +
 +
*'''[[Q18.1]]''' Preauricular sinus and cyst
 +
 +
Fistula of auricle, congenital
 +
 +
Cervicoaural fistula
 +
 +
*'''[[Q18.2]]''' Other branchial cleft malformations
 +
 +
Branchial cleft malformation NOS
 +
 +
Cervical auricle
 +
 +
Otocephaly
 +
 +
*'''[[Q18.3]]''' Webbing of neck
 +
 +
Pterygium colli
 +
 +
*'''[[Q18.4]]''' Macrostomia
 +
 +
*'''[[Q18.5]]''' Microstomia
 +
 +
*'''[[Q18.6]]''' Macrocheilia
 +
 +
Hypertrophy of lip, congenital
 +
 +
*'''[[Q18.7]]''' Microcheilia
 +
 +
*'''[[Q18.8]]''' Other specified congenital malformations of face and neck
 +
 +
Medial cyst of face and neck
 +
 +
Medial fistula of face and neck
 +
 +
Medial sinus of face and neck
 +
 +
*'''[[Q18.9]]''' Congenital malformation of face and neck, unspecified
 +
 +
Congenital anomaly NOS of face and neck
 +
 +
Congenital malformations of the circulatory system (Q20-Q28)
 +
 +
*'''[[Q20]]''' Congenital malformations of cardiac chambers and connections
 +
 +
''Excludes1''
 +
 +
dextrocardia with situs inversus (Q89.3)
 +
 +
mirror-image atrial arrangement with situs inversus (Q89.3)
 +
 +
*'''[[Q20.0]]''' Common arterial trunk
 +
 +
Persistent truncus arteriosus
 +
 +
''Excludes1''
 +
 +
aortic septal defect (Q21.4)
 +
 +
*'''[[Q20.1]]''' Double outlet right ventricle
 +
 +
Taussig-Bing syndrome
 +
 +
*'''[[Q20.2]]''' Double outlet left ventricle
 +
 +
*'''[[Q20.3]]''' Discordant ventriculoarterial connection
 +
 +
Dextrotransposition of aorta
 +
 +
Transposition of great vessels (complete)
 +
 +
*'''[[Q20.4]]''' Double inlet ventricle
 +
 +
Common ventricle
 +
 +
Cor triloculare biatriatum
 +
 +
Single ventricle
 +
 +
*'''[[Q20.5]]''' Discordant atrioventricular connection
 +
 +
Corrected transposition
 +
 +
Levotransposition
 +
 +
Ventricular inversion
 +
 +
*'''[[Q20.6]]''' Isomerism of atrial appendages
 +
 +
Isomerism of atrial appendages with asplenia or polysplenia
 +
 +
*'''[[Q20.8]]''' Other congenital malformations of cardiac chambers and connections
 +
 +
Cor binoculare
 +
 +
*'''[[Q20.9]]''' Congenital malformation of cardiac chambers and connections, unspecified
 +
 +
*'''[[Q21]]''' Congenital malformations of cardiac septa
 +
 +
''Excludes1''
 +
 +
acquired cardiac septal defect (I51.0)
 +
 +
*'''[[Q21.0]]''' Ventricular septal defect
 +
 +
Roger's disease
 +
 +
*'''[[Q21.1]]''' Atrial septal defect
 +
 +
Coronary sinus defect
 +
 +
Patent or persistent foramen ovale
 +
 +
Patent or persistent ostium secundum defect (type II)
 +
 +
Patent or persistent sinus venosus defect
 +
 +
*'''[[Q21.2]]''' Atrioventricular septal defect
 +
 +
Common atrioventricular canal
 +
 +
Endocardial cushion defect
 +
 +
Ostium primum atrial septal defect (type I)
 +
 +
*'''[[Q21.3]]''' Tetralogy of Fallot
 +
 +
Ventricular septal defect with pulmonary stenosis or atresia, dextroposition of aorta and hypertrophy of right
 +
 +
ventricle
 +
 +
*'''[[Q21.4]]''' Aortopulmonary septal defect
 +
 +
Aortic septal defect
 +
 +
Aortopulmonary window
 +
 +
*'''[[Q21.8]]''' Other congenital malformations of cardiac septa
 +
 +
Eisenmenger's defect
 +
 +
Pentalogy of Fallot ''Code also''
 +
 +
if applicable
 +
 +
Eisenmenger's complex (I27.83)
 +
 +
Eisenmenger's syndrome (I27.83)
 +
 +
*'''[[Q21.9]]''' Congenital malformation of cardiac septum, unspecified
 +
 +
Septal (heart) defect NOS
 +
 +
*'''[[Q22]]''' Congenital malformations of pulmonary and tricuspid valves
 +
 +
*'''[[Q22.0]]''' Pulmonary valve atresia
 +
 +
*'''[[Q22.1]]''' Congenital pulmonary valve stenosis
 +
 +
*'''[[Q22.2]]''' Congenital pulmonary valve insufficiency
 +
 +
Congenital pulmonary valve regurgitation
 +
 +
*'''[[Q22.3]]''' Other congenital malformations of pulmonary valve
 +
 +
Congenital malformation of pulmonary valve NOS
 +
 +
Supernumerary cusps of pulmonary valve
 +
 +
*'''[[Q22.4]]''' Congenital tricuspid stenosis
 +
 +
Congenital tricuspid atresia
 +
 +
*'''[[Q22.5]]''' Ebstein's anomaly
 +
 +
*'''[[Q22.6]]''' Hypoplastic right heart syndrome
 +
 +
*'''[[Q22.8]]''' Other congenital malformations of tricuspid valve
 +
 +
*'''[[Q22.9]]''' Congenital malformation of tricuspid valve, unspecified
 +
 +
*'''[[Q23]]''' Congenital malformations of aortic and mitral valves
 +
 +
*'''[[Q23.0]]''' Congenital stenosis of aortic valve
 +
 +
Congenital aortic atresia
 +
 +
Congenital aortic stenosis NOS
 +
 +
''Excludes1''
 +
 +
congenital stenosis of aortic valve in hypoplastic left heart syndrome (Q23.4)
 +
 +
congenital subaortic stenosis (Q24.4)
 +
 +
supravalvular aortic stenosis (congenital) (Q25.3)
 +
 +
*'''[[Q23.1]]''' Congenital insufficiency of aortic valve
 +
 +
Bicuspid aortic valve
 +
 +
Congenital aortic insufficiency
 +
 +
*'''[[Q23.2]]''' Congenital mitral stenosis
 +
 +
Congenital mitral atresia
 +
 +
*'''[[Q23.3]]''' Congenital mitral insufficiency
 +
 +
*'''[[Q23.4]]''' Hypoplastic left heart syndrome
 +
 +
*'''[[Q23.8]]''' Other congenital malformations of aortic and mitral valves
 +
 +
*'''[[Q23.9]]''' Congenital malformation of aortic and mitral valves, unspecified
 +
 +
*'''[[Q24]]''' Other congenital malformations of heart
 +
 +
''Excludes1''
 +
 +
endocardial fibroelastosis (I42.4)
 +
 +
*'''[[Q24.0]]''' Dextrocardia
 +
 +
''Excludes1''
 +
 +
dextrocardia with situs inversus (Q89.3)
 +
 +
isomerism of atrial appendages (with asplenia or polysplenia) (Q20.6)
 +
 +
mirror-image atrial arrangement with situs inversus (Q89.3)
 +
 +
*'''[[Q24.1]]''' Levocardia
 +
 +
*'''[[Q24.2]]''' Cor triatriatum
 +
 +
*'''[[Q24.3]]''' Pulmonary infundibular stenosis
 +
 +
Subvalvular pulmonic stenosis
 +
 +
*'''[[Q24.4]]''' Congenital subaortic stenosis
 +
 +
*'''[[Q24.5]]''' Malformation of coronary vessels
 +
 +
Congenital coronary (artery) aneurysm
 +
 +
*'''[[Q24.6]]''' Congenital heart block
 +
 +
*'''[[Q24.8]]''' Other specified congenital malformations of heart
 +
 +
Congenital diverticulum of left ventricle
 +
 +
Congenital malformation of myocardium
 +
 +
Congenital malformation of pericardium
 +
 +
Malposition of heart
 +
 +
Uhl's disease
 +
 +
*'''[[Q24.9]]''' Congenital malformation of heart, unspecified
 +
 +
Congenital anomaly of heart
 +
 +
Congenital disease of heart
 +
 +
*'''[[Q25]]''' Congenital malformations of great arteries
 +
 +
*'''[[Q25.0]]''' Patent ductus arteriosus
 +
 +
Patent ductus Botallo
 +
 +
Persistent ductus arteriosus
 +
 +
*'''[[Q25.1]]''' Coarctation of aorta
 +
 +
Coarctation of aorta (preductal) (postductal)
 +
 +
Stenosis of aorta
 +
 +
*'''[[Q25.2]]''' Atresia of aorta
 +
 +
*'''[[Q25.21]]''' Interruption of aortic arch
 +
 +
Atresia of aortic arch
 +
 +
*'''[[Q25.29]]''' Other atresia of aorta
 +
 +
Atresia of aorta
 +
 +
*'''[[Q25.3]]''' Supravalvular aortic stenosis
 +
 +
''Excludes1''
 +
 +
congenital aortic stenosis NOS (Q23.0)
 +
 +
congenital stenosis of aortic valve (Q23.0)
 +
 +
*'''[[Q25.4]]''' Other congenital malformations of aorta
 +
 +
''Excludes1''
 +
 +
hypoplasia of aorta in hypoplastic left heart syndrome (Q23.4)
 +
 +
*'''[[Q25.40]]''' Congenital malformation of aorta unspecified
 +
 +
*'''[[Q25.41]]''' Absence and aplasia of aorta
 +
 +
*'''[[Q25.42]]''' Hypoplasia of aorta
 +
 +
*'''[[Q25.43]]''' Congenital aneurysm of aorta
 +
 +
Congenital aneurysm of aortic root
 +
 +
Congenital aneurysm of aortic sinus
 +
 +
*'''[[Q25.44]]''' Congenital dilation of aorta
 +
 +
*'''[[Q25.45]]''' Double aortic arch
 +
 +
Vascular ring of aorta
 +
 +
*'''[[Q25.46]]''' Tortuous aortic arch
 +
 +
Persistent convolutions of aortic arch
 +
 +
*'''[[Q25.47]]''' Right aortic arch
 +
 +
Persistent right aortic arch
 +
 +
*'''[[Q25.48]]''' Anomalous origin of subclavian artery
 +
 +
*'''[[Q25.49]]''' Other congenital malformations of aorta
 +
 +
Aortic arch
 +
 +
Bovine arch
 +
 +
*'''[[Q25.5]]''' Atresia of pulmonary artery
 +
 +
*'''[[Q25.6]]''' Stenosis of pulmonary artery
 +
 +
Supravalvular pulmonary stenosis
 +
 +
*'''[[Q25.7]]''' Other congenital malformations of pulmonary artery
 +
 +
*'''[[Q25.71]]''' Coarctation of pulmonary artery
 +
 +
*'''[[Q25.72]]''' Congenital pulmonary arteriovenous malformation
 +
 +
Congenital pulmonary arteriovenous aneurysm
 +
 +
*'''[[Q25.79]]''' Other congenital malformations of pulmonary artery
 +
 +
Aberrant pulmonary artery
 +
 +
Agenesis of pulmonary artery
 +
 +
Congenital aneurysm of pulmonary artery
 +
 +
Congenital anomaly of pulmonary artery
 +
 +
Hypoplasia of pulmonary artery
 +
 +
*'''[[Q25.8]]''' Other congenital malformations of other great arteries
 +
 +
*'''[[Q25.9]]''' Congenital malformation of great arteries, unspecified
 +
 +
*'''[[Q26]]''' Congenital malformations of great veins
 +
 +
*'''[[Q26.0]]''' Congenital stenosis of vena cava
 +
 +
Congenital stenosis of vena cava (inferior)(superior)
 +
 +
*'''[[Q26.1]]''' Persistent left superior vena cava
 +
 +
*'''[[Q26.2]]''' Total anomalous pulmonary venous connection
 +
 +
Total anomalous pulmonary venous return (TAPVR), subdiaphragmatic
 +
 +
Total anomalous pulmonary venous return (TAPVR), supradiaphragmatic
 +
 +
*'''[[Q26.3]]''' Partial anomalous pulmonary venous connection
 +
 +
Partial anomalous pulmonary venous return
 +
 +
*'''[[Q26.4]]''' Anomalous pulmonary venous connection, unspecified
 +
 +
*'''[[Q26.5]]''' Anomalous portal venous connection
 +
 +
*'''[[Q26.6]]''' Portal vein-hepatic artery fistula
 +
 +
*'''[[Q26.8]]''' Other congenital malformations of great veins
 +
 +
Absence of vena cava (inferior) (superior)
 +
 +
Azygos continuation of inferior vena cava
 +
 +
Persistent left posterior cardinal vein
 +
 +
Scimitar syndrome
 +
 +
*'''[[Q26.9]]''' Congenital malformation of great vein, unspecified
 +
 +
Congenital anomaly of vena cava (inferior) (superior) NOS
 +
 +
*'''[[Q27]]''' Other congenital malformations of peripheral vascular system
 +
 +
''Excludes2''
 +
 +
anomalies of cerebral and precerebral vessels (Q28.0-Q28.3)
 +
 +
anomalies of coronary vessels (Q24.5)
 +
 +
anomalies of pulmonary artery (Q25.5-Q25.7)
 +
 +
congenital retinal aneurysm (Q14.1)
 +
 +
hemangioma and lymphangioma (D18.-)
 +
 +
*'''[[Q27.0]]''' Congenital absence and hypoplasia of umbilical artery
 +
 +
Single umbilical artery
 +
 +
*'''[[Q27.1]]''' Congenital renal artery stenosis
 +
 +
*'''[[Q27.2]]''' Other congenital malformations of renal artery
 +
 +
Congenital malformation of renal artery NOS
 +
 +
Multiple renal arteries
 +
 +
*'''[[Q27.3]]''' Arteriovenous malformation (peripheral)
 +
 +
Arteriovenous aneurysm
 +
 +
''Excludes1''
 +
 +
acquired arteriovenous aneurysm (I77.0)
 +
 +
''Excludes2''
 +
 +
arteriovenous malformation of cerebral vessels (Q28.2)
 +
 +
arteriovenous malformation of precerebral vessels (Q28.0)
 +
 +
*'''[[Q27.30]]''' Arteriovenous malformation, site unspecified
 +
 +
*'''[[Q27.31]]''' Arteriovenous malformation of vessel of upper limb
 +
 +
*'''[[Q27.32]]''' Arteriovenous malformation of vessel of lower limb
 +
 +
*'''[[Q27.33]]''' Arteriovenous malformation of digestive system vessel
 +
 +
*'''[[Q27.34]]''' Arteriovenous malformation of renal vessel
 +
 +
*'''[[Q27.39]]''' Arteriovenous malformation, other site
 +
 +
*'''[[Q27.4]]''' Congenital phlebectasia
 +
 +
*'''[[Q27.8]]''' Other specified congenital malformations of peripheral vascular system
 +
 +
Absence of peripheral vascular system
 +
 +
Atresia of peripheral vascular system
 +
 +
Congenital aneurysm (peripheral)
 +
 +
Congenital stricture, artery
 +
 +
Congenital varix
 +
 +
''Excludes1''
 +
 +
arteriovenous malformation (Q27.3-)
 +
 +
*'''[[Q27.9]]''' Congenital malformation of peripheral vascular system, unspecified
 +
 +
Anomaly of artery or vein NOS
 +
 +
*'''[[Q28]]''' Other congenital malformations of circulatory system
 +
 +
''Excludes1''
 +
 +
congenital aneurysm NOS (Q27.8)
 +
 +
congenital coronary aneurysm (Q24.5)
 +
 +
ruptured cerebral arteriovenous malformation (I60.8)
 +
 +
ruptured malformation of precerebral vessels (I72.0)
 +
 +
''Excludes2''
 +
 +
congenital peripheral aneurysm (Q27.8)
 +
 +
congenital pulmonary aneurysm (Q25.79)
 +
 +
congenital retinal aneurysm (Q14.1)
 +
 +
*'''[[Q28.0]]''' Arteriovenous malformation of precerebral vessels
 +
 +
Congenital arteriovenous precerebral aneurysm (nonruptured)
 +
 +
*'''[[Q28.1]]''' Other malformations of precerebral vessels
 +
 +
Congenital malformation of precerebral vessels NOS
 +
 +
Congenital precerebral aneurysm (nonruptured)
 +
 +
*'''[[Q28.2]]''' Arteriovenous malformation of cerebral vessels
 +
 +
Arteriovenous malformation of brain NOS
 +
 +
Congenital arteriovenous cerebral aneurysm (nonruptured)
 +
 +
*'''[[Q28.3]]''' Other malformations of cerebral vessels
 +
 +
Congenital cerebral aneurysm (nonruptured)
 +
 +
Congenital malformation of cerebral vessels NOS
 +
 +
Developmental venous anomaly
 +
 +
*'''[[Q28.8]]''' Other specified congenital malformations of circulatory system
 +
 +
Congenital aneurysm, specified site NEC
 +
 +
Spinal vessel anomaly
 +
 +
*'''[[Q28.9]]''' Congenital malformation of circulatory system, unspecified
 +
 +
Congenital malformations of the respiratory system (Q30-Q34)
 +
 +
*'''[[Q30]]''' Congenital malformations of nose
 +
 +
''Excludes1''
 +
 +
congenital deviation of nasal septum (Q67.4)
 +
 +
*'''[[Q30.0]]''' Choanal atresia
 +
 +
Atresia of nares (anterior) (posterior)
 +
 +
Congenital stenosis of nares (anterior) (posterior)
 +
 +
*'''[[Q30.1]]''' Agenesis and underdevelopment of nose
 +
 +
Congenital absent of nose
 +
 +
*'''[[Q30.2]]''' Fissured, notched and cleft nose
 +
 +
*'''[[Q30.3]]''' Congenital perforated nasal septum
 +
 +
*'''[[Q30.8]]''' Other congenital malformations of nose
 +
 +
Accessory nose
 +
 +
Congenital anomaly of nasal sinus wall
 +
 +
*'''[[Q30.9]]''' Congenital malformation of nose, unspecified
 +
 +
*'''[[Q31]]''' Congenital malformations of larynx
 +
 +
''Excludes1''
 +
 +
congenital laryngeal stridor NOS (P28.89)
 +
 +
*'''[[Q31.0]]''' Web of larynx
 +
 +
Glottic web of larynx
 +
 +
Subglottic web of larynx
 +
 +
Web of larynx NOS
 +
 +
*'''[[Q31.1]]''' Congenital subglottic stenosis
 +
 +
*'''[[Q31.2]]''' Laryngeal hypoplasia
 +
 +
*'''[[Q31.3]]''' Laryngocele
 +
 +
*'''[[Q31.5]]''' Congenital laryngomalacia
 +
 +
*'''[[Q31.8]]''' Other congenital malformations of larynx
 +
 +
Absence of larynx
 +
 +
Agenesis of larynx
 +
 +
Atresia of larynx
 +
 +
Congenital cleft thyroid cartilage
 +
 +
Congenital fissure of epiglottis
 +
 +
Congenital stenosis of larynx NEC
 +
 +
Posterior cleft of cricoid cartilage
 +
 +
*'''[[Q31.9]]''' Congenital malformation of larynx, unspecified
 +
 +
*'''[[Q32]]''' Congenital malformations of trachea and bronchus
 +
 +
''Excludes1''
 +
 +
congenital bronchiectasis (Q33.4)
 +
 +
*'''[[Q32.0]]''' Congenital tracheomalacia
 +
 +
*'''[[Q32.1]]''' Other congenital malformations of trachea
 +
 +
Atresia of trachea
 +
 +
Congenital anomaly of tracheal cartilage
 +
 +
Congenital dilatation of trachea
 +
 +
Congenital malformation of trachea
 +
 +
Congenital stenosis of trachea
 +
 +
Congenital tracheocele
 +
 +
*'''[[Q32.2]]''' Congenital bronchomalacia
 +
 +
*'''[[Q32.3]]''' Congenital stenosis of bronchus
 +
 +
*'''[[Q32.4]]''' Other congenital malformations of bronchus
 +
 +
Absence of bronchus
 +
 +
Agenesis of bronchus
 +
 +
Atresia of bronchus
 +
 +
Congenital diverticulum of bronchus
 +
 +
Congenital malformation of bronchus NOS
 +
 +
*'''[[Q33]]''' Congenital malformations of lung
 +
 +
*'''[[Q33.0]]''' Congenital cystic lung
 +
 +
Congenital cystic lung disease
 +
 +
Congenital honeycomb lung
 +
 +
Congenital polycystic lung disease
 +
 +
''Excludes1''
 +
 +
cystic fibrosis (E84.0)
 +
 +
cystic lung disease, acquired or unspecified (J98.4)
 +
 +
*'''[[Q33.1]]''' Accessory lobe of lung
 +
 +
Azygos lobe (fissured), lung
 +
 +
*'''[[Q33.2]]''' Sequestration of lung
 +
 +
*'''[[Q33.3]]''' Agenesis of lung
 +
 +
Congenital absence of lung (lobe)
 +
 +
*'''[[Q33.4]]''' Congenital bronchiectasis
 +
 +
*'''[[Q33.5]]''' Ectopic tissue in lung
 +
 +
*'''[[Q33.6]]''' Congenital hypoplasia and dysplasia of lung
 +
 +
''Excludes1''
 +
 +
pulmonary hypoplasia associated with short gestation (P28.0)
 +
 +
*'''[[Q33.8]]''' Other congenital malformations of lung
 +
 +
*'''[[Q33.9]]''' Congenital malformation of lung, unspecified
 +
 +
*'''[[Q34]]''' Other congenital malformations of respiratory system
 +
 +
''Excludes2''
 +
 +
congenital central alveolar hypoventilation syndrome (G47.35)
 +
 +
*'''[[Q34.0]]''' Anomaly of pleura
 +
 +
*'''[[Q34.1]]''' Congenital cyst of mediastinum
 +
 +
*'''[[Q34.8]]''' Other specified congenital malformations of respiratory system
 +
 +
Atresia of nasopharynx
 +
 +
*'''[[Q34.9]]''' Congenital malformation of respiratory system, unspecified
 +
 +
Congenital absence of respiratory system
 +
 +
Congenital anomaly of respiratory system NOS
 +
 +
Cleft lip and cleft palate (Q35-Q37) ''Use additional''
 +
 +
code to identify associated malformation of the nose (Q30.2)
 +
 +
''Excludes1''
 +
 +
Robin's syndrome (Q87.0)
 +
 +
*'''[[Q35]]''' Cleft palate
 +
 +
''Includes''
 +
 +
fissure of palate
 +
 +
palatoschisis
 +
 +
''Excludes1''
 +
 +
cleft palate with cleft lip (Q37.-)
 +
 +
*'''[[Q35.1]]''' Cleft hard palate
 +
 +
*'''[[Q35.3]]''' Cleft soft palate
 +
 +
*'''[[Q35.5]]''' Cleft hard palate with cleft soft palate
 +
 +
*'''[[Q35.7]]''' Cleft uvula
 +
 +
*'''[[Q35.9]]''' Cleft palate, unspecified
 +
 +
Cleft palate NOS
 +
 +
*'''[[Q36]]''' Cleft lip
 +
 +
''Includes''
 +
 +
cheiloschisis
 +
 +
congenital fissure of lip
 +
 +
harelip
 +
 +
labium leporinum
 +
 +
''Excludes1''
 +
 +
cleft lip with cleft palate (Q37.-)
 +
 +
*'''[[Q36.0]]''' Cleft lip, bilateral
 +
 +
*'''[[Q36.1]]''' Cleft lip, median
 +
 +
*'''[[Q36.9]]''' Cleft lip, unilateral
 +
 +
Cleft lip NOS
 +
 +
*'''[[Q37]]''' Cleft palate with cleft lip
 +
 +
''Includes''
 +
 +
cheilopalatoschisis
 +
 +
*'''[[Q37.0]]''' Cleft hard palate with bilateral cleft lip
 +
 +
*'''[[Q37.1]]''' Cleft hard palate with unilateral cleft lip
 +
 +
Cleft hard palate with cleft lip NOS
 +
 +
*'''[[Q37.2]]''' Cleft soft palate with bilateral cleft lip
 +
 +
*'''[[Q37.3]]''' Cleft soft palate with unilateral cleft lip
 +
 +
Cleft soft palate with cleft lip NOS
 +
 +
*'''[[Q37.4]]''' Cleft hard and soft palate with bilateral cleft lip
 +
 +
*'''[[Q37.5]]''' Cleft hard and soft palate with unilateral cleft lip
 +
 +
Cleft hard and soft palate with cleft lip NOS
 +
 +
*'''[[Q37.8]]''' Unspecified cleft palate with bilateral cleft lip
 +
 +
*'''[[Q37.9]]''' Unspecified cleft palate with unilateral cleft lip
 +
 +
Cleft palate with cleft lip NOS
 +
 +
Other congenital malformations of the digestive system (Q38-Q45)
 +
 +
*'''[[Q38]]''' Other congenital malformations of tongue, mouth and pharynx
 +
 +
''Excludes1''
 +
 +
dentofacial anomalies (M26.-)
 +
 +
macrostomia (Q18.4)
 +
 +
microstomia (Q18.5)
 +
 +
*'''[[Q38.0]]''' Congenital malformations of lips, not elsewhere classified
 +
 +
Congenital fistula of lip
 +
 +
Congenital malformation of lip NOS
 +
 +
Van der Woude's syndrome
 +
 +
''Excludes1''
 +
 +
cleft lip (Q36.-)
 +
 +
cleft lip with cleft palate (Q37.-)
 +
 +
macrocheilia (Q18.6)
 +
 +
microcheilia (Q18.7)
 +
 +
*'''[[Q38.1]]''' Ankyloglossia
 +
 +
Tongue tie
 +
 +
*'''[[Q38.2]]''' Macroglossia
 +
 +
Congenital hypertrophy of tongue
 +
 +
*'''[[Q38.3]]''' Other congenital malformations of tongue
 +
 +
Aglossia
 +
 +
Bifid tongue
 +
 +
Congenital adhesion of tongue
 +
 +
Congenital fissure of tongue
 +
 +
Congenital malformation of tongue NOS
 +
 +
Double tongue
 +
 +
Hypoglossia
 +
 +
Hypoplasia of tongue
 +
 +
Microglossia
 +
 +
*'''[[Q38.4]]''' Congenital malformations of salivary glands and ducts
 +
 +
Atresia of salivary glands and ducts
 +
 +
Congenital absence of salivary glands and ducts
 +
 +
Congenital accessory salivary glands and ducts
 +
 +
Congenital fistula of salivary gland
 +
 +
*'''[[Q38.5]]''' Congenital malformations of palate, not elsewhere classified
 +
 +
Congenital absence of uvula
 +
 +
Congenital malformation of palate NOS
 +
 +
Congenital high arched palate
 +
 +
''Excludes1''
 +
 +
cleft palate (Q35.-)
 +
 +
cleft palate with cleft lip (Q37.-)
 +
 +
*'''[[Q38.6]]''' Other congenital malformations of mouth
 +
 +
Congenital malformation of mouth NOS
 +
 +
*'''[[Q38.7]]''' Congenital pharyngeal pouch
 +
 +
Congenital diverticulum of pharynx
 +
 +
''Excludes1''
 +
 +
pharyngeal pouch syndrome (D82.1)
 +
 +
*'''[[Q38.8]]''' Other congenital malformations of pharynx
 +
 +
Congenital malformation of pharynx NOS
 +
 +
Imperforate pharynx
 +
 +
*'''[[Q39]]''' Congenital malformations of esophagus
 +
 +
*'''[[Q39.0]]''' Atresia of esophagus without fistula
 +
 +
Atresia of esophagus NOS
 +
 +
*'''[[Q39.1]]''' Atresia of esophagus with tracheo-esophageal fistula
 +
 +
Atresia of esophagus with broncho-esophageal fistula
 +
 +
*'''[[Q39.2]]''' Congenital tracheo-esophageal fistula without atresia
 +
 +
Congenital tracheo-esophageal fistula NOS
 +
 +
*'''[[Q39.3]]''' Congenital stenosis and stricture of esophagus
 +
 +
*'''[[Q39.4]]''' Esophageal web
 +
 +
*'''[[Q39.5]]''' Congenital dilatation of esophagus
 +
 +
Congenital cardiospasm
 +
 +
*'''[[Q39.6]]''' Congenital diverticulum of esophagus
 +
 +
Congenital esophageal pouch
 +
 +
*'''[[Q39.8]]''' Other congenital malformations of esophagus
 +
 +
Congenital absence of esophagus
 +
 +
Congenital displacement of esophagus
 +
 +
Congenital duplication of esophagus
 +
 +
*'''[[Q39.9]]''' Congenital malformation of esophagus, unspecified
 +
 +
*'''[[Q40]]''' Other congenital malformations of upper alimentary tract
 +
 +
*'''[[Q40.0]]''' Congenital hypertrophic pyloric stenosis
 +
 +
Congenital or infantile constriction
 +
 +
Congenital or infantile hypertrophy
 +
 +
Congenital or infantile spasm
 +
 +
Congenital or infantile stenosis
 +
 +
Congenital or infantile stricture
 +
 +
*'''[[Q40.1]]''' Congenital hiatus hernia
 +
 +
Congenital displacement of cardia through esophageal hiatus
 +
 +
''Excludes1''
 +
 +
congenital diaphragmatic hernia (Q79.0)
 +
 +
*'''[[Q40.2]]''' Other specified congenital malformations of stomach
 +
 +
Congenital displacement of stomach
 +
 +
Congenital diverticulum of stomach
 +
 +
Congenital hourglass stomach
 +
 +
Congenital duplication of stomach
 +
 +
Megalogastria
 +
 +
Microgastria
 +
 +
*'''[[Q40.3]]''' Congenital malformation of stomach, unspecified
 +
 +
*'''[[Q40.8]]''' Other specified congenital malformations of upper alimentary tract
 +
 +
*'''[[Q40.9]]''' Congenital malformation of upper alimentary tract, unspecified
 +
 +
Congenital anomaly of upper alimentary tract
 +
 +
Congenital deformity of upper alimentary tract
 +
 +
*'''[[Q41]]''' Congenital absence, atresia and stenosis of small intestine
 +
 +
''Includes''
 +
 +
congenital obstruction, occlusion or stricture of small intestine or intestine NOS
 +
 +
''Excludes1''
 +
 +
cystic fibrosis with intestinal manifestation (E84.11)
 +
 +
meconium ileus NOS (without cystic fibrosis) (P76.0)
 +
 +
*'''[[Q41.0]]''' Congenital absence, atresia and stenosis of duodenum
 +
 +
*'''[[Q41.1]]''' Congenital absence, atresia and stenosis of jejunum
 +
 +
Apple peel syndrome
 +
 +
Imperforate jejunum
 +
 +
*'''[[Q41.2]]''' Congenital absence, atresia and stenosis of ileum
 +
 +
*'''[[Q41.8]]''' Congenital absence, atresia and stenosis of other specified parts of small intestine
 +
 +
*'''[[Q41.9]]''' Congenital absence, atresia and stenosis of small intestine, part unspecified
 +
 +
Congenital absence, atresia and stenosis of intestine NOS
 +
 +
*'''[[Q42]]''' Congenital absence, atresia and stenosis of large intestine
 +
 +
''Includes''
 +
 +
congenital obstruction, occlusion and stricture of large intestine
 +
 +
*'''[[Q42.0]]''' Congenital absence, atresia and stenosis of rectum with fistula
 +
 +
*'''[[Q42.1]]''' Congenital absence, atresia and stenosis of rectum without fistula
 +
 +
Imperforate rectum
 +
 +
*'''[[Q42.2]]''' Congenital absence, atresia and stenosis of anus with fistula
 +
 +
*'''[[Q42.3]]''' Congenital absence, atresia and stenosis of anus without fistula
 +
 +
Imperforate anus
 +
 +
*'''[[Q42.8]]''' Congenital absence, atresia and stenosis of other parts of large intestine
 +
 +
*'''[[Q42.9]]''' Congenital absence, atresia and stenosis of large intestine, part unspecified
 +
 +
*'''[[Q43]]''' Other congenital malformations of intestine
 +
 +
*'''[[Q43.0]]''' Meckel's diverticulum (displaced) (hypertrophic)
 +
 +
Persistent omphalomesenteric duct
 +
 +
Persistent vitelline duct
 +
 +
*'''[[Q43.1]]''' Hirschsprung's disease
 +
 +
Aganglionosis
 +
 +
Congenital (aganglionic) megacolon
 +
 +
*'''[[Q43.2]]''' Other congenital functional disorders of colon
 +
 +
Congenital dilatation of colon
 +
 +
*'''[[Q43.3]]''' Congenital malformations of intestinal fixation
 +
 +
Congenital omental, anomalous adhesions (bands)
 +
 +
Congenital peritoneal adhesions (bands)
 +
 +
Incomplete rotation of cecum and colon
 +
 +
Insufficient rotation of cecum and colon
 +
 +
Jackson's membrane
 +
 +
Malrotation of colon
 +
 +
Rotation failure of cecum and colon
 +
 +
Universal mesentery
 +
 +
*'''[[Q43.4]]''' Duplication of intestine
 +
 +
*'''[[Q43.5]]''' Ectopic anus
 +
 +
*'''[[Q43.6]]''' Congenital fistula of rectum and anus
 +
 +
''Excludes1''
 +
 +
congenital fistula of anus with absence, atresia and stenosis (Q42.2)
 +
 +
congenital fistula of rectum with absence, atresia and stenosis (Q42.0)
 +
 +
congenital rectovaginal fistula (Q52.2)
 +
 +
congenital urethrorectal fistula (Q64.73)
 +
 +
pilonidal fistula or sinus (L05.-)
 +
 +
*'''[[Q43.7]]''' Persistent cloaca
 +
 +
Cloaca NOS
 +
 +
*'''[[Q43.8]]''' Other specified congenital malformations of intestine
 +
 +
Congenital blind loop syndrome
 +
 +
Congenital diverticulitis, colon
 +
 +
Congenital diverticulum, intestine
 +
 +
Dolichocolon
 +
 +
Megaloappendix
 +
 +
Megaloduodenum
 +
 +
Microcolon
 +
 +
Transposition of appendix
 +
 +
Transposition of colon
 +
 +
Transposition of intestine
 +
 +
*'''[[Q43.9]]''' Congenital malformation of intestine, unspecified
 +
 +
*'''[[Q44]]''' Congenital malformations of gallbladder, bile ducts and liver
 +
 +
*'''[[Q44.0]]''' Agenesis, aplasia and hypoplasia of gallbladder
 +
 +
Congenital absence of gallbladder
 +
 +
*'''[[Q44.1]]''' Other congenital malformations of gallbladder
 +
 +
Congenital malformation of gallbladder NOS
 +
 +
Intrahepatic gallbladder
 +
 +
*'''[[Q44.2]]''' Atresia of bile ducts
 +
 +
*'''[[Q44.3]]''' Congenital stenosis and stricture of bile ducts
 +
 +
*'''[[Q44.4]]''' Choledochal cyst
 +
 +
*'''[[Q44.5]]''' Other congenital malformations of bile ducts
 +
 +
Accessory hepatic duct
 +
 +
Biliary duct duplication
 +
 +
Congenital malformation of bile duct NOS
 +
 +
Cystic duct duplication
 +
 +
*'''[[Q44.6]]''' Cystic disease of liver
 +
 +
Fibrocystic disease of liver
 +
 +
*'''[[Q44.7]]''' Other congenital malformations of liver
 +
 +
Accessory liver
 +
 +
Alagille's syndrome
 +
 +
Congenital absence of liver
 +
 +
Congenital hepatomegaly
 +
 +
Congenital malformation of liver NOS
 +
 +
*'''[[Q45]]''' Other congenital malformations of digestive system
 +
 +
''Excludes2''
 +
 +
congenital diaphragmatic hernia (Q79.0)
 +
 +
congenital hiatus hernia (Q40.1)
 +
 +
*'''[[Q45.0]]''' Agenesis, aplasia and hypoplasia of pancreas
 +
 +
Congenital absence of pancreas
 +
 +
*'''[[Q45.1]]''' Annular pancreas
 +
 +
*'''[[Q45.2]]''' Congenital pancreatic cyst
 +
 +
*'''[[Q45.3]]''' Other congenital malformations of pancreas and pancreatic duct
 +
 +
Accessory pancreas
 +
 +
Congenital malformation of pancreas or pancreatic duct NOS
 +
 +
''Excludes1''
 +
 +
congenital diabetes mellitus (E10.-)
 +
 +
cystic fibrosis (E84.0-E84.9)
 +
 +
fibrocystic disease of pancreas (E84.-)
 +
 +
neonatal diabetes mellitus (P70.2)
 +
 +
*'''[[Q45.8]]''' Other specified congenital malformations of digestive system
 +
 +
Absence (complete) (partial) of alimentary tract NOS
 +
 +
Duplication of digestive system
 +
 +
Malposition, congenital of digestive system
 +
 +
*'''[[Q45.9]]''' Congenital malformation of digestive system, unspecified
 +
 +
Congenital anomaly of digestive system
 +
 +
Congenital deformity of digestive system
 +
 +
Congenital malformations of genital organs (Q50-Q56)
 +
 +
''Excludes1''
 +
 +
androgen insensitivity syndrome (E34.5-)
 +
 +
syndromes associated with anomalies in the number and form of chromosomes (Q90-Q99)
 +
 +
*'''[[Q50]]''' Congenital malformations of ovaries, fallopian tubes and broad ligaments
 +
 +
*'''[[Q50.0]]''' Congenital absence of ovary
 +
 +
''Excludes1''
 +
 +
Turner's syndrome (Q96.-)
 +
 +
*'''[[Q50.01]]''' Congenital absence of ovary, unilateral
 +
 +
*'''[[Q50.02]]''' Congenital absence of ovary, bilateral
 +
 +
*'''[[Q50.1]]''' Developmental ovarian cyst
 +
 +
*'''[[Q50.2]]''' Congenital torsion of ovary
 +
 +
*'''[[Q50.3]]''' Other congenital malformations of ovary
 +
 +
*'''[[Q50.31]]''' Accessory ovary
 +
 +
*'''[[Q50.32]]''' Ovarian streak
 +
 +
46, XX with streak gonads
 +
 +
*'''[[Q50.39]]''' Other congenital malformation of ovary
 +
 +
Congenital malformation of ovary NOS
 +
 +
*'''[[Q50.4]]''' Embryonic cyst of fallopian tube
 +
 +
Fimbrial cyst
 +
 +
*'''[[Q50.5]]''' Embryonic cyst of broad ligament
 +
 +
Epoophoron cyst
 +
 +
Parovarian cyst
 +
 +
*'''[[Q50.6]]''' Other congenital malformations of fallopian tube and broad ligament
 +
 +
Absence of fallopian tube and broad ligament
 +
 +
Accessory fallopian tube and broad ligament
 +
 +
Atresia of fallopian tube and broad ligament
 +
 +
Congenital malformation of fallopian tube or broad ligament NOS
 +
 +
*'''[[Q51]]''' Congenital malformations of uterus and cervix
 +
 +
*'''[[Q51.0]]''' Agenesis and aplasia of uterus
 +
 +
Congenital absence of uterus
 +
 +
*'''[[Q51.1]]''' Doubling of uterus with doubling of cervix and vagina
 +
 +
*'''[[Q51.10]]''' Doubling of uterus with doubling of cervix and vagina without obstruction
 +
 +
Doubling of uterus with doubling of cervix and vagina NOS
 +
 +
*'''[[Q51.11]]''' Doubling of uterus with doubling of cervix and vagina with obstruction
 +
 +
*'''[[Q51.2]]''' Other doubling of uterus
 +
 +
Doubling of uterus NOS
 +
 +
Septate uterus
 +
 +
*'''[[Q51.20]]''' Other doubling of uterus, unspecified
 +
 +
Septate uterus, unspecified
 +
 +
*'''[[Q51.21]]''' Other complete doubling of uterus
 +
 +
Complete septate uterus
 +
 +
*'''[[Q51.22]]''' Other partial doubling of uterus
 +
 +
Partial septate uterus
 +
 +
*'''[[Q51.28]]''' Other doubling of uterus, other specified
 +
 +
Septate uterus, other specified
 +
 +
*'''[[Q51.3]]''' Bicornate uterus
 +
 +
Bicornate uterus, complete or partial
 +
 +
*'''[[Q51.4]]''' Unicornate uterus
 +
 +
Unicornate uterus with or without a separate uterine horn
 +
 +
Uterus with only one functioning horn
 +
 +
*'''[[Q51.5]]''' Agenesis and aplasia of cervix
 +
 +
Congenital absence of cervix
 +
 +
*'''[[Q51.6]]''' Embryonic cyst of cervix
 +
 +
*'''[[Q51.7]]''' Congenital fistulae between uterus and digestive and urinary tracts
 +
 +
*'''[[Q51.8]]''' Other congenital malformations of uterus and cervix
 +
 +
*'''[[Q51.81]]''' Other congenital malformations of uterus
 +
 +
*'''[[Q51.810]]''' Arcuate uterus
 +
 +
Arcuatus uterus
 +
 +
*'''[[Q51.811]]''' Hypoplasia of uterus
 +
 +
*'''[[Q51.818]]''' Other congenital malformations of uterus
 +
 +
Müllerian anomaly of uterus NEC
 +
 +
*'''[[Q51.82]]''' Other congenital malformations of cervix
 +
 +
*'''[[Q51.820]]''' Cervical duplication
 +
 +
*'''[[Q51.821]]''' Hypoplasia of cervix
 +
 +
*'''[[Q51.828]]''' Other congenital malformations of cervix
 +
 +
*'''[[Q51.9]]''' Congenital malformation of uterus and cervix, unspecified
 +
 +
*'''[[Q52]]''' Other congenital malformations of female genitalia
 +
 +
*'''[[Q52.0]]''' Congenital absence of vagina
 +
 +
Vaginal agenesis, total or partial
 +
 +
*'''[[Q52.1]]''' Doubling of vagina
 +
 +
''Excludes1''
 +
 +
doubling of vagina with doubling of uterus and cervix (Q51.1-)
 +
 +
*'''[[Q52.10]]''' Doubling of vagina, unspecified
 +
 +
Septate vagina NOS
 +
 +
*'''[[Q52.11]]''' Transverse vaginal septum
 +
 +
*'''[[Q52.12]]''' Longitudinal vaginal septum
 +
 +
*'''[[Q52.120]]''' Longitudinal vaginal septum, nonobstructing
 +
 +
*'''[[Q52.121]]''' Longitudinal vaginal septum, obstructing, right side
 +
 +
*'''[[Q52.122]]''' Longitudinal vaginal septum, obstructing, left side
 +
 +
*'''[[Q52.123]]''' Longitudinal vaginal septum, microperforate, right side
 +
 +
*'''[[Q52.124]]''' Longitudinal vaginal septum, microperforate, left side
 +
 +
*'''[[Q52.129]]''' Other and unspecified longitudinal vaginal septum
 +
 +
*'''[[Q52.2]]''' Congenital rectovaginal fistula
 +
 +
''Excludes1''
 +
 +
cloaca (Q43.7)
 +
 +
*'''[[Q52.3]]''' Imperforate hymen
 +
 +
*'''[[Q52.4]]''' Other congenital malformations of vagina
 +
 +
Canal of Nuck cyst, congenital
 +
 +
Congenital malformation of vagina NOS
 +
 +
Embryonic vaginal cyst
 +
 +
Gartner's duct cyst
 +
 +
*'''[[Q52.5]]''' Fusion of labia
 +
 +
*'''[[Q52.6]]''' Congenital malformation of clitoris
 +
 +
*'''[[Q52.7]]''' Other and unspecified congenital malformations of vulva
 +
 +
*'''[[Q52.70]]''' Unspecified congenital malformations of vulva
 +
 +
Congenital malformation of vulva NOS
 +
 +
*'''[[Q52.71]]''' Congenital absence of vulva
 +
 +
*'''[[Q52.79]]''' Other congenital malformations of vulva
 +
 +
Congenital cyst of vulva
 +
 +
*'''[[Q52.8]]''' Other specified congenital malformations of female genitalia
 +
 +
*'''[[Q52.9]]''' Congenital malformation of female genitalia, unspecified
 +
 +
*'''[[Q53]]''' Undescended and ectopic testicle
 +
 +
*'''[[Q53.0]]''' Ectopic testis
 +
 +
*'''[[Q53.00]]''' Ectopic testis, unspecified
 +
 +
*'''[[Q53.01]]''' Ectopic testis, unilateral
 +
 +
*'''[[Q53.02]]''' Ectopic testes, bilateral
 +
 +
*'''[[Q53.1]]''' Undescended testicle, unilateral
 +
 +
*'''[[Q53.10]]''' Unspecified undescended testicle, unilateral
 +
 +
*'''[[Q53.11]]''' Abdominal testis, unilateral
 +
 +
*'''[[Q53.111]]''' Unilateral intraabdominal testis
 +
 +
*'''[[Q53.112]]''' Unilateral inguinal testis
 +
 +
*'''[[Q53.12]]''' Ectopic perineal testis, unilateral
 +
 +
*'''[[Q53.13]]''' Unilateral high scrotal testis
 +
 +
*'''[[Q53.2]]''' Undescended testicle, bilateral
 +
 +
*'''[[Q53.20]]''' Undescended testicle, unspecified, bilateral
 +
 +
*'''[[Q53.21]]''' Abdominal testis, bilateral
 +
 +
*'''[[Q53.211]]''' Bilateral intraabdominal testes
 +
 +
*'''[[Q53.212]]''' Bilateral inguinal testes
 +
 +
*'''[[Q53.22]]''' Ectopic perineal testis, bilateral
 +
 +
*'''[[Q53.23]]''' Bilateral high scrotal testes
 +
 +
*'''[[Q53.9]]''' Undescended testicle, unspecified
 +
 +
Cryptorchism NOS
 +
 +
*'''[[Q54]]''' Hypospadias
 +
 +
''Excludes1''
 +
 +
epispadias (Q64.0)
 +
 +
*'''[[Q54.0]]''' Hypospadias, balanic
 +
 +
Hypospadias, coronal
 +
 +
Hypospadias, glandular
 +
 +
*'''[[Q54.1]]''' Hypospadias, penile
 +
 +
*'''[[Q54.2]]''' Hypospadias, penoscrotal
 +
 +
*'''[[Q54.3]]''' Hypospadias, perineal
 +
 +
*'''[[Q54.4]]''' Congenital chordee
 +
 +
Chordee without hypospadias
 +
 +
*'''[[Q54.8]]''' Other hypospadias
 +
 +
Hypospadias with intersex state
 +
 +
*'''[[Q54.9]]''' Hypospadias, unspecified
 +
 +
*'''[[Q55]]''' Other congenital malformations of male genital organs
 +
 +
''Excludes1''
 +
 +
congenital hydrocele (P83.5)
 +
 +
hypospadias (Q54.-)
 +
 +
*'''[[Q55.0]]''' Absence and aplasia of testis
 +
 +
Monorchism
 +
 +
*'''[[Q55.1]]''' Hypoplasia of testis and scrotum
 +
 +
Fusion of testes
 +
 +
*'''[[Q55.2]]''' Other and unspecified congenital malformations of testis and scrotum
 +
 +
*'''[[Q55.20]]''' Unspecified congenital malformations of testis and scrotum
 +
 +
Congenital malformation of testis or scrotum NOS
 +
 +
*'''[[Q55.21]]''' Polyorchism
 +
 +
*'''[[Q55.22]]''' Retractile testis
 +
 +
*'''[[Q55.23]]''' Scrotal transposition
 +
 +
*'''[[Q55.29]]''' Other congenital malformations of testis and scrotum
 +
 +
*'''[[Q55.3]]''' Atresia of vas deferens ''Code first''
 +
 +
any associated cystic fibrosis (E84.-)
 +
 +
*'''[[Q55.4]]''' Other congenital malformations of vas deferens, epididymis, seminal vesicles and prostate
 +
 +
Absence or aplasia of prostate
 +
 +
Absence or aplasia of spermatic cord
 +
 +
Congenital malformation of vas deferens, epididymis, seminal vesicles or prostate NOS
 +
 +
*'''[[Q55.5]]''' Congenital absence and aplasia of penis
 +
 +
*'''[[Q55.6]]''' Other congenital malformations of penis
 +
 +
*'''[[Q55.61]]''' Curvature of penis (lateral)
 +
 +
*'''[[Q55.62]]''' Hypoplasia of penis
 +
 +
Micropenis
 +
 +
*'''[[Q55.63]]''' Congenital torsion of penis
 +
 +
''Excludes1''
 +
 +
acquired torsion of penis (N48.82)
 +
 +
*'''[[Q55.64]]''' Hidden penis
 +
 +
Buried penis
 +
 +
Concealed penis
 +
 +
''Excludes1''
 +
 +
acquired buried penis (N48.83)
 +
 +
*'''[[Q55.69]]''' Other congenital malformation of penis
 +
 +
Congenital malformation of penis NOS
 +
 +
*'''[[Q55.7]]''' Congenital vasocutaneous fistula
 +
 +
*'''[[Q55.8]]''' Other specified congenital malformations of male genital organs
 +
 +
*'''[[Q55.9]]''' Congenital malformation of male genital organ, unspecified
 +
 +
Congenital anomaly of male genital organ
 +
 +
Congenital deformity of male genital organ
 +
 +
*'''[[Q56]]''' Indeterminate sex and pseudohermaphroditism
 +
 +
''Excludes1''
 +
 +
46,XX true hermaphrodite (Q99.1)
 +
 +
androgen insensitivity syndrome (E34.5-)
 +
 +
chimera 46,XX/46,XY true hermaphrodite (Q99.0)
 +
 +
female pseudohermaphroditism with adrenocortical disorder (E25.-)
 +
 +
pseudohermaphroditism with specified chromosomal anomaly (Q96-Q99)
 +
 +
pure gonadal dysgenesis (Q99.1)
 +
 +
*'''[[Q56.0]]''' Hermaphroditism, not elsewhere classified
 +
 +
Ovotestis
 +
 +
*'''[[Q56.1]]''' Male pseudohermaphroditism, not elsewhere classified
 +
 +
46, XY with streak gonads
 +
 +
Male pseudohermaphroditism NOS
 +
 +
*'''[[Q56.2]]''' Female pseudohermaphroditism, not elsewhere classified
 +
 +
Female pseudohermaphroditism NOS
 +
 +
*'''[[Q56.3]]''' Pseudohermaphroditism, unspecified
 +
 +
*'''[[Q56.4]]''' Indeterminate sex, unspecified
 +
 +
Ambiguous genitalia
 +
 +
Congenital malformations of the urinary system (Q60-Q64)
 +
 +
*'''[[Q60]]''' Renal agenesis and other reduction defects of kidney
 +
 +
''Includes''
 +
 +
congenital absence of kidney
 +
 +
congenital atrophy of kidney
 +
 +
infantile atrophy of kidney
 +
 +
*'''[[Q60.0]]''' Renal agenesis, unilateral
 +
 +
*'''[[Q60.1]]''' Renal agenesis, bilateral
 +
 +
*'''[[Q60.2]]''' Renal agenesis, unspecified
 +
 +
*'''[[Q60.3]]''' Renal hypoplasia, unilateral
 +
 +
*'''[[Q60.4]]''' Renal hypoplasia, bilateral
 +
 +
*'''[[Q60.5]]''' Renal hypoplasia, unspecified
 +
 +
*'''[[Q60.6]]''' Potter's syndrome
 +
 +
*'''[[Q61]]''' Cystic kidney disease
 +
 +
''Excludes1''
 +
 +
acquired cyst of kidney (N28.1)
 +
 +
Potter's syndrome (Q60.6)
 +
 +
*'''[[Q61.0]]''' Congenital renal cyst
 +
 +
*'''[[Q61.00]]''' Congenital renal cyst, unspecified
 +
 +
Cyst of kidney NOS (congenital)
 +
 +
*'''[[Q61.01]]''' Congenital single renal cyst
 +
 +
*'''[[Q61.02]]''' Congenital multiple renal cysts
 +
 +
*'''[[Q61.1]]''' Polycystic kidney, infantile type
 +
 +
Polycystic kidney, autosomal recessive
 +
 +
*'''[[Q61.11]]''' Cystic dilatation of collecting ducts
 +
 +
*'''[[Q61.19]]''' Other polycystic kidney, infantile type
 +
 +
*'''[[Q61.2]]''' Polycystic kidney, adult type
 +
 +
Polycystic kidney, autosomal dominant
 +
 +
*'''[[Q61.3]]''' Polycystic kidney, unspecified
 +
 +
*'''[[Q61.4]]''' Renal dysplasia
 +
 +
Multicystic dysplastic kidney
 +
 +
Multicystic kidney (development)
 +
 +
Multicystic kidney disease
 +
 +
Multicystic renal dysplasia
 +
 +
''Excludes1''
 +
 +
polycystic kidney disease (Q61.11-Q61.3)
 +
 +
*'''[[Q61.5]]''' Medullary cystic kidney
 +
 +
Nephronopthisis
 +
 +
Sponge kidney NOS
 +
 +
*'''[[Q61.8]]''' Other cystic kidney diseases
 +
 +
Fibrocystic kidney
 +
 +
Fibrocystic renal degeneration or disease
 +
 +
*'''[[Q61.9]]''' Cystic kidney disease, unspecified
 +
 +
Meckel-Gruber syndrome
 +
 +
*'''[[Q62]]''' Congenital obstructive defects of renal pelvis and congenital malformations of ureter
 +
 +
*'''[[Q62.0]]''' Congenital hydronephrosis
 +
 +
*'''[[Q62.1]]''' Congenital occlusion of ureter
 +
 +
Atresia and stenosis of ureter
 +
 +
*'''[[Q62.10]]''' Congenital occlusion of ureter, unspecified
 +
 +
*'''[[Q62.11]]''' Congenital occlusion of ureteropelvic junction
 +
 +
*'''[[Q62.12]]''' Congenital occlusion of ureterovesical orifice
 +
 +
*'''[[Q62.2]]''' Congenital megaureter
 +
 +
Congenital dilatation of ureter
 +
 +
*'''[[Q62.3]]''' Other obstructive defects of renal pelvis and ureter
 +
 +
*'''[[Q62.31]]''' Congenital ureterocele, orthotopic
 +
 +
*'''[[Q62.32]]''' Cecoureterocele
 +
 +
Ectopic ureterocele
 +
 +
*'''[[Q62.39]]''' Other obstructive defects of renal pelvis and ureter
 +
 +
Ureteropelvic junction obstruction NOS
 +
 +
*'''[[Q62.4]]''' Agenesis of ureter
 +
 +
Congenital absence ureter
 +
 +
*'''[[Q62.5]]''' Duplication of ureter
 +
 +
Accessory ureter
 +
 +
Double ureter
 +
 +
*'''[[Q62.6]]''' Malposition of ureter
 +
 +
*'''[[Q62.60]]''' Malposition of ureter, unspecified
 +
 +
*'''[[Q62.61]]''' Deviation of ureter
 +
 +
*'''[[Q62.62]]''' Displacement of ureter
 +
 +
*'''[[Q62.63]]''' Anomalous implantation of ureter
 +
 +
Ectopia of ureter
 +
 +
Ectopic ureter
 +
 +
*'''[[Q62.69]]''' Other malposition of ureter
 +
 +
*'''[[Q62.7]]''' Congenital vesico-uretero-renal reflux
 +
 +
*'''[[Q62.8]]''' Other congenital malformations of ureter
 +
 +
Anomaly of ureter NOS
 +
 +
*'''[[Q63]]''' Other congenital malformations of kidney
 +
 +
''Excludes1''
 +
 +
congenital nephrotic syndrome (N04.-)
 +
 +
*'''[[Q63.0]]''' Accessory kidney
 +
 +
*'''[[Q63.1]]''' Lobulated, fused and horseshoe kidney
 +
 +
*'''[[Q63.2]]''' Ectopic kidney
 +
 +
Congenital displaced kidney
 +
 +
Malrotation of kidney
 +
 +
*'''[[Q63.3]]''' Hyperplastic and giant kidney
 +
 +
Compensatory hypertrophy of kidney
 +
 +
*'''[[Q63.8]]''' Other specified congenital malformations of kidney
 +
 +
Congenital renal calculi
 +
 +
*'''[[Q63.9]]''' Congenital malformation of kidney, unspecified
 +
 +
*'''[[Q64]]''' Other congenital malformations of urinary system
 +
 +
*'''[[Q64.0]]''' Epispadias
 +
 +
''Excludes1''
 +
 +
hypospadias (Q54.-)
 +
 +
*'''[[Q64.1]]''' Exstrophy of urinary bladder
 +
 +
*'''[[Q64.10]]''' Exstrophy of urinary bladder, unspecified
 +
 +
Ectopia vesicae
 +
 +
*'''[[Q64.11]]''' Supravesical fissure of urinary bladder
 +
 +
*'''[[Q64.12]]''' Cloacal exstrophy of urinary bladder
 +
 +
*'''[[Q64.19]]''' Other exstrophy of urinary bladder
 +
 +
Extroversion of bladder
 +
 +
*'''[[Q64.2]]''' Congenital posterior urethral valves
 +
 +
*'''[[Q64.3]]''' Other atresia and stenosis of urethra and bladder neck
 +
 +
*'''[[Q64.31]]''' Congenital bladder neck obstruction
 +
 +
Congenital obstruction of vesicourethral orifice
 +
 +
*'''[[Q64.32]]''' Congenital stricture of urethra
 +
 +
*'''[[Q64.33]]''' Congenital stricture of urinary meatus
 +
 +
*'''[[Q64.39]]''' Other atresia and stenosis of urethra and bladder neck
 +
 +
Atresia and stenosis of urethra and bladder neck NOS
 +
 +
*'''[[Q64.4]]''' Malformation of urachus
 +
 +
Cyst of urachus
 +
 +
Patent urachus
 +
 +
Prolapse of urachus
 +
 +
*'''[[Q64.5]]''' Congenital absence of bladder and urethra
 +
 +
*'''[[Q64.6]]''' Congenital diverticulum of bladder
 +
 +
*'''[[Q64.7]]''' Other and unspecified congenital malformations of bladder and urethra
 +
 +
''Excludes1''
 +
 +
congenital prolapse of bladder (mucosa) (Q79.4)
 +
 +
*'''[[Q64.70]]''' Unspecified congenital malformation of bladder and urethra
 +
 +
Malformation of bladder or urethra NOS
 +
 +
*'''[[Q64.71]]''' Congenital prolapse of urethra
 +
 +
*'''[[Q64.72]]''' Congenital prolapse of urinary meatus
 +
 +
*'''[[Q64.73]]''' Congenital urethrorectal fistula
 +
 +
*'''[[Q64.74]]''' Double urethra
 +
 +
*'''[[Q64.75]]''' Double urinary meatus
 +
 +
*'''[[Q64.79]]''' Other congenital malformations of bladder and urethra
 +
 +
*'''[[Q64.8]]''' Other specified congenital malformations of urinary system
 +
 +
*'''[[Q64.9]]''' Congenital malformation of urinary system, unspecified
 +
 +
Congenital anomaly NOS of urinary system
 +
 +
Congenital deformity NOS of urinary system
 +
 +
Congenital malformations and deformations of the musculoskeletal system (Q65-Q79)
 +
 +
*'''[[Q65]]''' Congenital deformities of hip
 +
 +
''Excludes1''
 +
 +
clicking hip (R29.4)
 +
 +
*'''[[Q65.0]]''' Congenital dislocation of hip, unilateral
 +
 +
*'''[[Q65.00]]''' Congenital dislocation of unspecified hip, unilateral
 +
 +
*'''[[Q65.01]]''' Congenital dislocation of right hip, unilateral
 +
 +
*'''[[Q65.02]]''' Congenital dislocation of left hip, unilateral
 +
 +
*'''[[Q65.1]]''' Congenital dislocation of hip, bilateral
 +
 +
*'''[[Q65.2]]''' Congenital dislocation of hip, unspecified
 +
 +
*'''[[Q65.3]]''' Congenital partial dislocation of hip, unilateral
 +
 +
*'''[[Q65.30]]''' Congenital partial dislocation of unspecified hip, unilateral
 +
 +
*'''[[Q65.31]]''' Congenital partial dislocation of right hip, unilateral
 +
 +
*'''[[Q65.32]]''' Congenital partial dislocation of left hip, unilateral
 +
 +
*'''[[Q65.4]]''' Congenital partial dislocation of hip, bilateral
 +
 +
*'''[[Q65.5]]''' Congenital partial dislocation of hip, unspecified
 +
 +
*'''[[Q65.6]]''' Congenital unstable hip
 +
 +
Congenital dislocatable hip
 +
 +
*'''[[Q65.8]]''' Other congenital deformities of hip
 +
 +
*'''[[Q65.81]]''' Congenital coxa valga
 +
 +
*'''[[Q65.82]]''' Congenital coxa vara
 +
 +
*'''[[Q65.89]]''' Other specified congenital deformities of hip
 +
 +
Anteversion of femoral neck
 +
 +
Congenital acetabular dysplasia
 +
 +
*'''[[Q65.9]]''' Congenital deformity of hip, unspecified
 +
 +
*'''[[Q66]]''' Congenital deformities of feet
 +
 +
''Excludes1''
 +
 +
reduction defects of feet (Q72.-)
 +
 +
valgus deformities (acquired) (M21.0-)
 +
 +
varus deformities (acquired) (M21.1-)
 +
 +
*'''[[Q66.0]]''' Congenital talipes equinovarus
 +
 +
*'''[[Q66.00]]''' Congenital talipes equinovarus, unspecified foot
 +
 +
*'''[[Q66.01]]''' Congenital talipes equinovarus, right foot
 +
 +
*'''[[Q66.02]]''' Congenital talipes equinovarus, left foot
 +
 +
*'''[[Q66.1]]''' Congenital talipes calcaneovarus
 +
 +
*'''[[Q66.10]]''' Congenital talipes calcaneovarus, unspecified foot
 +
 +
*'''[[Q66.11]]''' Congenital talipes calcaneovarus, right foot
 +
 +
*'''[[Q66.12]]''' Congenital talipes calcaneovarus, left foot
 +
 +
*'''[[Q66.2]]''' Congenital metatarsus (primus) varus
 +
 +
*'''[[Q66.21]]''' Congenital metatarsus primus varus
 +
 +
*'''[[Q66.211]]''' Congenital metatarsus primus varus, right foot
 +
 +
*'''[[Q66.212]]''' Congenital metatarsus primus varus, left foot
 +
 +
*'''[[Q66.219]]''' Congenital metatarsus primus varus, unspecified foot
 +
 +
*'''[[Q66.22]]''' Congenital metatarsus adductus
 +
 +
Congenital metatarsus varus
 +
 +
*'''[[Q66.221]]''' Congenital metatarsus adductus, right foot
 +
 +
*'''[[Q66.222]]''' Congenital metatarsus adductus, left foot
 +
 +
*'''[[Q66.229]]''' Congenital metatarsus adductus, unspecified foot
 +
 +
*'''[[Q66.3]]''' Other congenital varus deformities of feet
 +
 +
Hallux varus, congenital
 +
 +
*'''[[Q66.30]]''' Other congenital varus deformities of feet, unspecified foot
 +
 +
*'''[[Q66.31]]''' Other congenital varus deformities of feet, right foot
 +
 +
*'''[[Q66.32]]''' Other congenital varus deformities of feet, left foot
 +
 +
*'''[[Q66.4]]''' Congenital talipes calcaneovalgus
 +
 +
*'''[[Q66.40]]''' Congenital talipes calcaneovalgus, unspecified foot
 +
 +
*'''[[Q66.41]]''' Congenital talipes calcaneovalgus, right foot
 +
 +
*'''[[Q66.42]]''' Congenital talipes calcaneovalgus, left foot
 +
 +
*'''[[Q66.5]]''' Congenital pes planus
 +
 +
Congenital flat foot
 +
 +
Congenital rigid flat foot
 +
 +
Congenital spastic (everted) flat foot
 +
 +
''Excludes1''
 +
 +
pes planus, acquired (M21.4)
 +
 +
*'''[[Q66.50]]''' Congenital pes planus, unspecified foot
 +
 +
*'''[[Q66.51]]''' Congenital pes planus, right foot
 +
 +
*'''[[Q66.52]]''' Congenital pes planus, left foot
 +
 +
*'''[[Q66.6]]''' Other congenital valgus deformities of feet
 +
 +
Congenital metatarsus valgus
 +
 +
*'''[[Q66.7]]''' Congenital pes cavus
 +
 +
*'''[[Q66.70]]''' Congenital pes cavus, unspecified foot
 +
 +
*'''[[Q66.71]]''' Congenital pes cavus, right foot
 +
 +
*'''[[Q66.72]]''' Congenital pes cavus, left foot
 +
 +
*'''[[Q66.8]]''' Other congenital deformities of feet
 +
 +
*'''[[Q66.80]]''' Congenital vertical talus deformity, unspecified foot
 +
 +
*'''[[Q66.81]]''' Congenital vertical talus deformity, right foot
 +
 +
*'''[[Q66.82]]''' Congenital vertical talus deformity, left foot
 +
 +
*'''[[Q66.89]]''' Other specified congenital deformities of feet
 +
 +
Congenital asymmetric talipes
 +
 +
Congenital clubfoot NOS
 +
 +
Congenital talipes NOS
 +
 +
Congenital tarsal coalition
 +
 +
Hammer toe, congenital
 +
 +
*'''[[Q66.9]]''' Congenital deformity of feet, unspecified
 +
 +
*'''[[Q66.90]]''' Congenital deformity of feet, unspecified, unspecified foot
 +
 +
*'''[[Q66.91]]''' Congenital deformity of feet, unspecified, right foot
 +
 +
*'''[[Q66.92]]''' Congenital deformity of feet, unspecified, left foot
 +
 +
*'''[[Q67]]''' Congenital musculoskeletal deformities of head, face, spine and chest
 +
 +
''Excludes1''
 +
 +
congenital malformation syndromes classified to Q87.-
 +
 +
Potter's syndrome (Q60.6)
 +
 +
*'''[[Q67.0]]''' Congenital facial asymmetry
 +
 +
*'''[[Q67.1]]''' Congenital compression facies
 +
 +
*'''[[Q67.2]]''' Dolichocephaly
 +
 +
*'''[[Q67.3]]''' Plagiocephaly
 +
 +
*'''[[Q67.4]]''' Other congenital deformities of skull, face and jaw
 +
 +
Congenital depressions in skull
 +
 +
Congenital hemifacial atrophy or hypertrophy
 +
 +
Deviation of nasal septum, congenital
 +
 +
Squashed or bent nose, congenital
 +
 +
''Excludes1''
 +
 +
dentofacial anomalies (including malocclusion) (M26.-)
 +
 +
syphilitic saddle nose (A50.5)
 +
 +
*'''[[Q67.5]]''' Congenital deformity of spine
 +
 +
Congenital postural scoliosis
 +
 +
Congenital scoliosis NOS
 +
 +
''Excludes1''
 +
 +
infantile idiopathic scoliosis (M41.0)
 +
 +
scoliosis due to congenital bony malformation (Q76.3)
 +
 +
*'''[[Q67.6]]''' Pectus excavatum
 +
 +
Congenital funnel chest
 +
 +
*'''[[Q67.7]]''' Pectus carinatum
 +
 +
Congenital pigeon chest
 +
 +
*'''[[Q67.8]]''' Other congenital deformities of chest
 +
 +
Congenital deformity of chest wall NOS
 +
 +
*'''[[Q68]]''' Other congenital musculoskeletal deformities
 +
 +
''Excludes1''
 +
 +
reduction defects of limb(s) (Q71-Q73)
 +
 +
''Excludes2''
 +
 +
congenital myotonic chondrodystrophy (G71.13)
 +
 +
*'''[[Q68.0]]''' Congenital deformity of sternocleidomastoid muscle
 +
 +
Congenital contracture of sternocleidomastoid (muscle)
 +
 +
Congenital (sternomastoid) torticollis
 +
 +
Sternomastoid tumor (congenital)
 +
 +
*'''[[Q68.1]]''' Congenital deformity of finger(s) and hand
 +
 +
Congenital clubfinger
 +
 +
Spade-like hand (congenital)
 +
 +
*'''[[Q68.2]]''' Congenital deformity of knee
 +
 +
Congenital dislocation of knee
 +
 +
Congenital genu recurvatum
 +
 +
*'''[[Q68.3]]''' Congenital bowing of femur
 +
 +
''Excludes1''
 +
 +
anteversion of femur (neck) (Q65.89)
 +
 +
*'''[[Q68.4]]''' Congenital bowing of tibia and fibula
 +
 +
*'''[[Q68.5]]''' Congenital bowing of long bones of leg, unspecified
 +
 +
*'''[[Q68.6]]''' Discoid meniscus
 +
 +
*'''[[Q68.8]]''' Other specified congenital musculoskeletal deformities
 +
 +
Congenital deformity of clavicle
 +
 +
Congenital deformity of elbow
 +
 +
Congenital deformity of forearm
 +
 +
Congenital deformity of scapula
 +
 +
Congenital deformity of wrist
 +
 +
Congenital dislocation of elbow
 +
 +
Congenital dislocation of shoulder
 +
 +
Congenital dislocation of wrist
 +
 +
*'''[[Q69]]''' Polydactyly
 +
 +
*'''[[Q69.0]]''' Accessory finger(s)
 +
 +
*'''[[Q69.1]]''' Accessory thumb(s)
 +
 +
*'''[[Q69.2]]''' Accessory toe(s)
 +
 +
Accessory hallux
 +
 +
*'''[[Q69.9]]''' Polydactyly, unspecified
 +
 +
Supernumerary digit(s) NOS
 +
 +
*'''[[Q70]]''' Syndactyly
 +
 +
*'''[[Q70.0]]''' Fused fingers
 +
 +
Complex syndactyly of fingers with synostosis
 +
 +
*'''[[Q70.00]]''' Fused fingers, unspecified hand
 +
 +
*'''[[Q70.01]]''' Fused fingers, right hand
 +
 +
*'''[[Q70.02]]''' Fused fingers, left hand
 +
 +
*'''[[Q70.03]]''' Fused fingers, bilateral
 +
 +
*'''[[Q70.1]]''' Webbed fingers
 +
 +
Simple syndactyly of fingers without synostosis
 +
 +
*'''[[Q70.10]]''' Webbed fingers, unspecified hand
 +
 +
*'''[[Q70.11]]''' Webbed fingers, right hand
 +
 +
*'''[[Q70.12]]''' Webbed fingers, left hand
 +
 +
*'''[[Q70.13]]''' Webbed fingers, bilateral
 +
 +
*'''[[Q70.2]]''' Fused toes
 +
 +
Complex syndactyly of toes with synostosis
 +
 +
*'''[[Q70.20]]''' Fused toes, unspecified foot
 +
 +
*'''[[Q70.21]]''' Fused toes, right foot
 +
 +
*'''[[Q70.22]]''' Fused toes, left foot
 +
 +
*'''[[Q70.23]]''' Fused toes, bilateral
 +
 +
*'''[[Q70.3]]''' Webbed toes
 +
 +
Simple syndactyly of toes without synostosis
 +
 +
*'''[[Q70.30]]''' Webbed toes, unspecified foot
 +
 +
*'''[[Q70.31]]''' Webbed toes, right foot
 +
 +
*'''[[Q70.32]]''' Webbed toes, left foot
 +
 +
*'''[[Q70.33]]''' Webbed toes, bilateral
 +
 +
*'''[[Q70.4]]''' Polysyndactyly, unspecified
 +
 +
''Excludes1''
 +
 +
specified syndactyly of hand and feet - code to specified conditions (Q70.0- -Q70.3-)
 +
 +
*'''[[Q70.9]]''' Syndactyly, unspecified
 +
 +
Symphalangy NOS
 +
 +
*'''[[Q71]]''' Reduction defects of upper limb
 +
 +
*'''[[Q71.0]]''' Congenital complete absence of upper limb
 +
 +
*'''[[Q71.00]]''' Congenital complete absence of unspecified upper limb
 +
 +
*'''[[Q71.01]]''' Congenital complete absence of right upper limb
 +
 +
*'''[[Q71.02]]''' Congenital complete absence of left upper limb
 +
 +
*'''[[Q71.03]]''' Congenital complete absence of upper limb, bilateral
 +
 +
*'''[[Q71.1]]''' Congenital absence of upper arm and forearm with hand present
 +
 +
*'''[[Q71.10]]''' Congenital absence of unspecified upper arm and forearm with hand present
 +
 +
*'''[[Q71.11]]''' Congenital absence of right upper arm and forearm with hand present
 +
 +
*'''[[Q71.12]]''' Congenital absence of left upper arm and forearm with hand present
 +
 +
*'''[[Q71.13]]''' Congenital absence of upper arm and forearm with hand present, bilateral
 +
 +
*'''[[Q71.2]]''' Congenital absence of both forearm and hand
 +
 +
*'''[[Q71.20]]''' Congenital absence of both forearm and hand, unspecified upper limb
 +
 +
*'''[[Q71.21]]''' Congenital absence of both forearm and hand, right upper limb
 +
 +
*'''[[Q71.22]]''' Congenital absence of both forearm and hand, left upper limb
 +
 +
*'''[[Q71.23]]''' Congenital absence of both forearm and hand, bilateral
 +
 +
*'''[[Q71.3]]''' Congenital absence of hand and finger
 +
 +
*'''[[Q71.30]]''' Congenital absence of unspecified hand and finger
 +
 +
*'''[[Q71.31]]''' Congenital absence of right hand and finger
 +
 +
*'''[[Q71.32]]''' Congenital absence of left hand and finger
 +
 +
*'''[[Q71.33]]''' Congenital absence of hand and finger, bilateral
 +
 +
*'''[[Q71.4]]''' Longitudinal reduction defect of radius
 +
 +
Clubhand (congenital)
 +
 +
Radial clubhand
 +
 +
*'''[[Q71.40]]''' Longitudinal reduction defect of unspecified radius
 +
 +
*'''[[Q71.41]]''' Longitudinal reduction defect of right radius
 +
 +
*'''[[Q71.42]]''' Longitudinal reduction defect of left radius
 +
 +
*'''[[Q71.43]]''' Longitudinal reduction defect of radius, bilateral
 +
 +
*'''[[Q71.5]]''' Longitudinal reduction defect of ulna
 +
 +
*'''[[Q71.50]]''' Longitudinal reduction defect of unspecified ulna
 +
 +
*'''[[Q71.51]]''' Longitudinal reduction defect of right ulna
 +
 +
*'''[[Q71.52]]''' Longitudinal reduction defect of left ulna
 +
 +
*'''[[Q71.53]]''' Longitudinal reduction defect of ulna, bilateral
 +
 +
*'''[[Q71.6]]''' Lobster-claw hand
 +
 +
*'''[[Q71.60]]''' Lobster-claw hand, unspecified hand
 +
 +
*'''[[Q71.61]]''' Lobster-claw right hand
 +
 +
*'''[[Q71.62]]''' Lobster-claw left hand
 +
 +
*'''[[Q71.63]]''' Lobster-claw hand, bilateral
 +
 +
*'''[[Q71.8]]''' Other reduction defects of upper limb
 +
 +
*'''[[Q71.81]]''' Congenital shortening of upper limb
 +
 +
*'''[[Q71.811]]''' Congenital shortening of right upper limb
 +
 +
*'''[[Q71.812]]''' Congenital shortening of left upper limb
 +
 +
*'''[[Q71.813]]''' Congenital shortening of upper limb, bilateral
 +
 +
*'''[[Q71.819]]''' Congenital shortening of unspecified upper limb
 +
 +
*'''[[Q71.89]]''' Other reduction defects of upper limb
 +
 +
*'''[[Q71.891]]''' Other reduction defects of right upper limb
 +
 +
*'''[[Q71.892]]''' Other reduction defects of left upper limb
 +
 +
*'''[[Q71.893]]''' Other reduction defects of upper limb, bilateral
 +
 +
*'''[[Q71.899]]''' Other reduction defects of unspecified upper limb
 +
 +
*'''[[Q71.9]]''' Unspecified reduction defect of upper limb
 +
 +
*'''[[Q71.90]]''' Unspecified reduction defect of unspecified upper limb
 +
 +
*'''[[Q71.91]]''' Unspecified reduction defect of right upper limb
 +
 +
*'''[[Q71.92]]''' Unspecified reduction defect of left upper limb
 +
 +
*'''[[Q71.93]]''' Unspecified reduction defect of upper limb, bilateral
 +
 +
*'''[[Q72]]''' Reduction defects of lower limb
 +
 +
*'''[[Q72.0]]''' Congenital complete absence of lower limb
 +
 +
*'''[[Q72.00]]''' Congenital complete absence of unspecified lower limb
 +
 +
*'''[[Q72.01]]''' Congenital complete absence of right lower limb
 +
 +
*'''[[Q72.02]]''' Congenital complete absence of left lower limb
 +
 +
*'''[[Q72.03]]''' Congenital complete absence of lower limb, bilateral
 +
 +
*'''[[Q72.1]]''' Congenital absence of thigh and lower leg with foot present
 +
 +
*'''[[Q72.10]]''' Congenital absence of unspecified thigh and lower leg with foot present
 +
 +
*'''[[Q72.11]]''' Congenital absence of right thigh and lower leg with foot present
 +
 +
*'''[[Q72.12]]''' Congenital absence of left thigh and lower leg with foot present
 +
 +
*'''[[Q72.13]]''' Congenital absence of thigh and lower leg with foot present, bilateral
 +
 +
*'''[[Q72.2]]''' Congenital absence of both lower leg and foot
 +
 +
*'''[[Q72.20]]''' Congenital absence of both lower leg and foot, unspecified lower limb
 +
 +
*'''[[Q72.21]]''' Congenital absence of both lower leg and foot, right lower limb
 +
 +
*'''[[Q72.22]]''' Congenital absence of both lower leg and foot, left lower limb
 +
 +
*'''[[Q72.23]]''' Congenital absence of both lower leg and foot, bilateral
 +
 +
*'''[[Q72.3]]''' Congenital absence of foot and toe(s)
 +
 +
*'''[[Q72.30]]''' Congenital absence of unspecified foot and toe(s)
 +
 +
*'''[[Q72.31]]''' Congenital absence of right foot and toe(s)
 +
 +
*'''[[Q72.32]]''' Congenital absence of left foot and toe(s)
 +
 +
*'''[[Q72.33]]''' Congenital absence of foot and toe(s), bilateral
 +
 +
*'''[[Q72.4]]''' Longitudinal reduction defect of femur
 +
 +
Proximal femoral focal deficiency
 +
 +
*'''[[Q72.40]]''' Longitudinal reduction defect of unspecified femur
 +
 +
*'''[[Q72.41]]''' Longitudinal reduction defect of right femur
 +
 +
*'''[[Q72.42]]''' Longitudinal reduction defect of left femur
 +
 +
*'''[[Q72.43]]''' Longitudinal reduction defect of femur, bilateral
 +
 +
*'''[[Q72.5]]''' Longitudinal reduction defect of tibia
 +
 +
*'''[[Q72.50]]''' Longitudinal reduction defect of unspecified tibia
 +
 +
*'''[[Q72.51]]''' Longitudinal reduction defect of right tibia
 +
 +
*'''[[Q72.52]]''' Longitudinal reduction defect of left tibia
 +
 +
*'''[[Q72.53]]''' Longitudinal reduction defect of tibia, bilateral
 +
 +
*'''[[Q72.6]]''' Longitudinal reduction defect of fibula
 +
 +
*'''[[Q72.60]]''' Longitudinal reduction defect of unspecified fibula
 +
 +
*'''[[Q72.61]]''' Longitudinal reduction defect of right fibula
 +
 +
*'''[[Q72.62]]''' Longitudinal reduction defect of left fibula
 +
 +
*'''[[Q72.63]]''' Longitudinal reduction defect of fibula, bilateral
 +
 +
*'''[[Q72.7]]''' Split foot
 +
 +
*'''[[Q72.70]]''' Split foot, unspecified lower limb
 +
 +
*'''[[Q72.71]]''' Split foot, right lower limb
 +
 +
*'''[[Q72.72]]''' Split foot, left lower limb
 +
 +
*'''[[Q72.73]]''' Split foot, bilateral
 +
 +
*'''[[Q72.8]]''' Other reduction defects of lower limb
 +
 +
*'''[[Q72.81]]''' Congenital shortening of lower limb
 +
 +
*'''[[Q72.811]]''' Congenital shortening of right lower limb
 +
 +
*'''[[Q72.812]]''' Congenital shortening of left lower limb
 +
 +
*'''[[Q72.813]]''' Congenital shortening of lower limb, bilateral
 +
 +
*'''[[Q72.819]]''' Congenital shortening of unspecified lower limb
 +
 +
*'''[[Q72.89]]''' Other reduction defects of lower limb
 +
 +
*'''[[Q72.891]]''' Other reduction defects of right lower limb
 +
 +
*'''[[Q72.892]]''' Other reduction defects of left lower limb
 +
 +
*'''[[Q72.893]]''' Other reduction defects of lower limb, bilateral
 +
 +
*'''[[Q72.899]]''' Other reduction defects of unspecified lower limb
 +
 +
*'''[[Q72.9]]''' Unspecified reduction defect of lower limb
 +
 +
*'''[[Q72.90]]''' Unspecified reduction defect of unspecified lower limb
 +
 +
*'''[[Q72.91]]''' Unspecified reduction defect of right lower limb
 +
 +
*'''[[Q72.92]]''' Unspecified reduction defect of left lower limb
 +
 +
*'''[[Q72.93]]''' Unspecified reduction defect of lower limb, bilateral
 +
 +
*'''[[Q73]]''' Reduction defects of unspecified limb
 +
 +
*'''[[Q73.0]]''' Congenital absence of unspecified limb(s)
 +
 +
Amelia NOS
 +
 +
*'''[[Q73.1]]''' Phocomelia, unspecified limb(s)
 +
 +
Phocomelia NOS
 +
 +
*'''[[Q73.8]]''' Other reduction defects of unspecified limb(s)
 +
 +
Longitudinal reduction deformity of unspecified limb(s)
 +
 +
Ectromelia of limb NOS
 +
 +
Hemimelia of limb NOS
 +
 +
Reduction defect of limb NOS
 +
 +
*'''[[Q74]]''' Other congenital malformations of limb(s)
 +
 +
''Excludes1''
 +
 +
polydactyly (Q69.-)
 +
 +
reduction defect of limb (Q71-Q73)
 +
 +
syndactyly (Q70.-)
 +
 +
*'''[[Q74.0]]''' Other congenital malformations of upper limb(s), including shoulder girdle
 +
 +
Accessory carpal bones
 +
 +
Cleidocranial dysostosis
 +
 +
Congenital pseudarthrosis of clavicle
 +
 +
Macrodactylia (fingers)
 +
 +
Madelung's deformity
 +
 +
Radioulnar synostosis
 +
 +
Sprengel's deformity
 +
 +
Triphalangeal thumb
 +
 +
*'''[[Q74.1]]''' Congenital malformation of knee
 +
 +
Congenital absence of patella
 +
 +
Congenital dislocation of patella
 +
 +
Congenital genu valgum
 +
 +
Congenital genu varum
 +
 +
Rudimentary patella
 +
 +
''Excludes1''
 +
 +
congenital dislocation of knee (Q68.2)
 +
 +
congenital genu recurvatum (Q68.2)
 +
 +
nail patella syndrome (Q87.2)
 +
 +
*'''[[Q74.2]]''' Other congenital malformations of lower limb(s), including pelvic girdle
 +
 +
Congenital fusion of sacroiliac joint
 +
 +
Congenital malformation of ankle joint
 +
 +
Congenital malformation of sacroiliac joint
 +
 +
''Excludes1''
 +
 +
anteversion of femur (neck) (Q65.89)
 +
 +
*'''[[Q74.3]]''' Arthrogryposis multiplex congenita
 +
 +
*'''[[Q74.8]]''' Other specified congenital malformations of limb(s)
 +
 +
*'''[[Q74.9]]''' Unspecified congenital malformation of limb(s)
 +
 +
Congenital anomaly of limb(s) NOS
 +
 +
*'''[[Q75]]''' Other congenital malformations of skull and face bones
 +
 +
''Excludes1''
 +
 +
congenital malformation of face NOS (Q18.-)
 +
 +
congenital malformation syndromes classified to Q87.-
 +
 +
dentofacial anomalies (including malocclusion) (M26.-)
 +
 +
musculoskeletal deformities of head and face (Q67.0-Q67.4)
 +
 +
skull defects associated with congenital anomalies of brain such as
 +
 +
anencephaly (Q00.0)
 +
 +
encephalocele (Q01.-)
 +
 +
hydrocephalus (Q03.-)
 +
 +
microcephaly (Q02)
 +
 +
*'''[[Q75.0]]''' Craniosynostosis
 +
 +
Acrocephaly
 +
 +
Imperfect fusion of skull
 +
 +
Oxycephaly
 +
 +
Trigonocephaly
 +
 +
*'''[[Q75.1]]''' Craniofacial dysostosis
 +
 +
Crouzon's disease
 +
 +
*'''[[Q75.2]]''' Hypertelorism
 +
 +
*'''[[Q75.3]]''' Macrocephaly
 +
 +
*'''[[Q75.4]]''' Mandibulofacial dysostosis
 +
 +
Franceschetti syndrome
 +
 +
Treacher Collins syndrome
 +
 +
*'''[[Q75.5]]''' Oculomandibular dysostosis
 +
 +
*'''[[Q75.8]]''' Other specified congenital malformations of skull and face bones
 +
 +
Absence of skull bone, congenital
 +
 +
Congenital deformity of forehead
 +
 +
Platybasia
 +
 +
*'''[[Q75.9]]''' Congenital malformation of skull and face bones, unspecified
 +
 +
Congenital anomaly of face bones NOS
 +
 +
Congenital anomaly of skull NOS
 +
 +
*'''[[Q76]]''' Congenital malformations of spine and bony thorax
 +
 +
''Excludes1''
 +
 +
congenital musculoskeletal deformities of spine and chest (Q67.5-Q67.8)
 +
 +
*'''[[Q76.0]]''' Spina bifida occulta
 +
 +
''Excludes1''
 +
 +
meningocele (spinal) (Q05.-)
 +
 +
spina bifida (aperta) (cystica) (Q05.-)
 +
 +
*'''[[Q76.1]]''' Klippel-Feil syndrome
 +
 +
Cervical fusion syndrome
 +
 +
*'''[[Q76.2]]''' Congenital spondylolisthesis
 +
 +
Congenital spondylolysis
 +
 +
''Excludes1''
 +
 +
spondylolisthesis (acquired) (M43.1-)
 +
 +
spondylolysis (acquired) (M43.0-)
 +
 +
*'''[[Q76.3]]''' Congenital scoliosis due to congenital bony malformation
 +
 +
Hemivertebra fusion or failure of segmentation with scoliosis
 +
 +
*'''[[Q76.4]]''' Other congenital malformations of spine, not associated with scoliosis
 +
 +
*'''[[Q76.41]]''' Congenital kyphosis
 +
 +
*'''[[Q76.411]]''' Congenital kyphosis, occipito-atlanto-axial region
 +
 +
*'''[[Q76.412]]''' Congenital kyphosis, cervical region
 +
 +
*'''[[Q76.413]]''' Congenital kyphosis, cervicothoracic region
 +
 +
*'''[[Q76.414]]''' Congenital kyphosis, thoracic region
 +
 +
*'''[[Q76.415]]''' Congenital kyphosis, thoracolumbar region
 +
 +
*'''[[Q76.419]]''' Congenital kyphosis, unspecified region
 +
 +
*'''[[Q76.42]]''' Congenital lordosis
 +
 +
*'''[[Q76.425]]''' Congenital lordosis, thoracolumbar region
 +
 +
*'''[[Q76.426]]''' Congenital lordosis, lumbar region
 +
 +
*'''[[Q76.427]]''' Congenital lordosis, lumbosacral region
 +
 +
*'''[[Q76.428]]''' Congenital lordosis, sacral and sacrococcygeal region
 +
 +
*'''[[Q76.429]]''' Congenital lordosis, unspecified region
 +
 +
*'''[[Q76.49]]''' Other congenital malformations of spine, not associated with scoliosis
 +
 +
Congenital absence of vertebra NOS
 +
 +
Congenital fusion of spine NOS
 +
 +
Congenital malformation of lumbosacral (joint) (region) NOS
 +
 +
Congenital malformation of spine NOS
 +
 +
Hemivertebra NOS
 +
 +
Malformation of spine NOS
 +
 +
Platyspondylisis NOS
 +
 +
Supernumerary vertebra NOS
 +
 +
*'''[[Q76.5]]''' Cervical rib
 +
 +
Supernumerary rib in cervical region
 +
 +
*'''[[Q76.6]]''' Other congenital malformations of ribs
 +
 +
Accessory rib
 +
 +
Congenital absence of rib
 +
 +
Congenital fusion of ribs
 +
 +
Congenital malformation of ribs NOS
 +
 +
''Excludes1''
 +
 +
short rib syndrome (Q77.2)
 +
 +
*'''[[Q76.7]]''' Congenital malformation of sternum
 +
 +
Congenital absence of sternum
 +
 +
Sternum bifidum
 +
 +
*'''[[Q76.8]]''' Other congenital malformations of bony thorax
 +
 +
*'''[[Q76.9]]''' Congenital malformation of bony thorax, unspecified
 +
 +
*'''[[Q77]]''' Osteochondrodysplasia with defects of growth of tubular bones and spine
 +
 +
''Excludes1''
 +
 +
mucopolysaccharidosis (E76.0-E76.3)
 +
 +
''Excludes2''
 +
 +
congenital myotonic chondrodystrophy (G71.13)
 +
 +
*'''[[Q77.0]]''' Achondrogenesis
 +
 +
Hypochondrogenesis
 +
 +
*'''[[Q77.1]]''' Thanatophoric short stature
 +
 +
*'''[[Q77.2]]''' Short rib syndrome
 +
 +
Asphyxiating thoracic dysplasia (Jeune)
 +
 +
*'''[[Q77.3]]''' Chondrodysplasia punctata
 +
 +
''Excludes1''
 +
 +
Rhizomelic chondrodysplasia punctata (E71.43)
 +
 +
*'''[[Q77.4]]''' Achondroplasia
 +
 +
Hypochondroplasia
 +
 +
Osteosclerosis congenita
 +
 +
*'''[[Q77.5]]''' Diastrophic dysplasia
 +
 +
*'''[[Q77.6]]''' Chondroectodermal dysplasia
 +
 +
Ellis-van Creveld syndrome
 +
 +
*'''[[Q77.7]]''' Spondyloepiphyseal dysplasia
 +
 +
*'''[[Q77.8]]''' Other osteochondrodysplasia with defects of growth of tubular bones and spine
 +
 +
*'''[[Q77.9]]''' Osteochondrodysplasia with defects of growth of tubular bones and spine, unspecified
 +
 +
*'''[[Q78]]''' Other osteochondrodysplasias
 +
 +
''Excludes2''
 +
 +
congenital myotonic chondrodystrophy (G71.13)
 +
 +
*'''[[Q78.0]]''' Osteogenesis imperfecta
 +
 +
Fragilitas ossium
 +
 +
Osteopsathyrosis
 +
 +
*'''[[Q78.1]]''' Polyostotic fibrous dysplasia
 +
 +
Albright(-McCune)(-Sternberg) syndrome
 +
 +
*'''[[Q78.2]]''' Osteopetrosis
 +
 +
Albers-Schönberg syndrome
 +
 +
Osteosclerosis NOS
 +
 +
*'''[[Q78.3]]''' Progressive diaphyseal dysplasia
 +
 +
Camurati-Engelmann syndrome
 +
 +
*'''[[Q78.4]]''' Enchondromatosis
 +
 +
Maffucci's syndrome
 +
 +
Ollier's disease
 +
 +
*'''[[Q78.5]]''' Metaphyseal dysplasia
 +
 +
Pyle's syndrome
 +
 +
*'''[[Q78.6]]''' Multiple congenital exostoses
 +
 +
Diaphyseal aclasis
 +
 +
*'''[[Q78.8]]''' Other specified osteochondrodysplasias
 +
 +
Osteopoikilosis
 +
 +
*'''[[Q78.9]]''' Osteochondrodysplasia, unspecified
 +
 +
Chondrodystrophy NOS
 +
 +
Osteodystrophy NOS
 +
 +
*'''[[Q79]]''' Congenital malformations of musculoskeletal system, not elsewhere classified
 +
 +
''Excludes2''
 +
 +
congenital (sternomastoid) torticollis (Q68.0)
 +
 +
*'''[[Q79.0]]''' Congenital diaphragmatic hernia
 +
 +
''Excludes1''
 +
 +
congenital hiatus hernia (Q40.1)
 +
 +
*'''[[Q79.1]]''' Other congenital malformations of diaphragm
 +
 +
Absence of diaphragm
 +
 +
Congenital malformation of diaphragm NOS
 +
 +
Eventration of diaphragm
 +
 +
*'''[[Q79.2]]''' Exomphalos
 +
 +
Omphalocele
 +
 +
''Excludes1''
 +
 +
umbilical hernia (K42.-)
 +
 +
*'''[[Q79.3]]''' Gastroschisis
 +
 +
*'''[[Q79.4]]''' Prune belly syndrome
 +
 +
Congenital prolapse of bladder mucosa
 +
 +
Eagle-Barrett syndrome
 +
 +
*'''[[Q79.5]]''' Other congenital malformations of abdominal wall
 +
 +
''Excludes1''
 +
 +
umbilical hernia (K42.-)
 +
 +
*'''[[Q79.51]]''' Congenital hernia of bladder
 +
 +
*'''[[Q79.59]]''' Other congenital malformations of abdominal wall
 +
 +
*'''[[Q79.6]]''' Ehlers-Danlos syndromes
 +
 +
*'''[[Q79.60]]''' Ehlers-Danlos syndrome, unspecified
 +
 +
*'''[[Q79.61]]''' Classical Ehlers-Danlos syndrome
 +
 +
Classical EDS (cEDS)
 +
 +
*'''[[Q79.62]]''' Hypermobile Ehlers-Danlos syndrome
 +
 +
Hypermobile EDS (hEDS)
 +
 +
*'''[[Q79.63]]''' Vascular Ehlers-Danlos syndrome
 +
 +
Vascular EDS (vEDS)
 +
 +
*'''[[Q79.69]]''' Other Ehlers-Danlos syndromes
 +
 +
*'''[[Q79.8]]''' Other congenital malformations of musculoskeletal system
 +
 +
Absence of muscle
 +
 +
Absence of tendon
 +
 +
Accessory muscle
 +
 +
Amyotrophia congenita
 +
 +
Congenital constricting bands
 +
 +
Congenital shortening of tendon
 +
 +
Poland syndrome
 +
 +
*'''[[Q79.9]]''' Congenital malformation of musculoskeletal system, unspecified
 +
 +
Congenital anomaly of musculoskeletal system NOS
 +
 +
Congenital deformity of musculoskeletal system NOS
 +
 +
Other congenital malformations (Q80-Q89)
 +
 +
*'''[[Q80]]''' Congenital ichthyosis
 +
 +
''Excludes1''
 +
 +
Refsum's disease (G60.1)
 +
 +
*'''[[Q80.0]]''' Ichthyosis vulgaris
 +
 +
*'''[[Q80.1]]''' X-linked ichthyosis
 +
 +
*'''[[Q80.2]]''' Lamellar ichthyosis
 +
 +
Collodion baby
 +
 +
*'''[[Q80.3]]''' Congenital bullous ichthyosiform erythroderma
 +
 +
*'''[[Q80.4]]''' Harlequin fetus
 +
 +
*'''[[Q80.8]]''' Other congenital ichthyosis
 +
 +
*'''[[Q80.9]]''' Congenital ichthyosis, unspecified
 +
 +
*'''[[Q81]]''' Epidermolysis bullosa
 +
 +
*'''[[Q81.0]]''' Epidermolysis bullosa simplex
 +
 +
''Excludes1''
 +
 +
Cockayne's syndrome (Q87.19)
 +
 +
*'''[[Q81.1]]''' Epidermolysis bullosa letalis
 +
 +
Herlitz' syndrome
 +
 +
*'''[[Q81.2]]''' Epidermolysis bullosa dystrophica
 +
 +
*'''[[Q81.8]]''' Other epidermolysis bullosa
 +
 +
*'''[[Q81.9]]''' Epidermolysis bullosa, unspecified
 +
 +
*'''[[Q82]]''' Other congenital malformations of skin
 +
 +
''Excludes1''
 +
 +
acrodermatitis enteropathica (E83.2)
 +
 +
congenital erythropoietic porphyria (E80.0)
 +
 +
pilonidal cyst or sinus (L05.-)
 +
 +
Sturge-Weber (-Dimitri) syndrome (Q85.8)
 +
 +
*'''[[Q82.0]]''' Hereditary lymphedema
 +
 +
*'''[[Q82.1]]''' Xeroderma pigmentosum
 +
 +
*'''[[Q82.2]]''' Congenital cutaneous mastocytosis
 +
 +
Congenital diffuse cutaneous mastocytosis
 +
 +
Congenital maculopapular cutaneous mastocytosis
 +
 +
Congenital urticaria pigmentosa
 +
 +
''Excludes1''
 +
 +
cutaneous mastocytosis NOS (D47.01)
 +
 +
diffuse cutaneous mastocytosis (with onset after newborn period) (D47.01)
 +
 +
malignant mastocytosis (C96.2-)
 +
 +
systemic mastocytosis (D47.02)
 +
 +
urticaria pigmentosa (non-congenital) (with onset after newborn period) (D47.01)
 +
 +
*'''[[Q82.3]]''' Incontinentia pigmenti
 +
 +
*'''[[Q82.4]]''' Ectodermal dysplasia (anhidrotic)
 +
 +
''Excludes1''
 +
 +
Ellis-van Creveld syndrome (Q77.6)
 +
 +
*'''[[Q82.5]]''' Congenital non-neoplastic nevus
 +
 +
Birthmark NOS
 +
 +
Flammeus Nevus
 +
 +
Portwine Nevus
 +
 +
Sanguineous Nevus
 +
 +
Strawberry Nevus
 +
 +
Vascular Nevus NOS
 +
 +
Verrucous Nevus
 +
 +
''Excludes2''
 +
 +
Café au lait spots (L81.3)
 +
 +
lentigo (L81.4)
 +
 +
nevus NOS (D22.-)
 +
 +
araneus nevus (I78.1)
 +
 +
melanocytic nevus (D22.-)
 +
 +
pigmented nevus (D22.-)
 +
 +
spider nevus (I78.1)
 +
 +
stellar nevus (I78.1)
 +
 +
*'''[[Q82.6]]''' Congenital sacral dimple
 +
 +
Parasacral dimple
 +
 +
''Excludes2''
 +
 +
pilonidal cyst with abscess (L05.01)
 +
 +
pilonidal cyst without abscess (L05.91)
 +
 +
*'''[[Q82.8]]''' Other specified congenital malformations of skin
 +
 +
Abnormal palmar creases
 +
 +
Accessory skin tags
 +
 +
Benign familial pemphigus (Hailey-Hailey)
 +
 +
Congenital poikiloderma
 +
 +
Cutis laxa (hyperelastica)
 +
 +
Dermatoglyphic anomalies
 +
 +
Inherited keratosis palmaris et plantaris
 +
 +
Keratosis follicularis (Darier-White)
 +
 +
''Excludes1''
 +
 +
Ehlers-Danlos syndrome (Q79.6-)
 +
 +
*'''[[Q82.9]]''' Congenital malformation of skin, unspecified
 +
 +
*'''[[Q83]]''' Congenital malformations of breast
 +
 +
''Excludes2''
 +
 +
absence of pectoral muscle (Q79.8)
 +
 +
hypoplasia of breast (N64.82)
 +
 +
micromastia (N64.82)
 +
 +
*'''[[Q83.0]]''' Congenital absence of breast with absent nipple
 +
 +
*'''[[Q83.1]]''' Accessory breast
 +
 +
Supernumerary breast
 +
 +
*'''[[Q83.2]]''' Absent nipple
 +
 +
*'''[[Q83.3]]''' Accessory nipple
 +
 +
Supernumerary nipple
 +
 +
*'''[[Q83.8]]''' Other congenital malformations of breast
 +
 +
*'''[[Q83.9]]''' Congenital malformation of breast, unspecified
 +
 +
*'''[[Q84]]''' Other congenital malformations of integument
 +
 +
*'''[[Q84.0]]''' Congenital alopecia
 +
 +
Congenital atrichosis
 +
 +
*'''[[Q84.1]]''' Congenital morphological disturbances of hair, not elsewhere classified
 +
 +
Beaded hair
 +
 +
Monilethrix
 +
 +
Pili annulati
 +
 +
''Excludes1''
 +
 +
Menkes' kinky hair syndrome (E83.0)
 +
 +
*'''[[Q84.2]]''' Other congenital malformations of hair
 +
 +
Congenital hypertrichosis
 +
 +
Congenital malformation of hair NOS
 +
 +
Persistent lanugo
 +
 +
*'''[[Q84.3]]''' Anonychia
 +
 +
''Excludes1''
 +
 +
nail patella syndrome (Q87.2)
 +
 +
*'''[[Q84.4]]''' Congenital leukonychia
 +
 +
*'''[[Q84.5]]''' Enlarged and hypertrophic nails
 +
 +
Congenital onychauxis
 +
 +
Pachyonychia
 +
 +
*'''[[Q84.6]]''' Other congenital malformations of nails
 +
 +
Congenital clubnail
 +
 +
Congenital koilonychia
 +
 +
Congenital malformation of nail NOS
 +
 +
*'''[[Q84.8]]''' Other specified congenital malformations of integument
 +
 +
Aplasia cutis congenita
 +
 +
*'''[[Q84.9]]''' Congenital malformation of integument, unspecified
 +
 +
Congenital anomaly of integument NOS
 +
 +
Congenital deformity of integument NOS
 +
 +
*'''[[Q85]]''' Phakomatoses, not elsewhere classified
 +
 +
''Excludes1''
 +
 +
ataxia telangiectasia (Louis-Bar) (G11.3)
 +
 +
familial dysautonomia (Riley-Day) (G90.1)
 +
 +
*'''[[Q85.0]]''' Neurofibromatosis (nonmalignant)
 +
 +
*'''[[Q85.00]]''' Neurofibromatosis, unspecified
 +
 +
*'''[[Q85.01]]''' Neurofibromatosis, type 1
 +
 +
Von Recklinghausen disease
 +
 +
*'''[[Q85.02]]''' Neurofibromatosis, type 2
 +
 +
Acoustic neurofibromatosis
 +
 +
*'''[[Q85.03]]''' Schwannomatosis
 +
 +
*'''[[Q85.09]]''' Other neurofibromatosis
 +
 +
*'''[[Q85.1]]''' Tuberous sclerosis
 +
 +
Bourneville's disease
 +
 +
Epiloia
 +
 +
*'''[[Q85.8]]''' Other phakomatoses, not elsewhere classified
 +
 +
Peutz-Jeghers Syndrome
 +
 +
Sturge-Weber(-Dimitri) syndrome
 +
 +
von Hippel-Lindau syndrome
 +
 +
''Excludes1''
 +
 +
Meckel-Gruber syndrome (Q61.9)
 +
 +
*'''[[Q85.9]]''' Phakomatosis, unspecified
 +
 +
Hamartosis NOS
 +
 +
*'''[[Q86]]''' Congenital malformation syndromes due to known exogenous causes, not elsewhere classified
 +
 +
''Excludes2''
 +
 +
iodine-deficiency-related hypothyroidism (E00-E02)
 +
 +
nonteratogenic effects of substances transmitted via placenta or breast milk (P04.-)
 +
 +
*'''[[Q86.0]]''' Fetal alcohol syndrome (dysmorphic)
 +
 +
*'''[[Q86.1]]''' Fetal hydantoin syndrome
 +
 +
Meadow's syndrome
 +
 +
*'''[[Q86.2]]''' Dysmorphism due to warfarin
 +
 +
*'''[[Q86.8]]''' Other congenital malformation syndromes due to known exogenous causes
 +
 +
*'''[[Q87]]''' Other specified congenital malformation syndromes affecting multiple systems ''Use additional''
 +
 +
code(s) to identify all associated manifestations
 +
 +
*'''[[Q87.0]]''' Congenital malformation syndromes predominantly affecting facial appearance
 +
 +
Acrocephalopolysyndactyly
 +
 +
Acrocephalosyndactyly (Apert)
 +
 +
Cryptophthalmos syndrome
 +
 +
Cyclopia
 +
 +
Goldenhar syndrome
 +
 +
Moebius syndrome
 +
 +
Oro-facial-digital syndrome
 +
 +
Robin syndrome
 +
 +
Whistling face
 +
 +
*'''[[Q87.1]]''' Congenital malformation syndromes predominantly associated with short stature
 +
 +
''Excludes1''
 +
 +
Ellis-van Creveld syndrome (Q77.6)
 +
 +
Smith-Lemli-Opitz syndrome (E78.72)
 +
 +
*'''[[Q87.11]]''' Prader-Willi syndrome
 +
 +
*'''[[Q87.19]]''' Other congenital malformation syndromes predominantly associated with short stature
 +
 +
Aarskog syndrome
 +
 +
Cockayne syndrome
 +
 +
De Lange syndrome
 +
 +
Dubowitz syndrome
 +
 +
Noonan syndrome
 +
 +
Robinow-Silverman-Smith syndrome
 +
 +
Russell-Silver syndrome
 +
 +
Seckel syndrome
 +
 +
*'''[[Q87.2]]''' Congenital malformation syndromes predominantly involving limbs
 +
 +
Holt-Oram syndrome
 +
 +
Klippel-Trenaunay-Weber syndrome
 +
 +
Nail patella syndrome
 +
 +
Rubinstein-Taybi syndrome
 +
 +
Sirenomelia syndrome
 +
 +
Thrombocytopenia with absent radius (TAR) syndrome
 +
 +
VATER syndrome
 +
 +
*'''[[Q87.3]]''' Congenital malformation syndromes involving early overgrowth
 +
 +
Beckwith-Wiedemann syndrome
 +
 +
Sotos syndrome
 +
 +
Weaver syndrome
 +
 +
*'''[[Q87.4]]''' Marfan's syndrome
 +
 +
*'''[[Q87.40]]''' Marfan's syndrome, unspecified
 +
 +
*'''[[Q87.41]]''' Marfan's syndrome with cardiovascular manifestations
 +
 +
*'''[[Q87.410]]''' Marfan's syndrome with aortic dilation
 +
 +
*'''[[Q87.418]]''' Marfan's syndrome with other cardiovascular manifestations
 +
 +
*'''[[Q87.42]]''' Marfan's syndrome with ocular manifestations
 +
 +
*'''[[Q87.43]]''' Marfan's syndrome with skeletal manifestation
 +
 +
*'''[[Q87.5]]''' Other congenital malformation syndromes with other skeletal changes
 +
 +
*'''[[Q87.8]]''' Other specified congenital malformation syndromes, not elsewhere classified
 +
 +
''Excludes1''
 +
 +
Zellweger syndrome (E71.510)
 +
 +
*'''[[Q87.81]]''' Alport syndrome ''Use additional''
 +
 +
code to identify stage of chronic kidney disease (N18.1-N18.6)
 +
 +
*'''[[Q87.82]]''' Arterial tortuosity syndrome
 +
 +
*'''[[Q87.89]]''' Other specified congenital malformation syndromes, not elsewhere classified
 +
 +
Laurence-Moon (-Bardet)-Biedl syndrome
 +
 +
*'''[[Q89]]''' Other congenital malformations, not elsewhere classified
 +
 +
*'''[[Q89.0]]''' Congenital absence and malformations of spleen
 +
 +
''Excludes1''
 +
 +
isomerism of atrial appendages (with asplenia or polysplenia) (Q20.6)
 +
 +
*'''[[Q89.01]]''' Asplenia (congenital)
 +
 +
*'''[[Q89.09]]''' Congenital malformations of spleen
 +
 +
Congenital splenomegaly
 +
 +
*'''[[Q89.1]]''' Congenital malformations of adrenal gland
 +
 +
''Excludes1''
 +
 +
adrenogenital disorders (E25.-)
 +
 +
congenital adrenal hyperplasia (E25.0)
 +
 +
*'''[[Q89.2]]''' Congenital malformations of other endocrine glands
 +
 +
Congenital malformation of parathyroid or thyroid gland
 +
 +
Persistent thyroglossal duct
 +
 +
Thyroglossal cyst
 +
 +
''Excludes1''
 +
 +
congenital goiter (E03.0)
 +
 +
congenital hypothyroidism (E03.1)
 +
 +
*'''[[Q89.3]]''' Situs inversus
 +
 +
Dextrocardia with situs inversus
 +
 +
Mirror-image atrial arrangement with situs inversus
 +
 +
Situs inversus or transversus abdominalis
 +
 +
Situs inversus or transversus thoracis
 +
 +
Transposition of abdominal viscera
 +
 +
Transposition of thoracic viscera
 +
 +
''Excludes1''
 +
 +
dextrocardia NOS (Q24.0)
 +
 +
*'''[[Q89.4]]''' Conjoined twins
 +
 +
Craniopagus
 +
 +
Dicephaly
 +
 +
Pygopagus
 +
 +
Thoracopagus
 +
 +
*'''[[Q89.7]]''' Multiple congenital malformations, not elsewhere classified
 +
 +
Multiple congenital anomalies NOS
 +
 +
Multiple congenital deformities NOS
 +
 +
''Excludes1''
 +
 +
congenital malformation syndromes affecting multiple systems (Q87.-)
 +
 +
*'''[[Q89.8]]''' Other specified congenital malformations ''Use additional''
 +
 +
code(s) to identify all associated manifestations
 +
 +
*'''[[Q89.9]]''' Congenital malformation, unspecified
 +
 +
Congenital anomaly NOS
 +
 +
Congenital deformity NOS
 +
 +
Chromosomal abnormalities, not elsewhere classified (Q90-Q99)
 +
 +
''Excludes2''
 +
 +
mitochondrial metabolic disorders (E88.4-)
 +
 +
*'''[[Q90]]''' Down syndrome ''Use additional''
 +
 +
code(s) to identify any associated physical conditions and degree of intellectual disabilities (F70-F79)
 +
 +
*'''[[Q90.0]]''' Trisomy 21, nonmosaicism (meiotic nondisjunction)
 +
 +
*'''[[Q90.1]]''' Trisomy 21, mosaicism (mitotic nondisjunction)
 +
 +
*'''[[Q90.2]]''' Trisomy 21, translocation
 +
 +
*'''[[Q90.9]]''' Down syndrome, unspecified
 +
 +
Trisomy 21 NOS
 +
 +
*'''[[Q91]]''' Trisomy 18 and Trisomy 13
 +
 +
*'''[[Q91.0]]''' Trisomy 18, nonmosaicism (meiotic nondisjunction)
 +
 +
*'''[[Q91.1]]''' Trisomy 18, mosaicism (mitotic nondisjunction)
 +
 +
*'''[[Q91.2]]''' Trisomy 18, translocation
 +
 +
*'''[[Q91.3]]''' Trisomy 18, unspecified
 +
 +
*'''[[Q91.4]]''' Trisomy 13, nonmosaicism (meiotic nondisjunction)
 +
 +
*'''[[Q91.5]]''' Trisomy 13, mosaicism (mitotic nondisjunction)
 +
 +
*'''[[Q91.6]]''' Trisomy 13, translocation
 +
 +
*'''[[Q91.7]]''' Trisomy 13, unspecified
 +
 +
*'''[[Q92]]''' Other trisomies and partial trisomies of the autosomes, not elsewhere classified
 +
 +
''Includes''
 +
 +
unbalanced translocations and insertions
 +
 +
''Excludes1''
 +
 +
trisomies of chromosomes 13, 18, 21 (Q90-Q91)
 +
 +
*'''[[Q92.0]]''' Whole chromosome trisomy, nonmosaicism (meiotic nondisjunction)
 +
 +
*'''[[Q92.1]]''' Whole chromosome trisomy, mosaicism (mitotic nondisjunction)
 +
 +
*'''[[Q92.2]]''' Partial trisomy
 +
 +
Less than whole arm duplicated
 +
 +
Whole arm or more duplicated
 +
 +
''Excludes1''
 +
 +
partial trisomy due to unbalanced translocation (Q92.5)
 +
 +
*'''[[Q92.5]]''' Duplications with other complex rearrangements
 +
 +
Partial trisomy due to unbalanced translocations ''Code also''
 +
 +
any associated deletions due to unbalanced translocations, inversions and insertions (Q93.7)
 +
 +
*'''[[Q92.6]]''' Marker chromosomes
 +
 +
Trisomies due to dicentrics
 +
 +
Trisomies due to extra rings
 +
 +
Trisomies due to isochromosomes
 +
 +
Individual with marker heterochromatin
 +
 +
*'''[[Q92.61]]''' Marker chromosomes in normal individual
 +
 +
*'''[[Q92.62]]''' Marker chromosomes in abnormal individual
 +
 +
*'''[[Q92.7]]''' Triploidy and polyploidy
 +
 +
*'''[[Q92.8]]''' Other specified trisomies and partial trisomies of autosomes
 +
 +
Duplications identified by fluorescence in situ hybridization (FISH)
 +
 +
Duplications identified by in situ hybridization (ISH)
 +
 +
Duplications seen only at prometaphase
 +
 +
*'''[[Q92.9]]''' Trisomy and partial trisomy of autosomes, unspecified
 +
 +
*'''[[Q93]]''' Monosomies and deletions from the autosomes, not elsewhere classified
 +
 +
*'''[[Q93.0]]''' Whole chromosome monosomy, nonmosaicism (meiotic nondisjunction)
 +
 +
*'''[[Q93.1]]''' Whole chromosome monosomy, mosaicism (mitotic nondisjunction)
 +
 +
*'''[[Q93.2]]''' Chromosome replaced with ring, dicentric or isochromosome
 +
 +
*'''[[Q93.3]]''' Deletion of short arm of chromosome 4
 +
 +
Wolff-Hirschorn syndrome
 +
 +
*'''[[Q93.4]]''' Deletion of short arm of chromosome 5
 +
 +
Cri-du-chat syndrome
 +
 +
*'''[[Q93.5]]''' Other deletions of part of a chromosome
 +
 +
*'''[[Q93.51]]''' Angelman syndrome
 +
 +
*'''[[Q93.59]]''' Other deletions of part of a chromosome
 +
 +
*'''[[Q93.7]]''' Deletions with other complex rearrangements
 +
 +
Deletions due to unbalanced translocations, inversions and insertions ''Code also''
 +
 +
any associated duplications due to unbalanced translocations, inversions and insertions (Q92.5)
 +
 +
*'''[[Q93.8]]''' Other deletions from the autosomes
 +
 +
*'''[[Q93.81]]''' Velo-cardio-facial syndrome
 +
 +
Deletion 22q11.2
 +
 +
*'''[[Q93.82]]''' Williams syndrome
 +
 +
*'''[[Q93.88]]''' Other microdeletions
 +
 +
Miller-Dieker syndrome
 +
 +
Smith-Magenis syndrome
 +
 +
*'''[[Q93.89]]''' Other deletions from the autosomes
 +
 +
Deletions identified by fluorescence in situ hybridization (FISH)
 +
 +
Deletions identified by in situ hybridization (ISH)
 +
 +
Deletions seen only at prometaphase
 +
 +
*'''[[Q93.9]]''' Deletion from autosomes, unspecified
 +
 +
*'''[[Q95]]''' Balanced rearrangements and structural markers, not elsewhere classified
 +
 +
''Includes''
 +
 +
Robertsonian and balanced reciprocal translocations and insertions
 +
 +
*'''[[Q95.0]]''' Balanced translocation and insertion in normal individual
 +
 +
*'''[[Q95.1]]''' Chromosome inversion in normal individual
 +
 +
*'''[[Q95.2]]''' Balanced autosomal rearrangement in abnormal individual
 +
 +
*'''[[Q95.3]]''' Balanced sex/autosomal rearrangement in abnormal individual
 +
 +
*'''[[Q95.5]]''' Individual with autosomal fragile site
 +
 +
*'''[[Q95.8]]''' Other balanced rearrangements and structural markers
 +
 +
*'''[[Q95.9]]''' Balanced rearrangement and structural marker, unspecified
 +
 +
*'''[[Q96]]''' Turner's syndrome
 +
 +
''Excludes1''
 +
 +
Noonan syndrome (Q87.19)
 +
 +
*'''[[Q96.0]]''' Karyotype 45, X
 +
 +
*'''[[Q96.1]]''' Karyotype 46, X iso (Xq)
 +
 +
Karyotype 46, isochromosome Xq
 +
 +
*'''[[Q96.2]]''' Karyotype 46, X with abnormal sex chromosome, except iso (Xq)
 +
 +
Karyotype 46, X with abnormal sex chromosome, except isochromosome Xq
 +
 +
*'''[[Q96.3]]''' Mosaicism, 45, X/46, XX or XY
 +
 +
*'''[[Q96.4]]''' Mosaicism, 45, X/other cell line(s) with abnormal sex chromosome
 +
 +
*'''[[Q96.8]]''' Other variants of Turner's syndrome
 +
 +
*'''[[Q96.9]]''' Turner's syndrome, unspecified
 +
 +
*'''[[Q97]]''' Other sex chromosome abnormalities, female phenotype, not elsewhere classified
 +
 +
''Excludes1''
 +
 +
Turner's syndrome (Q96.-)
 +
 +
*'''[[Q97.0]]''' Karyotype 47, XXX
 +
 +
*'''[[Q97.1]]''' Female with more than three X chromosomes
 +
 +
*'''[[Q97.2]]''' Mosaicism, lines with various numbers of X chromosomes
 +
 +
*'''[[Q97.3]]''' Female with 46, XY karyotype
 +
 +
*'''[[Q97.8]]''' Other specified sex chromosome abnormalities, female phenotype
 +
 +
*'''[[Q97.9]]''' Sex chromosome abnormality, female phenotype, unspecified
 +
 +
*'''[[Q98]]''' Other sex chromosome abnormalities, male phenotype, not elsewhere classified
 +
 +
*'''[[Q98.0]]''' Klinefelter syndrome karyotype 47, XXY
 +
 +
*'''[[Q98.1]]''' Klinefelter syndrome, male with more than two X chromosomes
 +
 +
*'''[[Q98.3]]''' Other male with 46, XX karyotype
 +
 +
*'''[[Q98.4]]''' Klinefelter syndrome, unspecified
 +
 +
*'''[[Q98.5]]''' Karyotype 47, XYY
 +
 +
*'''[[Q98.6]]''' Male with structurally abnormal sex chromosome
 +
 +
*'''[[Q98.7]]''' Male with sex chromosome mosaicism
 +
 +
*'''[[Q98.8]]''' Other specified sex chromosome abnormalities, male phenotype
 +
 +
*'''[[Q98.9]]''' Sex chromosome abnormality, male phenotype, unspecified
 +
 +
*'''[[Q99]]''' Other chromosome abnormalities, not elsewhere classified
 +
 +
*'''[[Q99.0]]''' Chimera 46, XX/46, XY
 +
 +
Chimera 46, XX/46, XY true hermaphrodite
 +
 +
*'''[[Q99.1]]''' 46, XX true hermaphrodite
 +
 +
46, XX with streak gonads
 +
 +
46, XY with streak gonads
 +
 +
Pure gonadal dysgenesis
 +
 +
*'''[[Q99.2]]''' Fragile X chromosome
 +
 +
Fragile X syndrome
 +
 +
*'''[[Q99.8]]''' Other specified chromosome abnormalities
 +
 +
*'''[[Q99.9]]''' Chromosomal abnormality, unspecified
 
{{stb}}
 
{{stb}}

Revision as of 00:28, 13 October 2019

ICD-10-CM-Chapter 17

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

Note

Codes from this chapter are not for use on maternal records

Excludes2

inborn errors of metabolism (E70-E88)

This chapter contains the following blocks

Congenital malformations of the nervous system

Congenital malformations of eye, ear, face and neck

Congenital malformations of the circulatory system

Congenital malformations of the respiratory system

Cleft lip and cleft palate

Other congenital malformations of the digestive system

Congenital malformations of genital organs

Congenital malformations of the urinary system

Congenital malformations and deformations of the musculoskeletal system

Other congenital malformations

Chromosomal abnormalities, not elsewhere classified

Congenital malformations of the nervous system (Q00-Q07)

  • Q00 Anencephaly and similar malformations

Acephaly

Acrania

Amyelencephaly

Hemianencephaly

Hemicephaly

  • Q01 Encephalocele

Includes

Arnold-Chiari syndrome, type III

encephalocystocele

encephalomyelocele

hydroencephalocele

hydromeningocele, cranial

meningocele, cerebral

meningoencephalocele

Excludes1

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

Includes

hydromicrocephaly

micrencephalon Code first

, if applicable, congenital Zika virus disease

Excludes1

Meckel-Gruber syndrome (Q61.9)

  • Q03 Congenital hydrocephalus

Includes

hydrocephalus in newborn

Excludes1

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)

  • Q03.0 Malformations of aqueduct of Sylvius

Anomaly of aqueduct of Sylvius

Obstruction of aqueduct of Sylvius, congenital

Stenosis of aqueduct of Sylvius

  • 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

Excludes1

cyclopia (Q87.0)

macrocephaly (Q75.3)

  • Q04.0 Congenital malformations of corpus callosum

Agenesis of corpus callosum

  • Q04.3 Other 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

Excludes1

congenital malformations of corpus callosum (Q04.0)

  • Q04.4 Septo-optic dysplasia of brain
  • Q04.6 Congenital cerebral cysts

Porencephaly

Schizencephaly

Excludes1

acquired porencephalic cyst (G93.0)

  • Q04.8 Other specified congenital malformations of brain

Arnold-Chiari syndrome, type IV

Macrogyria

  • Q04.9 Congenital 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

  • Q05 Spina bifida

Includes

hydromeningocele (spinal)

meningocele (spinal)

meningomyelocele

myelocele

myelomeningocele

rachischisis

spina bifida (aperta)(cystica)

syringomyelocele Use additional code for any associated

paraplegia (paraparesis) (G82.2-)

Excludes1

Arnold-Chiari syndrome, type II (Q07.0-)

spina bifida occulta (Q76.0)

  • Q05.0 Cervical spina bifida with hydrocephalus
  • Q05.1 Thoracic spina bifida with hydrocephalus

Dorsal spina bifida with hydrocephalus

Thoracolumbar spina bifida 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

Dorsal spina bifida NOS

Thoracolumbar spina bifida 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
  • Q06.1 Hypoplasia and dysplasia of spinal cord

Atelomyelia

Myelatelia

Myelodysplasia of spinal cord

  • Q06.3 Other congenital cauda equina malformations

Hydrorachis

  • Q06.8 Other specified congenital malformations of spinal cord
  • Q06.9 Congenital 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

  • Q07 Other congenital malformations of nervous system

Excludes2

congenital central alveolar hypoventilation syndrome (G47.35)

familial dysautonomia (Riley-Day) (G90.1)

neurofibromatosis (nonmalignant) (Q85.0-)

  • Q07.0 Arnold-Chiari syndrome

Arnold-Chiari syndrome, type II

Excludes1

Arnold-Chiari syndrome, type III (Q01.-)

Arnold-Chiari syndrome, type IV (Q04.8)

  • Q07.00 Arnold-Chiari syndrome without spina bifida or hydrocephalus
  • Q07.01 Arnold-Chiari syndrome with spina bifida
  • Q07.02 Arnold-Chiari syndrome with hydrocephalus
  • Q07.03 Arnold-Chiari syndrome with spina bifida and hydrocephalus
  • 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 NOS of nervous system

Congenital deformity NOS of nervous system

Congenital disease or lesion NOS of nervous system

Congenital malformations of eye, ear, face and neck (Q10-Q18)

Excludes2

cleft lip and cleft palate (Q35-Q37)

congenital malformation of cervical spine (Q05.0, Q05.5, Q67.5, Q76.0-Q76.4)

congenital malformation of larynx (Q31.-)

congenital malformation of lip NEC (Q38.0)

congenital malformation of nose (Q30.-)

congenital malformation of parathyroid gland (Q89.2)

congenital malformation of thyroid gland (Q89.2)

  • Q10 Congenital malformations of eyelid, lacrimal apparatus and orbit

Excludes1

cryptophthalmos NOS (Q11.2)

cryptophthalmos syndrome (Q87.0)

  • Q10.1 Congenital ectropion
  • Q10.2 Congenital entropion
  • Q10.3 Other congenital malformations of eyelid

Ablepharon

Blepharophimosis, congenital

Coloboma of eyelid

Congenital absence or agenesis of cilia

Congenital absence or agenesis of eyelid

Congenital accessory eyelid

Congenital accessory eye muscle

Congenital malformation of eyelid NOS

  • Q10.4 Absence and agenesis of lacrimal apparatus

Congenital absence of punctum lacrimale

  • Q10.5 Congenital stenosis and stricture of lacrimal duct
  • Q10.6 Other congenital malformations of lacrimal apparatus

Congenital malformation of lacrimal apparatus NOS

  • Q10.7 Congenital malformation of orbit
  • Q11 Anophthalmos, microphthalmos and macrophthalmos

Anophthalmos NOS

Agenesis of eye

Aplasia of eye

Cryptophthalmos NOS

Dysplasia of eye

Hypoplasia of eye

Rudimentary eye

Excludes1

cryptophthalmos syndrome (Q87.0)

Excludes1

macrophthalmos in congenital glaucoma (Q15.0)

  • Q12 Congenital lens malformations
  • Q12.0 Congenital cataract
  • Q12.1 Congenital displaced lens
  • Q12.8 Other congenital lens malformations

Microphakia

  • Q12.9 Congenital lens malformation, unspecified
  • Q13 Congenital malformations of anterior segment of eye

Coloboma NOS

Aniridia Use additional

code for associated glaucoma (H42)

  • Q13.2 Other congenital malformations of iris

Anisocoria, congenital

Atresia of pupil

Congenital malformation of iris NOS

Corectopia

  • Q13.3 Congenital corneal opacity
  • Q13.4 Other congenital corneal malformations

Congenital malformation of cornea NOS

Microcornea

Peter's anomaly

  • Q13.8 Other congenital malformations of anterior segment of eye
  • Q13.81 Rieger's anomaly Use additional

code for associated glaucoma (H42)

  • Q13.89 Other congenital malformations of anterior segment of eye
  • Q13.9 Congenital malformation of anterior segment of eye, unspecified
  • Q14 Congenital malformations of posterior segment of eye

Excludes2

optic nerve hypoplasia (H47.03-)

  • Q14.0 Congenital malformation of vitreous humor

Congenital vitreous opacity

  • Q14.1 Congenital malformation of retina

Congenital retinal aneurysm

  • Q14.2 Congenital malformation of optic disc

Coloboma of optic disc

  • Q14.3 Congenital malformation of choroid
  • Q14.8 Other congenital malformations of posterior segment of eye

Coloboma of the fundus

  • Q14.9 Congenital malformation of posterior segment of eye, unspecified
  • Q15 Other congenital malformations of eye

Excludes1

congenital nystagmus (H55.01)

ocular albinism (E70.31-)

optic nerve hypoplasia (H47.03-)

retinitis pigmentosa (H35.52)

  • Q15.0 Congenital glaucoma

Axenfeld's anomaly

Buphthalmos

Glaucoma of childhood

Glaucoma of newborn

Hydrophthalmos

Keratoglobus, congenital, with glaucoma

Macrocornea with glaucoma

Macrophthalmos in congenital glaucoma

Megalocornea with glaucoma

  • Q15.8 Other specified congenital malformations of eye
  • Q15.9 Congenital malformation of eye, unspecified

Congenital anomaly of eye

Congenital deformity of eye

  • Q16 Congenital malformations of ear causing impairment of hearing

Excludes1

congenital deafness (H90.-)

  • Q16.0 Congenital absence of (ear) auricle
  • Q16.1 Congenital absence, atresia and stricture of auditory canal (external)

Congenital atresia or stricture of osseous meatus

  • Q16.2 Absence of eustachian tube
  • Q16.3 Congenital malformation of ear ossicles

Congenital fusion of ear ossicles

  • Q16.4 Other congenital malformations of middle ear

Congenital malformation of middle ear NOS

  • Q16.5 Congenital malformation of inner ear

Congenital anomaly of membranous labyrinth

Congenital anomaly of organ of Corti

  • Q16.9 Congenital malformation of ear causing impairment of hearing, unspecified

Congenital absence of ear NOS

  • Q17 Other congenital malformations of ear

Excludes1

congenital malformations of ear with impairment of hearing (Q16.0-Q16.9)

preauricular sinus (Q18.1)

Accessory tragus

Polyotia

Preauricular appendage or tag

Supernumerary ear

Supernumerary lobule

  • Q17.3 Other misshapen ear

Pointed ear

Low-set ears

Excludes1

cervical auricle (Q18.2)

Bat ear

  • Q17.8 Other specified congenital malformations of ear

Congenital absence of lobe of ear

  • Q17.9 Congenital malformation of ear, unspecified

Congenital anomaly of ear NOS

  • Q18 Other congenital malformations of face and neck

Excludes1

cleft lip and cleft palate (Q35-Q37)

conditions classified to Q67.0-Q67.4

congenital malformations of skull and face bones (Q75.-)

cyclopia (Q87.0)

dentofacial anomalies (including malocclusion) (M26.-)

malformation syndromes affecting facial appearance (Q87.0)

persistent thyroglossal duct (Q89.2)

  • Q18.0 Sinus, fistula and cyst of branchial cleft

Branchial vestige

  • Q18.1 Preauricular sinus and cyst

Fistula of auricle, congenital

Cervicoaural fistula

  • Q18.2 Other branchial cleft malformations

Branchial cleft malformation NOS

Cervical auricle

Otocephaly

Pterygium colli

Hypertrophy of lip, congenital

  • Q18.8 Other specified congenital malformations of face and neck

Medial cyst of face and neck

Medial fistula of face and neck

Medial sinus of face and neck

  • Q18.9 Congenital malformation of face and neck, unspecified

Congenital anomaly NOS of face and neck

Congenital malformations of the circulatory system (Q20-Q28)

  • Q20 Congenital malformations of cardiac chambers and connections

Excludes1

dextrocardia with situs inversus (Q89.3)

mirror-image atrial arrangement with situs inversus (Q89.3)

  • Q20.0 Common arterial trunk

Persistent truncus arteriosus

Excludes1

aortic septal defect (Q21.4)

  • Q20.1 Double outlet right ventricle

Taussig-Bing syndrome

  • Q20.2 Double outlet left ventricle
  • Q20.3 Discordant ventriculoarterial connection

Dextrotransposition of aorta

Transposition of great vessels (complete)

  • Q20.4 Double inlet ventricle

Common ventricle

Cor triloculare biatriatum

Single ventricle

  • Q20.5 Discordant atrioventricular connection

Corrected transposition

Levotransposition

Ventricular inversion

  • Q20.6 Isomerism of atrial appendages

Isomerism of atrial appendages with asplenia or polysplenia

  • Q20.8 Other congenital malformations of cardiac chambers and connections

Cor binoculare

  • Q20.9 Congenital malformation of cardiac chambers and connections, unspecified
  • Q21 Congenital malformations of cardiac septa

Excludes1

acquired cardiac septal defect (I51.0)

  • Q21.0 Ventricular septal defect

Roger's disease

  • Q21.1 Atrial septal defect

Coronary sinus defect

Patent or persistent foramen ovale

Patent or persistent ostium secundum defect (type II)

Patent or persistent sinus venosus defect

  • Q21.2 Atrioventricular septal defect

Common atrioventricular canal

Endocardial cushion defect

Ostium primum atrial septal defect (type I)

  • Q21.3 Tetralogy of Fallot

Ventricular septal defect with pulmonary stenosis or atresia, dextroposition of aorta and hypertrophy of right

ventricle

  • Q21.4 Aortopulmonary septal defect

Aortic septal defect

Aortopulmonary window

  • Q21.8 Other congenital malformations of cardiac septa

Eisenmenger's defect

Pentalogy of Fallot Code also

if applicable

Eisenmenger's complex (I27.83)

Eisenmenger's syndrome (I27.83)

  • Q21.9 Congenital malformation of cardiac septum, unspecified

Septal (heart) defect NOS

  • Q22 Congenital malformations of pulmonary and tricuspid valves
  • Q22.0 Pulmonary valve atresia
  • Q22.1 Congenital pulmonary valve stenosis
  • Q22.2 Congenital pulmonary valve insufficiency

Congenital pulmonary valve regurgitation

  • Q22.3 Other congenital malformations of pulmonary valve

Congenital malformation of pulmonary valve NOS

Supernumerary cusps of pulmonary valve

  • Q22.4 Congenital tricuspid stenosis

Congenital tricuspid atresia

  • Q22.6 Hypoplastic right heart syndrome
  • Q22.8 Other congenital malformations of tricuspid valve
  • Q22.9 Congenital malformation of tricuspid valve, unspecified
  • Q23 Congenital malformations of aortic and mitral valves
  • Q23.0 Congenital stenosis of aortic valve

Congenital aortic atresia

Congenital aortic stenosis NOS

Excludes1

congenital stenosis of aortic valve in hypoplastic left heart syndrome (Q23.4)

congenital subaortic stenosis (Q24.4)

supravalvular aortic stenosis (congenital) (Q25.3)

  • Q23.1 Congenital insufficiency of aortic valve

Bicuspid aortic valve

Congenital aortic insufficiency

  • Q23.2 Congenital mitral stenosis

Congenital mitral atresia

  • Q23.3 Congenital mitral insufficiency
  • Q23.4 Hypoplastic left heart syndrome
  • Q23.8 Other congenital malformations of aortic and mitral valves
  • Q23.9 Congenital malformation of aortic and mitral valves, unspecified
  • Q24 Other congenital malformations of heart

Excludes1

endocardial fibroelastosis (I42.4)

Excludes1

dextrocardia with situs inversus (Q89.3)

isomerism of atrial appendages (with asplenia or polysplenia) (Q20.6)

mirror-image atrial arrangement with situs inversus (Q89.3)

  • Q24.3 Pulmonary infundibular stenosis

Subvalvular pulmonic stenosis

  • Q24.4 Congenital subaortic stenosis
  • Q24.5 Malformation of coronary vessels

Congenital coronary (artery) aneurysm

  • Q24.6 Congenital heart block
  • Q24.8 Other specified congenital malformations of heart

Congenital diverticulum of left ventricle

Congenital malformation of myocardium

Congenital malformation of pericardium

Malposition of heart

Uhl's disease

  • Q24.9 Congenital malformation of heart, unspecified

Congenital anomaly of heart

Congenital disease of heart

  • Q25 Congenital malformations of great arteries
  • Q25.0 Patent ductus arteriosus

Patent ductus Botallo

Persistent ductus arteriosus

  • Q25.1 Coarctation of aorta

Coarctation of aorta (preductal) (postductal)

Stenosis of aorta

  • Q25.21 Interruption of aortic arch

Atresia of aortic arch

Atresia of aorta

  • Q25.3 Supravalvular aortic stenosis

Excludes1

congenital aortic stenosis NOS (Q23.0)

congenital stenosis of aortic valve (Q23.0)

  • Q25.4 Other congenital malformations of aorta

Excludes1

hypoplasia of aorta in hypoplastic left heart syndrome (Q23.4)

  • Q25.40 Congenital malformation of aorta unspecified
  • Q25.41 Absence and aplasia of aorta
  • Q25.43 Congenital aneurysm of aorta

Congenital aneurysm of aortic root

Congenital aneurysm of aortic sinus

  • Q25.44 Congenital dilation of aorta

Vascular ring of aorta

Persistent convolutions of aortic arch

Persistent right aortic arch

  • Q25.48 Anomalous origin of subclavian artery
  • Q25.49 Other congenital malformations of aorta

Aortic arch

Bovine arch

  • Q25.5 Atresia of pulmonary artery
  • Q25.6 Stenosis of pulmonary artery

Supravalvular pulmonary stenosis

  • Q25.7 Other congenital malformations of pulmonary artery
  • Q25.71 Coarctation of pulmonary artery
  • Q25.72 Congenital pulmonary arteriovenous malformation

Congenital pulmonary arteriovenous aneurysm

  • Q25.79 Other congenital malformations of pulmonary artery

Aberrant pulmonary artery

Agenesis of pulmonary artery

Congenital aneurysm of pulmonary artery

Congenital anomaly of pulmonary artery

Hypoplasia of pulmonary artery

  • Q25.8 Other congenital malformations of other great arteries
  • Q25.9 Congenital malformation of great arteries, unspecified
  • Q26 Congenital malformations of great veins
  • Q26.0 Congenital stenosis of vena cava

Congenital stenosis of vena cava (inferior)(superior)

  • Q26.1 Persistent left superior vena cava
  • Q26.2 Total anomalous pulmonary venous connection

Total anomalous pulmonary venous return (TAPVR), subdiaphragmatic

Total anomalous pulmonary venous return (TAPVR), supradiaphragmatic

  • Q26.3 Partial anomalous pulmonary venous connection

Partial anomalous pulmonary venous return

  • Q26.4 Anomalous pulmonary venous connection, unspecified
  • Q26.5 Anomalous portal venous connection
  • Q26.6 Portal vein-hepatic artery fistula
  • Q26.8 Other congenital malformations of great veins

Absence of vena cava (inferior) (superior)

Azygos continuation of inferior vena cava

Persistent left posterior cardinal vein

Scimitar syndrome

  • Q26.9 Congenital malformation of great vein, unspecified

Congenital anomaly of vena cava (inferior) (superior) NOS

  • Q27 Other congenital malformations of peripheral vascular system

Excludes2

anomalies of cerebral and precerebral vessels (Q28.0-Q28.3)

anomalies of coronary vessels (Q24.5)

anomalies of pulmonary artery (Q25.5-Q25.7)

congenital retinal aneurysm (Q14.1)

hemangioma and lymphangioma (D18.-)

  • Q27.0 Congenital absence and hypoplasia of umbilical artery

Single umbilical artery

  • Q27.1 Congenital renal artery stenosis
  • Q27.2 Other congenital malformations of renal artery

Congenital malformation of renal artery NOS

Multiple renal arteries

  • Q27.3 Arteriovenous malformation (peripheral)

Arteriovenous aneurysm

Excludes1

acquired arteriovenous aneurysm (I77.0)

Excludes2

arteriovenous malformation of cerebral vessels (Q28.2)

arteriovenous malformation of precerebral vessels (Q28.0)

  • Q27.30 Arteriovenous malformation, site unspecified
  • Q27.31 Arteriovenous malformation of vessel of upper limb
  • Q27.32 Arteriovenous malformation of vessel of lower limb
  • Q27.33 Arteriovenous malformation of digestive system vessel
  • Q27.34 Arteriovenous malformation of renal vessel
  • Q27.39 Arteriovenous malformation, other site
  • Q27.4 Congenital phlebectasia
  • Q27.8 Other specified congenital malformations of peripheral vascular system

Absence of peripheral vascular system

Atresia of peripheral vascular system

Congenital aneurysm (peripheral)

Congenital stricture, artery

Congenital varix

Excludes1

arteriovenous malformation (Q27.3-)

  • Q27.9 Congenital malformation of peripheral vascular system, unspecified

Anomaly of artery or vein NOS

  • Q28 Other congenital malformations of circulatory system

Excludes1

congenital aneurysm NOS (Q27.8)

congenital coronary aneurysm (Q24.5)

ruptured cerebral arteriovenous malformation (I60.8)

ruptured malformation of precerebral vessels (I72.0)

Excludes2

congenital peripheral aneurysm (Q27.8)

congenital pulmonary aneurysm (Q25.79)

congenital retinal aneurysm (Q14.1)

  • Q28.0 Arteriovenous malformation of precerebral vessels

Congenital arteriovenous precerebral aneurysm (nonruptured)

  • Q28.1 Other malformations of precerebral vessels

Congenital malformation of precerebral vessels NOS

Congenital precerebral aneurysm (nonruptured)

  • Q28.2 Arteriovenous malformation of cerebral vessels

Arteriovenous malformation of brain NOS

Congenital arteriovenous cerebral aneurysm (nonruptured)

  • Q28.3 Other malformations of cerebral vessels

Congenital cerebral aneurysm (nonruptured)

Congenital malformation of cerebral vessels NOS

Developmental venous anomaly

  • Q28.8 Other specified congenital malformations of circulatory system

Congenital aneurysm, specified site NEC

Spinal vessel anomaly

  • Q28.9 Congenital malformation of circulatory system, unspecified

Congenital malformations of the respiratory system (Q30-Q34)

  • Q30 Congenital malformations of nose

Excludes1

congenital deviation of nasal septum (Q67.4)

Atresia of nares (anterior) (posterior)

Congenital stenosis of nares (anterior) (posterior)

  • Q30.1 Agenesis and underdevelopment of nose

Congenital absent of nose

  • Q30.2 Fissured, notched and cleft nose
  • Q30.3 Congenital perforated nasal septum
  • Q30.8 Other congenital malformations of nose

Accessory nose

Congenital anomaly of nasal sinus wall

  • Q30.9 Congenital malformation of nose, unspecified
  • Q31 Congenital malformations of larynx

Excludes1

congenital laryngeal stridor NOS (P28.89)

Glottic web of larynx

Subglottic web of larynx

Web of larynx NOS

  • Q31.1 Congenital subglottic stenosis
  • Q31.2 Laryngeal hypoplasia
  • Q31.5 Congenital laryngomalacia
  • Q31.8 Other congenital malformations of larynx

Absence of larynx

Agenesis of larynx

Atresia of larynx

Congenital cleft thyroid cartilage

Congenital fissure of epiglottis

Congenital stenosis of larynx NEC

Posterior cleft of cricoid cartilage

  • Q31.9 Congenital malformation of larynx, unspecified
  • Q32 Congenital malformations of trachea and bronchus

Excludes1

congenital bronchiectasis (Q33.4)

  • Q32.0 Congenital tracheomalacia
  • Q32.1 Other congenital malformations of trachea

Atresia of trachea

Congenital anomaly of tracheal cartilage

Congenital dilatation of trachea

Congenital malformation of trachea

Congenital stenosis of trachea

Congenital tracheocele

  • Q32.2 Congenital bronchomalacia
  • Q32.3 Congenital stenosis of bronchus
  • Q32.4 Other congenital malformations of bronchus

Absence of bronchus

Agenesis of bronchus

Atresia of bronchus

Congenital diverticulum of bronchus

Congenital malformation of bronchus NOS

  • Q33 Congenital malformations of lung
  • Q33.0 Congenital cystic lung

Congenital cystic lung disease

Congenital honeycomb lung

Congenital polycystic lung disease

Excludes1

cystic fibrosis (E84.0)

cystic lung disease, acquired or unspecified (J98.4)

  • Q33.1 Accessory lobe of lung

Azygos lobe (fissured), lung

  • Q33.2 Sequestration of lung

Congenital absence of lung (lobe)

  • Q33.4 Congenital bronchiectasis
  • Q33.5 Ectopic tissue in lung
  • Q33.6 Congenital hypoplasia and dysplasia of lung

Excludes1

pulmonary hypoplasia associated with short gestation (P28.0)

  • Q33.8 Other congenital malformations of lung
  • Q33.9 Congenital malformation of lung, unspecified
  • Q34 Other congenital malformations of respiratory system

Excludes2

congenital central alveolar hypoventilation syndrome (G47.35)

  • Q34.1 Congenital cyst of mediastinum
  • Q34.8 Other specified congenital malformations of respiratory system

Atresia of nasopharynx

  • Q34.9 Congenital malformation of respiratory system, unspecified

Congenital absence of respiratory system

Congenital anomaly of respiratory system NOS

Cleft lip and cleft palate (Q35-Q37) Use additional

code to identify associated malformation of the nose (Q30.2)

Excludes1

Robin's syndrome (Q87.0)

  • Q35 Cleft palate

Includes

fissure of palate

palatoschisis

Excludes1

cleft palate with cleft lip (Q37.-)

  • Q35.5 Cleft hard palate with cleft soft palate
  • Q35.9 Cleft palate, unspecified

Cleft palate NOS

Includes

cheiloschisis

congenital fissure of lip

harelip

labium leporinum

Excludes1

cleft lip with cleft palate (Q37.-)

  • Q36.0 Cleft lip, bilateral
  • Q36.9 Cleft lip, unilateral

Cleft lip NOS

  • Q37 Cleft palate with cleft lip

Includes

cheilopalatoschisis

  • Q37.0 Cleft hard palate with bilateral cleft lip
  • Q37.1 Cleft hard palate with unilateral cleft lip

Cleft hard palate with cleft lip NOS

  • Q37.2 Cleft soft palate with bilateral cleft lip
  • Q37.3 Cleft soft palate with unilateral cleft lip

Cleft soft palate with cleft lip NOS

  • Q37.4 Cleft hard and soft palate with bilateral cleft lip
  • Q37.5 Cleft hard and soft palate with unilateral cleft lip

Cleft hard and soft palate with cleft lip NOS

  • Q37.8 Unspecified cleft palate with bilateral cleft lip
  • Q37.9 Unspecified cleft palate with unilateral cleft lip

Cleft palate with cleft lip NOS

Other congenital malformations of the digestive system (Q38-Q45)

  • Q38 Other congenital malformations of tongue, mouth and pharynx

Excludes1

dentofacial anomalies (M26.-)

macrostomia (Q18.4)

microstomia (Q18.5)

  • Q38.0 Congenital malformations of lips, not elsewhere classified

Congenital fistula of lip

Congenital malformation of lip NOS

Van der Woude's syndrome

Excludes1

cleft lip (Q36.-)

cleft lip with cleft palate (Q37.-)

macrocheilia (Q18.6)

microcheilia (Q18.7)

Tongue tie

Congenital hypertrophy of tongue

  • Q38.3 Other congenital malformations of tongue

Aglossia

Bifid tongue

Congenital adhesion of tongue

Congenital fissure of tongue

Congenital malformation of tongue NOS

Double tongue

Hypoglossia

Hypoplasia of tongue

Microglossia

  • Q38.4 Congenital malformations of salivary glands and ducts

Atresia of salivary glands and ducts

Congenital absence of salivary glands and ducts

Congenital accessory salivary glands and ducts

Congenital fistula of salivary gland

  • Q38.5 Congenital malformations of palate, not elsewhere classified

Congenital absence of uvula

Congenital malformation of palate NOS

Congenital high arched palate

Excludes1

cleft palate (Q35.-)

cleft palate with cleft lip (Q37.-)

  • Q38.6 Other congenital malformations of mouth

Congenital malformation of mouth NOS

  • Q38.7 Congenital pharyngeal pouch

Congenital diverticulum of pharynx

Excludes1

pharyngeal pouch syndrome (D82.1)

  • Q38.8 Other congenital malformations of pharynx

Congenital malformation of pharynx NOS

Imperforate pharynx

  • Q39 Congenital malformations of esophagus
  • Q39.0 Atresia of esophagus without fistula

Atresia of esophagus NOS

  • Q39.1 Atresia of esophagus with tracheo-esophageal fistula

Atresia of esophagus with broncho-esophageal fistula

  • Q39.2 Congenital tracheo-esophageal fistula without atresia

Congenital tracheo-esophageal fistula NOS

  • Q39.3 Congenital stenosis and stricture of esophagus
  • Q39.5 Congenital dilatation of esophagus

Congenital cardiospasm

  • Q39.6 Congenital diverticulum of esophagus

Congenital esophageal pouch

  • Q39.8 Other congenital malformations of esophagus

Congenital absence of esophagus

Congenital displacement of esophagus

Congenital duplication of esophagus

  • Q39.9 Congenital malformation of esophagus, unspecified
  • Q40 Other congenital malformations of upper alimentary tract
  • Q40.0 Congenital hypertrophic pyloric stenosis

Congenital or infantile constriction

Congenital or infantile hypertrophy

Congenital or infantile spasm

Congenital or infantile stenosis

Congenital or infantile stricture

  • Q40.1 Congenital hiatus hernia

Congenital displacement of cardia through esophageal hiatus

Excludes1

congenital diaphragmatic hernia (Q79.0)

  • Q40.2 Other specified congenital malformations of stomach

Congenital displacement of stomach

Congenital diverticulum of stomach

Congenital hourglass stomach

Congenital duplication of stomach

Megalogastria

Microgastria

  • Q40.3 Congenital malformation of stomach, unspecified
  • Q40.8 Other specified congenital malformations of upper alimentary tract
  • Q40.9 Congenital malformation of upper alimentary tract, unspecified

Congenital anomaly of upper alimentary tract

Congenital deformity of upper alimentary tract

  • Q41 Congenital absence, atresia and stenosis of small intestine

Includes

congenital obstruction, occlusion or stricture of small intestine or intestine NOS

Excludes1

cystic fibrosis with intestinal manifestation (E84.11)

meconium ileus NOS (without cystic fibrosis) (P76.0)

  • Q41.0 Congenital absence, atresia and stenosis of duodenum
  • Q41.1 Congenital absence, atresia and stenosis of jejunum

Apple peel syndrome

Imperforate jejunum

  • Q41.2 Congenital absence, atresia and stenosis of ileum
  • Q41.8 Congenital absence, atresia and stenosis of other specified parts of small intestine
  • Q41.9 Congenital absence, atresia and stenosis of small intestine, part unspecified

Congenital absence, atresia and stenosis of intestine NOS

  • Q42 Congenital absence, atresia and stenosis of large intestine

Includes

congenital obstruction, occlusion and stricture of large intestine

  • Q42.0 Congenital absence, atresia and stenosis of rectum with fistula
  • Q42.1 Congenital absence, atresia and stenosis of rectum without fistula

Imperforate rectum

  • Q42.2 Congenital absence, atresia and stenosis of anus with fistula
  • Q42.3 Congenital absence, atresia and stenosis of anus without fistula

Imperforate anus

  • Q42.8 Congenital absence, atresia and stenosis of other parts of large intestine
  • Q42.9 Congenital absence, atresia and stenosis of large intestine, part unspecified
  • Q43 Other congenital malformations of intestine
  • Q43.0 Meckel's diverticulum (displaced) (hypertrophic)

Persistent omphalomesenteric duct

Persistent vitelline duct

  • Q43.1 Hirschsprung's disease

Aganglionosis

Congenital (aganglionic) megacolon

  • Q43.2 Other congenital functional disorders of colon

Congenital dilatation of colon

  • Q43.3 Congenital malformations of intestinal fixation

Congenital omental, anomalous adhesions (bands)

Congenital peritoneal adhesions (bands)

Incomplete rotation of cecum and colon

Insufficient rotation of cecum and colon

Jackson's membrane

Malrotation of colon

Rotation failure of cecum and colon

Universal mesentery

  • Q43.4 Duplication of intestine
  • Q43.6 Congenital fistula of rectum and anus

Excludes1

congenital fistula of anus with absence, atresia and stenosis (Q42.2)

congenital fistula of rectum with absence, atresia and stenosis (Q42.0)

congenital rectovaginal fistula (Q52.2)

congenital urethrorectal fistula (Q64.73)

pilonidal fistula or sinus (L05.-)

Cloaca NOS

  • Q43.8 Other specified congenital malformations of intestine

Congenital blind loop syndrome

Congenital diverticulitis, colon

Congenital diverticulum, intestine

Dolichocolon

Megaloappendix

Megaloduodenum

Microcolon

Transposition of appendix

Transposition of colon

Transposition of intestine

  • Q43.9 Congenital malformation of intestine, unspecified
  • Q44 Congenital malformations of gallbladder, bile ducts and liver
  • Q44.0 Agenesis, aplasia and hypoplasia of gallbladder

Congenital absence of gallbladder

  • Q44.1 Other congenital malformations of gallbladder

Congenital malformation of gallbladder NOS

Intrahepatic gallbladder

  • Q44.2 Atresia of bile ducts
  • Q44.3 Congenital stenosis and stricture of bile ducts
  • Q44.5 Other congenital malformations of bile ducts

Accessory hepatic duct

Biliary duct duplication

Congenital malformation of bile duct NOS

Cystic duct duplication

  • Q44.6 Cystic disease of liver

Fibrocystic disease of liver

  • Q44.7 Other congenital malformations of liver

Accessory liver

Alagille's syndrome

Congenital absence of liver

Congenital hepatomegaly

Congenital malformation of liver NOS

  • Q45 Other congenital malformations of digestive system

Excludes2

congenital diaphragmatic hernia (Q79.0)

congenital hiatus hernia (Q40.1)

  • Q45.0 Agenesis, aplasia and hypoplasia of pancreas

Congenital absence of pancreas

  • Q45.2 Congenital pancreatic cyst
  • Q45.3 Other congenital malformations of pancreas and pancreatic duct

Accessory pancreas

Congenital malformation of pancreas or pancreatic duct NOS

Excludes1

congenital diabetes mellitus (E10.-)

cystic fibrosis (E84.0-E84.9)

fibrocystic disease of pancreas (E84.-)

neonatal diabetes mellitus (P70.2)

  • Q45.8 Other specified congenital malformations of digestive system

Absence (complete) (partial) of alimentary tract NOS

Duplication of digestive system

Malposition, congenital of digestive system

  • Q45.9 Congenital malformation of digestive system, unspecified

Congenital anomaly of digestive system

Congenital deformity of digestive system

Congenital malformations of genital organs (Q50-Q56)

Excludes1

androgen insensitivity syndrome (E34.5-)

syndromes associated with anomalies in the number and form of chromosomes (Q90-Q99)

  • Q50 Congenital malformations of ovaries, fallopian tubes and broad ligaments
  • Q50.0 Congenital absence of ovary

Excludes1

Turner's syndrome (Q96.-)

  • Q50.01 Congenital absence of ovary, unilateral
  • Q50.02 Congenital absence of ovary, bilateral
  • Q50.1 Developmental ovarian cyst
  • Q50.2 Congenital torsion of ovary
  • Q50.3 Other congenital malformations of ovary

46, XX with streak gonads

  • Q50.39 Other congenital malformation of ovary

Congenital malformation of ovary NOS

  • Q50.4 Embryonic cyst of fallopian tube

Fimbrial cyst

  • Q50.5 Embryonic cyst of broad ligament

Epoophoron cyst

Parovarian cyst

  • Q50.6 Other congenital malformations of fallopian tube and broad ligament

Absence of fallopian tube and broad ligament

Accessory fallopian tube and broad ligament

Atresia of fallopian tube and broad ligament

Congenital malformation of fallopian tube or broad ligament NOS

  • Q51 Congenital malformations of uterus and cervix
  • Q51.0 Agenesis and aplasia of uterus

Congenital absence of uterus

  • Q51.1 Doubling of uterus with doubling of cervix and vagina
  • Q51.10 Doubling of uterus with doubling of cervix and vagina without obstruction

Doubling of uterus with doubling of cervix and vagina NOS

  • Q51.11 Doubling of uterus with doubling of cervix and vagina with obstruction
  • Q51.2 Other doubling of uterus

Doubling of uterus NOS

Septate uterus

  • Q51.20 Other doubling of uterus, unspecified

Septate uterus, unspecified

  • Q51.21 Other complete doubling of uterus

Complete septate uterus

  • Q51.22 Other partial doubling of uterus

Partial septate uterus

  • Q51.28 Other doubling of uterus, other specified

Septate uterus, other specified

Bicornate uterus, complete or partial

Unicornate uterus with or without a separate uterine horn

Uterus with only one functioning horn

  • Q51.5 Agenesis and aplasia of cervix

Congenital absence of cervix

  • Q51.6 Embryonic cyst of cervix
  • Q51.7 Congenital fistulae between uterus and digestive and urinary tracts
  • Q51.8 Other congenital malformations of uterus and cervix
  • Q51.81 Other congenital malformations of uterus

Arcuatus uterus

  • Q51.818 Other congenital malformations of uterus

Müllerian anomaly of uterus NEC

  • Q51.82 Other congenital malformations of cervix
  • Q51.828 Other congenital malformations of cervix
  • Q51.9 Congenital malformation of uterus and cervix, unspecified
  • Q52 Other congenital malformations of female genitalia
  • Q52.0 Congenital absence of vagina

Vaginal agenesis, total or partial

Excludes1

doubling of vagina with doubling of uterus and cervix (Q51.1-)

  • Q52.10 Doubling of vagina, unspecified

Septate vagina NOS

  • Q52.11 Transverse vaginal septum
  • Q52.12 Longitudinal vaginal septum
  • Q52.120 Longitudinal vaginal septum, nonobstructing
  • Q52.121 Longitudinal vaginal septum, obstructing, right side
  • Q52.122 Longitudinal vaginal septum, obstructing, left side
  • Q52.123 Longitudinal vaginal septum, microperforate, right side
  • Q52.124 Longitudinal vaginal septum, microperforate, left side
  • Q52.129 Other and unspecified longitudinal vaginal septum
  • Q52.2 Congenital rectovaginal fistula

Excludes1

cloaca (Q43.7)

  • Q52.4 Other congenital malformations of vagina

Canal of Nuck cyst, congenital

Congenital malformation of vagina NOS

Embryonic vaginal cyst

Gartner's duct cyst

  • Q52.6 Congenital malformation of clitoris
  • Q52.7 Other and unspecified congenital malformations of vulva
  • Q52.70 Unspecified congenital malformations of vulva

Congenital malformation of vulva NOS

  • Q52.71 Congenital absence of vulva
  • Q52.79 Other congenital malformations of vulva

Congenital cyst of vulva

  • Q52.8 Other specified congenital malformations of female genitalia
  • Q52.9 Congenital malformation of female genitalia, unspecified
  • Q53 Undescended and ectopic testicle
  • Q53.00 Ectopic testis, unspecified
  • Q53.01 Ectopic testis, unilateral
  • Q53.02 Ectopic testes, bilateral
  • Q53.1 Undescended testicle, unilateral
  • Q53.10 Unspecified undescended testicle, unilateral
  • Q53.11 Abdominal testis, unilateral
  • Q53.111 Unilateral intraabdominal testis
  • Q53.12 Ectopic perineal testis, unilateral
  • Q53.13 Unilateral high scrotal testis
  • Q53.2 Undescended testicle, bilateral
  • Q53.20 Undescended testicle, unspecified, bilateral
  • Q53.21 Abdominal testis, bilateral
  • Q53.211 Bilateral intraabdominal testes
  • Q53.22 Ectopic perineal testis, bilateral
  • Q53.23 Bilateral high scrotal testes
  • Q53.9 Undescended testicle, unspecified

Cryptorchism NOS

  • Q54 Hypospadias

Excludes1

epispadias (Q64.0)

  • Q54.0 Hypospadias, balanic

Hypospadias, coronal

Hypospadias, glandular

  • Q54.1 Hypospadias, penile
  • Q54.2 Hypospadias, penoscrotal
  • Q54.3 Hypospadias, perineal

Chordee without hypospadias

Hypospadias with intersex state

  • Q54.9 Hypospadias, unspecified
  • Q55 Other congenital malformations of male genital organs

Excludes1

congenital hydrocele (P83.5)

hypospadias (Q54.-)

  • Q55.0 Absence and aplasia of testis

Monorchism

  • Q55.1 Hypoplasia of testis and scrotum

Fusion of testes

  • Q55.2 Other and unspecified congenital malformations of testis and scrotum
  • Q55.20 Unspecified congenital malformations of testis and scrotum

Congenital malformation of testis or scrotum NOS

  • Q55.29 Other congenital malformations of testis and scrotum
  • Q55.3 Atresia of vas deferens Code first

any associated cystic fibrosis (E84.-)

  • Q55.4 Other congenital malformations of vas deferens, epididymis, seminal vesicles and prostate

Absence or aplasia of prostate

Absence or aplasia of spermatic cord

Congenital malformation of vas deferens, epididymis, seminal vesicles or prostate NOS

  • Q55.5 Congenital absence and aplasia of penis
  • Q55.6 Other congenital malformations of penis
  • Q55.61 Curvature of penis (lateral)

Micropenis

  • Q55.63 Congenital torsion of penis

Excludes1

acquired torsion of penis (N48.82)

Buried penis

Concealed penis

Excludes1

acquired buried penis (N48.83)

  • Q55.69 Other congenital malformation of penis

Congenital malformation of penis NOS

  • Q55.7 Congenital vasocutaneous fistula
  • Q55.8 Other specified congenital malformations of male genital organs
  • Q55.9 Congenital malformation of male genital organ, unspecified

Congenital anomaly of male genital organ

Congenital deformity of male genital organ

  • Q56 Indeterminate sex and pseudohermaphroditism

Excludes1

46,XX true hermaphrodite (Q99.1)

androgen insensitivity syndrome (E34.5-)

chimera 46,XX/46,XY true hermaphrodite (Q99.0)

female pseudohermaphroditism with adrenocortical disorder (E25.-)

pseudohermaphroditism with specified chromosomal anomaly (Q96-Q99)

pure gonadal dysgenesis (Q99.1)

  • Q56.0 Hermaphroditism, not elsewhere classified

Ovotestis

  • Q56.1 Male pseudohermaphroditism, not elsewhere classified

46, XY with streak gonads

Male pseudohermaphroditism NOS

  • Q56.2 Female pseudohermaphroditism, not elsewhere classified

Female pseudohermaphroditism NOS

  • Q56.3 Pseudohermaphroditism, unspecified
  • Q56.4 Indeterminate sex, unspecified

Ambiguous genitalia

Congenital malformations of the urinary system (Q60-Q64)

  • Q60 Renal agenesis and other reduction defects of kidney

Includes

congenital absence of kidney

congenital atrophy of kidney

infantile atrophy of kidney

  • Q60.0 Renal agenesis, unilateral
  • Q60.1 Renal agenesis, bilateral
  • Q60.2 Renal agenesis, unspecified
  • Q60.3 Renal hypoplasia, unilateral
  • Q60.4 Renal hypoplasia, bilateral
  • Q60.5 Renal hypoplasia, unspecified
  • Q61 Cystic kidney disease

Excludes1

acquired cyst of kidney (N28.1)

Potter's syndrome (Q60.6)

  • Q61.0 Congenital renal cyst
  • Q61.00 Congenital renal cyst, unspecified

Cyst of kidney NOS (congenital)

  • Q61.01 Congenital single renal cyst
  • Q61.02 Congenital multiple renal cysts
  • Q61.1 Polycystic kidney, infantile type

Polycystic kidney, autosomal recessive

  • Q61.11 Cystic dilatation of collecting ducts
  • Q61.19 Other polycystic kidney, infantile type
  • Q61.2 Polycystic kidney, adult type

Polycystic kidney, autosomal dominant

  • Q61.3 Polycystic kidney, unspecified

Multicystic dysplastic kidney

Multicystic kidney (development)

Multicystic kidney disease

Multicystic renal dysplasia

Excludes1

polycystic kidney disease (Q61.11-Q61.3)

  • Q61.5 Medullary cystic kidney

Nephronopthisis

Sponge kidney NOS

  • Q61.8 Other cystic kidney diseases

Fibrocystic kidney

Fibrocystic renal degeneration or disease

  • Q61.9 Cystic kidney disease, unspecified

Meckel-Gruber syndrome

  • Q62 Congenital obstructive defects of renal pelvis and congenital malformations of ureter
  • Q62.0 Congenital hydronephrosis
  • Q62.1 Congenital occlusion of ureter

Atresia and stenosis of ureter

  • Q62.10 Congenital occlusion of ureter, unspecified
  • Q62.11 Congenital occlusion of ureteropelvic junction
  • Q62.12 Congenital occlusion of ureterovesical orifice
  • Q62.2 Congenital megaureter

Congenital dilatation of ureter

  • Q62.3 Other obstructive defects of renal pelvis and ureter
  • Q62.31 Congenital ureterocele, orthotopic

Ectopic ureterocele

  • Q62.39 Other obstructive defects of renal pelvis and ureter

Ureteropelvic junction obstruction NOS

Congenital absence ureter

  • Q62.5 Duplication of ureter

Accessory ureter

Double ureter

  • Q62.6 Malposition of ureter
  • Q62.60 Malposition of ureter, unspecified
  • Q62.63 Anomalous implantation of ureter

Ectopia of ureter

Ectopic ureter

  • Q62.69 Other malposition of ureter
  • Q62.7 Congenital vesico-uretero-renal reflux
  • Q62.8 Other congenital malformations of ureter

Anomaly of ureter NOS

  • Q63 Other congenital malformations of kidney

Excludes1

congenital nephrotic syndrome (N04.-)

  • Q63.1 Lobulated, fused and horseshoe kidney

Congenital displaced kidney

Malrotation of kidney

  • Q63.3 Hyperplastic and giant kidney

Compensatory hypertrophy of kidney

  • Q63.8 Other specified congenital malformations of kidney

Congenital renal calculi

  • Q63.9 Congenital malformation of kidney, unspecified
  • Q64 Other congenital malformations of urinary system

Excludes1

hypospadias (Q54.-)

  • Q64.1 Exstrophy of urinary bladder
  • Q64.10 Exstrophy of urinary bladder, unspecified

Ectopia vesicae

  • Q64.11 Supravesical fissure of urinary bladder
  • Q64.12 Cloacal exstrophy of urinary bladder
  • Q64.19 Other exstrophy of urinary bladder

Extroversion of bladder

  • Q64.2 Congenital posterior urethral valves
  • Q64.3 Other atresia and stenosis of urethra and bladder neck
  • Q64.31 Congenital bladder neck obstruction

Congenital obstruction of vesicourethral orifice

  • Q64.32 Congenital stricture of urethra
  • Q64.33 Congenital stricture of urinary meatus
  • Q64.39 Other atresia and stenosis of urethra and bladder neck

Atresia and stenosis of urethra and bladder neck NOS

  • Q64.4 Malformation of urachus

Cyst of urachus

Patent urachus

Prolapse of urachus

  • Q64.5 Congenital absence of bladder and urethra
  • Q64.6 Congenital diverticulum of bladder
  • Q64.7 Other and unspecified congenital malformations of bladder and urethra

Excludes1

congenital prolapse of bladder (mucosa) (Q79.4)

  • Q64.70 Unspecified congenital malformation of bladder and urethra

Malformation of bladder or urethra NOS

  • Q64.71 Congenital prolapse of urethra
  • Q64.72 Congenital prolapse of urinary meatus
  • Q64.73 Congenital urethrorectal fistula
  • Q64.79 Other congenital malformations of bladder and urethra
  • Q64.8 Other specified congenital malformations of urinary system
  • Q64.9 Congenital malformation of urinary system, unspecified

Congenital anomaly NOS of urinary system

Congenital deformity NOS of urinary system

Congenital malformations and deformations of the musculoskeletal system (Q65-Q79)

  • Q65 Congenital deformities of hip

Excludes1

clicking hip (R29.4)

  • Q65.0 Congenital dislocation of hip, unilateral
  • Q65.00 Congenital dislocation of unspecified hip, unilateral
  • Q65.01 Congenital dislocation of right hip, unilateral
  • Q65.02 Congenital dislocation of left hip, unilateral
  • Q65.1 Congenital dislocation of hip, bilateral
  • Q65.2 Congenital dislocation of hip, unspecified
  • Q65.3 Congenital partial dislocation of hip, unilateral
  • Q65.30 Congenital partial dislocation of unspecified hip, unilateral
  • Q65.31 Congenital partial dislocation of right hip, unilateral
  • Q65.32 Congenital partial dislocation of left hip, unilateral
  • Q65.4 Congenital partial dislocation of hip, bilateral
  • Q65.5 Congenital partial dislocation of hip, unspecified
  • Q65.6 Congenital unstable hip

Congenital dislocatable hip

  • Q65.8 Other congenital deformities of hip
  • Q65.89 Other specified congenital deformities of hip

Anteversion of femoral neck

Congenital acetabular dysplasia

  • Q65.9 Congenital deformity of hip, unspecified
  • Q66 Congenital deformities of feet

Excludes1

reduction defects of feet (Q72.-)

valgus deformities (acquired) (M21.0-)

varus deformities (acquired) (M21.1-)

  • Q66.0 Congenital talipes equinovarus
  • Q66.00 Congenital talipes equinovarus, unspecified foot
  • Q66.01 Congenital talipes equinovarus, right foot
  • Q66.02 Congenital talipes equinovarus, left foot
  • Q66.1 Congenital talipes calcaneovarus
  • Q66.10 Congenital talipes calcaneovarus, unspecified foot
  • Q66.11 Congenital talipes calcaneovarus, right foot
  • Q66.12 Congenital talipes calcaneovarus, left foot
  • Q66.2 Congenital metatarsus (primus) varus
  • Q66.21 Congenital metatarsus primus varus
  • Q66.211 Congenital metatarsus primus varus, right foot
  • Q66.212 Congenital metatarsus primus varus, left foot
  • Q66.219 Congenital metatarsus primus varus, unspecified foot
  • Q66.22 Congenital metatarsus adductus

Congenital metatarsus varus

  • Q66.221 Congenital metatarsus adductus, right foot
  • Q66.222 Congenital metatarsus adductus, left foot
  • Q66.229 Congenital metatarsus adductus, unspecified foot
  • Q66.3 Other congenital varus deformities of feet

Hallux varus, congenital

  • Q66.30 Other congenital varus deformities of feet, unspecified foot
  • Q66.31 Other congenital varus deformities of feet, right foot
  • Q66.32 Other congenital varus deformities of feet, left foot
  • Q66.4 Congenital talipes calcaneovalgus
  • Q66.40 Congenital talipes calcaneovalgus, unspecified foot
  • Q66.41 Congenital talipes calcaneovalgus, right foot
  • Q66.42 Congenital talipes calcaneovalgus, left foot
  • Q66.5 Congenital pes planus

Congenital flat foot

Congenital rigid flat foot

Congenital spastic (everted) flat foot

Excludes1

pes planus, acquired (M21.4)

  • Q66.50 Congenital pes planus, unspecified foot
  • Q66.51 Congenital pes planus, right foot
  • Q66.52 Congenital pes planus, left foot
  • Q66.6 Other congenital valgus deformities of feet

Congenital metatarsus valgus

  • Q66.7 Congenital pes cavus
  • Q66.70 Congenital pes cavus, unspecified foot
  • Q66.71 Congenital pes cavus, right foot
  • Q66.72 Congenital pes cavus, left foot
  • Q66.8 Other congenital deformities of feet
  • Q66.80 Congenital vertical talus deformity, unspecified foot
  • Q66.81 Congenital vertical talus deformity, right foot
  • Q66.82 Congenital vertical talus deformity, left foot
  • Q66.89 Other specified congenital deformities of feet

Congenital asymmetric talipes

Congenital clubfoot NOS

Congenital talipes NOS

Congenital tarsal coalition

Hammer toe, congenital

  • Q66.9 Congenital deformity of feet, unspecified
  • Q66.90 Congenital deformity of feet, unspecified, unspecified foot
  • Q66.91 Congenital deformity of feet, unspecified, right foot
  • Q66.92 Congenital deformity of feet, unspecified, left foot
  • Q67 Congenital musculoskeletal deformities of head, face, spine and chest

Excludes1

congenital malformation syndromes classified to Q87.-

Potter's syndrome (Q60.6)

  • Q67.0 Congenital facial asymmetry
  • Q67.1 Congenital compression facies
  • Q67.4 Other congenital deformities of skull, face and jaw

Congenital depressions in skull

Congenital hemifacial atrophy or hypertrophy

Deviation of nasal septum, congenital

Squashed or bent nose, congenital

Excludes1

dentofacial anomalies (including malocclusion) (M26.-)

syphilitic saddle nose (A50.5)

  • Q67.5 Congenital deformity of spine

Congenital postural scoliosis

Congenital scoliosis NOS

Excludes1

infantile idiopathic scoliosis (M41.0)

scoliosis due to congenital bony malformation (Q76.3)

Congenital funnel chest

Congenital pigeon chest

  • Q67.8 Other congenital deformities of chest

Congenital deformity of chest wall NOS

  • Q68 Other congenital musculoskeletal deformities

Excludes1

reduction defects of limb(s) (Q71-Q73)

Excludes2

congenital myotonic chondrodystrophy (G71.13)

  • Q68.0 Congenital deformity of sternocleidomastoid muscle

Congenital contracture of sternocleidomastoid (muscle)

Congenital (sternomastoid) torticollis

Sternomastoid tumor (congenital)

  • Q68.1 Congenital deformity of finger(s) and hand

Congenital clubfinger

Spade-like hand (congenital)

  • Q68.2 Congenital deformity of knee

Congenital dislocation of knee

Congenital genu recurvatum

  • Q68.3 Congenital bowing of femur

Excludes1

anteversion of femur (neck) (Q65.89)

  • Q68.4 Congenital bowing of tibia and fibula
  • Q68.5 Congenital bowing of long bones of leg, unspecified
  • Q68.8 Other specified congenital musculoskeletal deformities

Congenital deformity of clavicle

Congenital deformity of elbow

Congenital deformity of forearm

Congenital deformity of scapula

Congenital deformity of wrist

Congenital dislocation of elbow

Congenital dislocation of shoulder

Congenital dislocation of wrist

  • Q69 Polydactyly
  • Q69.0 Accessory finger(s)

Accessory hallux

  • Q69.9 Polydactyly, unspecified

Supernumerary digit(s) NOS

Complex syndactyly of fingers with synostosis

  • Q70.00 Fused fingers, unspecified hand
  • Q70.01 Fused fingers, right hand
  • Q70.02 Fused fingers, left hand
  • Q70.03 Fused fingers, bilateral

Simple syndactyly of fingers without synostosis

  • Q70.10 Webbed fingers, unspecified hand
  • Q70.11 Webbed fingers, right hand
  • Q70.12 Webbed fingers, left hand
  • Q70.13 Webbed fingers, bilateral

Complex syndactyly of toes with synostosis

  • Q70.20 Fused toes, unspecified foot

Simple syndactyly of toes without synostosis

  • Q70.30 Webbed toes, unspecified foot
  • Q70.31 Webbed toes, right foot
  • Q70.4 Polysyndactyly, unspecified

Excludes1

specified syndactyly of hand and feet - code to specified conditions (Q70.0- -Q70.3-)

  • Q70.9 Syndactyly, unspecified

Symphalangy NOS

  • Q71 Reduction defects of upper limb
  • Q71.0 Congenital complete absence of upper limb
  • Q71.00 Congenital complete absence of unspecified upper limb
  • Q71.01 Congenital complete absence of right upper limb
  • Q71.02 Congenital complete absence of left upper limb
  • Q71.03 Congenital complete absence of upper limb, bilateral
  • Q71.1 Congenital absence of upper arm and forearm with hand present
  • Q71.10 Congenital absence of unspecified upper arm and forearm with hand present
  • Q71.11 Congenital absence of right upper arm and forearm with hand present
  • Q71.12 Congenital absence of left upper arm and forearm with hand present
  • Q71.13 Congenital absence of upper arm and forearm with hand present, bilateral
  • Q71.2 Congenital absence of both forearm and hand
  • Q71.20 Congenital absence of both forearm and hand, unspecified upper limb
  • Q71.21 Congenital absence of both forearm and hand, right upper limb
  • Q71.22 Congenital absence of both forearm and hand, left upper limb
  • Q71.23 Congenital absence of both forearm and hand, bilateral
  • Q71.3 Congenital absence of hand and finger
  • Q71.30 Congenital absence of unspecified hand and finger
  • Q71.31 Congenital absence of right hand and finger
  • Q71.32 Congenital absence of left hand and finger
  • Q71.33 Congenital absence of hand and finger, bilateral
  • Q71.4 Longitudinal reduction defect of radius

Clubhand (congenital)

Radial clubhand

  • Q71.40 Longitudinal reduction defect of unspecified radius
  • Q71.41 Longitudinal reduction defect of right radius
  • Q71.42 Longitudinal reduction defect of left radius
  • Q71.43 Longitudinal reduction defect of radius, bilateral
  • Q71.5 Longitudinal reduction defect of ulna
  • Q71.50 Longitudinal reduction defect of unspecified ulna
  • Q71.51 Longitudinal reduction defect of right ulna
  • Q71.52 Longitudinal reduction defect of left ulna
  • Q71.53 Longitudinal reduction defect of ulna, bilateral
  • Q71.60 Lobster-claw hand, unspecified hand
  • Q71.61 Lobster-claw right hand
  • Q71.63 Lobster-claw hand, bilateral
  • Q71.8 Other reduction defects of upper limb
  • Q71.81 Congenital shortening of upper limb
  • Q71.811 Congenital shortening of right upper limb
  • Q71.812 Congenital shortening of left upper limb
  • Q71.813 Congenital shortening of upper limb, bilateral
  • Q71.819 Congenital shortening of unspecified upper limb
  • Q71.89 Other reduction defects of upper limb
  • Q71.891 Other reduction defects of right upper limb
  • Q71.892 Other reduction defects of left upper limb
  • Q71.893 Other reduction defects of upper limb, bilateral
  • Q71.899 Other reduction defects of unspecified upper limb
  • Q71.9 Unspecified reduction defect of upper limb
  • Q71.90 Unspecified reduction defect of unspecified upper limb
  • Q71.91 Unspecified reduction defect of right upper limb
  • Q71.92 Unspecified reduction defect of left upper limb
  • Q71.93 Unspecified reduction defect of upper limb, bilateral
  • Q72 Reduction defects of lower limb
  • Q72.0 Congenital complete absence of lower limb
  • Q72.00 Congenital complete absence of unspecified lower limb
  • Q72.01 Congenital complete absence of right lower limb
  • Q72.02 Congenital complete absence of left lower limb
  • Q72.03 Congenital complete absence of lower limb, bilateral
  • Q72.1 Congenital absence of thigh and lower leg with foot present
  • Q72.10 Congenital absence of unspecified thigh and lower leg with foot present
  • Q72.11 Congenital absence of right thigh and lower leg with foot present
  • Q72.12 Congenital absence of left thigh and lower leg with foot present
  • Q72.13 Congenital absence of thigh and lower leg with foot present, bilateral
  • Q72.2 Congenital absence of both lower leg and foot
  • Q72.20 Congenital absence of both lower leg and foot, unspecified lower limb
  • Q72.21 Congenital absence of both lower leg and foot, right lower limb
  • Q72.22 Congenital absence of both lower leg and foot, left lower limb
  • Q72.23 Congenital absence of both lower leg and foot, bilateral
  • Q72.3 Congenital absence of foot and toe(s)
  • Q72.30 Congenital absence of unspecified foot and toe(s)
  • Q72.31 Congenital absence of right foot and toe(s)
  • Q72.32 Congenital absence of left foot and toe(s)
  • Q72.33 Congenital absence of foot and toe(s), bilateral
  • Q72.4 Longitudinal reduction defect of femur

Proximal femoral focal deficiency

  • Q72.40 Longitudinal reduction defect of unspecified femur
  • Q72.41 Longitudinal reduction defect of right femur
  • Q72.42 Longitudinal reduction defect of left femur
  • Q72.43 Longitudinal reduction defect of femur, bilateral
  • Q72.5 Longitudinal reduction defect of tibia
  • Q72.50 Longitudinal reduction defect of unspecified tibia
  • Q72.51 Longitudinal reduction defect of right tibia
  • Q72.52 Longitudinal reduction defect of left tibia
  • Q72.53 Longitudinal reduction defect of tibia, bilateral
  • Q72.6 Longitudinal reduction defect of fibula
  • Q72.60 Longitudinal reduction defect of unspecified fibula
  • Q72.61 Longitudinal reduction defect of right fibula
  • Q72.62 Longitudinal reduction defect of left fibula
  • Q72.63 Longitudinal reduction defect of fibula, bilateral
  • Q72.70 Split foot, unspecified lower limb
  • Q72.71 Split foot, right lower limb
  • Q72.72 Split foot, left lower limb
  • Q72.8 Other reduction defects of lower limb
  • Q72.81 Congenital shortening of lower limb
  • Q72.811 Congenital shortening of right lower limb
  • Q72.812 Congenital shortening of left lower limb
  • Q72.813 Congenital shortening of lower limb, bilateral
  • Q72.819 Congenital shortening of unspecified lower limb
  • Q72.89 Other reduction defects of lower limb
  • Q72.891 Other reduction defects of right lower limb
  • Q72.892 Other reduction defects of left lower limb
  • Q72.893 Other reduction defects of lower limb, bilateral
  • Q72.899 Other reduction defects of unspecified lower limb
  • Q72.9 Unspecified reduction defect of lower limb
  • Q72.90 Unspecified reduction defect of unspecified lower limb
  • Q72.91 Unspecified reduction defect of right lower limb
  • Q72.92 Unspecified reduction defect of left lower limb
  • Q72.93 Unspecified reduction defect of lower limb, bilateral
  • Q73 Reduction defects of unspecified limb
  • Q73.0 Congenital absence of unspecified limb(s)

Amelia NOS

  • Q73.1 Phocomelia, unspecified limb(s)

Phocomelia NOS

  • Q73.8 Other reduction defects of unspecified limb(s)

Longitudinal reduction deformity of unspecified limb(s)

Ectromelia of limb NOS

Hemimelia of limb NOS

Reduction defect of limb NOS

  • Q74 Other congenital malformations of limb(s)

Excludes1

polydactyly (Q69.-)

reduction defect of limb (Q71-Q73)

syndactyly (Q70.-)

  • Q74.0 Other congenital malformations of upper limb(s), including shoulder girdle

Accessory carpal bones

Cleidocranial dysostosis

Congenital pseudarthrosis of clavicle

Macrodactylia (fingers)

Madelung's deformity

Radioulnar synostosis

Sprengel's deformity

Triphalangeal thumb

  • Q74.1 Congenital malformation of knee

Congenital absence of patella

Congenital dislocation of patella

Congenital genu valgum

Congenital genu varum

Rudimentary patella

Excludes1

congenital dislocation of knee (Q68.2)

congenital genu recurvatum (Q68.2)

nail patella syndrome (Q87.2)

  • Q74.2 Other congenital malformations of lower limb(s), including pelvic girdle

Congenital fusion of sacroiliac joint

Congenital malformation of ankle joint

Congenital malformation of sacroiliac joint

Excludes1

anteversion of femur (neck) (Q65.89)

  • Q74.3 Arthrogryposis multiplex congenita
  • Q74.8 Other specified congenital malformations of limb(s)
  • Q74.9 Unspecified congenital malformation of limb(s)

Congenital anomaly of limb(s) NOS

  • Q75 Other congenital malformations of skull and face bones

Excludes1

congenital malformation of face NOS (Q18.-)

congenital malformation syndromes classified to Q87.-

dentofacial anomalies (including malocclusion) (M26.-)

musculoskeletal deformities of head and face (Q67.0-Q67.4)

skull defects associated with congenital anomalies of brain such as

anencephaly (Q00.0)

encephalocele (Q01.-)

hydrocephalus (Q03.-)

microcephaly (Q02)

Acrocephaly

Imperfect fusion of skull

Oxycephaly

Trigonocephaly

  • Q75.1 Craniofacial dysostosis

Crouzon's disease

  • Q75.4 Mandibulofacial dysostosis

Franceschetti syndrome

Treacher Collins syndrome

  • Q75.5 Oculomandibular dysostosis
  • Q75.8 Other specified congenital malformations of skull and face bones

Absence of skull bone, congenital

Congenital deformity of forehead

Platybasia

  • Q75.9 Congenital malformation of skull and face bones, unspecified

Congenital anomaly of face bones NOS

Congenital anomaly of skull NOS

  • Q76 Congenital malformations of spine and bony thorax

Excludes1

congenital musculoskeletal deformities of spine and chest (Q67.5-Q67.8)

  • Q76.0 Spina bifida occulta

Excludes1

meningocele (spinal) (Q05.-)

spina bifida (aperta) (cystica) (Q05.-)

  • Q76.1 Klippel-Feil syndrome

Cervical fusion syndrome

  • Q76.2 Congenital spondylolisthesis

Congenital spondylolysis

Excludes1

spondylolisthesis (acquired) (M43.1-)

spondylolysis (acquired) (M43.0-)

  • Q76.3 Congenital scoliosis due to congenital bony malformation

Hemivertebra fusion or failure of segmentation with scoliosis

  • Q76.4 Other congenital malformations of spine, not associated with scoliosis
  • Q76.411 Congenital kyphosis, occipito-atlanto-axial region
  • Q76.412 Congenital kyphosis, cervical region
  • Q76.413 Congenital kyphosis, cervicothoracic region
  • Q76.414 Congenital kyphosis, thoracic region
  • Q76.415 Congenital kyphosis, thoracolumbar region
  • Q76.419 Congenital kyphosis, unspecified region
  • Q76.425 Congenital lordosis, thoracolumbar region
  • Q76.426 Congenital lordosis, lumbar region
  • Q76.427 Congenital lordosis, lumbosacral region
  • Q76.428 Congenital lordosis, sacral and sacrococcygeal region
  • Q76.429 Congenital lordosis, unspecified region
  • Q76.49 Other congenital malformations of spine, not associated with scoliosis

Congenital absence of vertebra NOS

Congenital fusion of spine NOS

Congenital malformation of lumbosacral (joint) (region) NOS

Congenital malformation of spine NOS

Hemivertebra NOS

Malformation of spine NOS

Platyspondylisis NOS

Supernumerary vertebra NOS

Supernumerary rib in cervical region

  • Q76.6 Other congenital malformations of ribs

Accessory rib

Congenital absence of rib

Congenital fusion of ribs

Congenital malformation of ribs NOS

Excludes1

short rib syndrome (Q77.2)

  • Q76.7 Congenital malformation of sternum

Congenital absence of sternum

Sternum bifidum

  • Q76.8 Other congenital malformations of bony thorax
  • Q76.9 Congenital malformation of bony thorax, unspecified
  • Q77 Osteochondrodysplasia with defects of growth of tubular bones and spine

Excludes1

mucopolysaccharidosis (E76.0-E76.3)

Excludes2

congenital myotonic chondrodystrophy (G71.13)

Hypochondrogenesis

  • Q77.1 Thanatophoric short stature

Asphyxiating thoracic dysplasia (Jeune)

  • Q77.3 Chondrodysplasia punctata

Excludes1

Rhizomelic chondrodysplasia punctata (E71.43)

Hypochondroplasia

Osteosclerosis congenita

  • Q77.5 Diastrophic dysplasia
  • Q77.6 Chondroectodermal dysplasia

Ellis-van Creveld syndrome

  • Q77.7 Spondyloepiphyseal dysplasia
  • Q77.8 Other osteochondrodysplasia with defects of growth of tubular bones and spine
  • Q77.9 Osteochondrodysplasia with defects of growth of tubular bones and spine, unspecified
  • Q78 Other osteochondrodysplasias

Excludes2

congenital myotonic chondrodystrophy (G71.13)

  • Q78.0 Osteogenesis imperfecta

Fragilitas ossium

Osteopsathyrosis

  • Q78.1 Polyostotic fibrous dysplasia

Albright(-McCune)(-Sternberg) syndrome

Albers-Schönberg syndrome

Osteosclerosis NOS

  • Q78.3 Progressive diaphyseal dysplasia

Camurati-Engelmann syndrome

Maffucci's syndrome

Ollier's disease

  • Q78.5 Metaphyseal dysplasia

Pyle's syndrome

  • Q78.6 Multiple congenital exostoses

Diaphyseal aclasis

  • Q78.8 Other specified osteochondrodysplasias

Osteopoikilosis

  • Q78.9 Osteochondrodysplasia, unspecified

Chondrodystrophy NOS

Osteodystrophy NOS

  • Q79 Congenital malformations of musculoskeletal system, not elsewhere classified

Excludes2

congenital (sternomastoid) torticollis (Q68.0)

  • Q79.0 Congenital diaphragmatic hernia

Excludes1

congenital hiatus hernia (Q40.1)

  • Q79.1 Other congenital malformations of diaphragm

Absence of diaphragm

Congenital malformation of diaphragm NOS

Eventration of diaphragm

Omphalocele

Excludes1

umbilical hernia (K42.-)

  • Q79.4 Prune belly syndrome

Congenital prolapse of bladder mucosa

Eagle-Barrett syndrome

  • Q79.5 Other congenital malformations of abdominal wall

Excludes1

umbilical hernia (K42.-)

  • Q79.51 Congenital hernia of bladder
  • Q79.59 Other congenital malformations of abdominal wall
  • Q79.6 Ehlers-Danlos syndromes
  • Q79.60 Ehlers-Danlos syndrome, unspecified
  • Q79.61 Classical Ehlers-Danlos syndrome

Classical EDS (cEDS)

  • Q79.62 Hypermobile Ehlers-Danlos syndrome

Hypermobile EDS (hEDS)

  • Q79.63 Vascular Ehlers-Danlos syndrome

Vascular EDS (vEDS)

  • Q79.69 Other Ehlers-Danlos syndromes
  • Q79.8 Other congenital malformations of musculoskeletal system

Absence of muscle

Absence of tendon

Accessory muscle

Amyotrophia congenita

Congenital constricting bands

Congenital shortening of tendon

Poland syndrome

  • Q79.9 Congenital malformation of musculoskeletal system, unspecified

Congenital anomaly of musculoskeletal system NOS

Congenital deformity of musculoskeletal system NOS

Other congenital malformations (Q80-Q89)

  • Q80 Congenital ichthyosis

Excludes1

Refsum's disease (G60.1)

  • Q80.0 Ichthyosis vulgaris
  • Q80.1 X-linked ichthyosis
  • Q80.2 Lamellar ichthyosis

Collodion baby

  • Q80.3 Congenital bullous ichthyosiform erythroderma
  • Q80.8 Other congenital ichthyosis
  • Q80.9 Congenital ichthyosis, unspecified
  • Q81 Epidermolysis bullosa
  • Q81.0 Epidermolysis bullosa simplex

Excludes1

Cockayne's syndrome (Q87.19)

  • Q81.1 Epidermolysis bullosa letalis

Herlitz' syndrome

  • Q81.2 Epidermolysis bullosa dystrophica
  • Q81.8 Other epidermolysis bullosa
  • Q81.9 Epidermolysis bullosa, unspecified
  • Q82 Other congenital malformations of skin

Excludes1

acrodermatitis enteropathica (E83.2)

congenital erythropoietic porphyria (E80.0)

pilonidal cyst or sinus (L05.-)

Sturge-Weber (-Dimitri) syndrome (Q85.8)

  • Q82.0 Hereditary lymphedema
  • Q82.1 Xeroderma pigmentosum
  • Q82.2 Congenital cutaneous mastocytosis

Congenital diffuse cutaneous mastocytosis

Congenital maculopapular cutaneous mastocytosis

Congenital urticaria pigmentosa

Excludes1

cutaneous mastocytosis NOS (D47.01)

diffuse cutaneous mastocytosis (with onset after newborn period) (D47.01)

malignant mastocytosis (C96.2-)

systemic mastocytosis (D47.02)

urticaria pigmentosa (non-congenital) (with onset after newborn period) (D47.01)

  • Q82.3 Incontinentia pigmenti
  • Q82.4 Ectodermal dysplasia (anhidrotic)

Excludes1

Ellis-van Creveld syndrome (Q77.6)

  • Q82.5 Congenital non-neoplastic nevus

Birthmark NOS

Flammeus Nevus

Portwine Nevus

Sanguineous Nevus

Strawberry Nevus

Vascular Nevus NOS

Verrucous Nevus

Excludes2

Café au lait spots (L81.3)

lentigo (L81.4)

nevus NOS (D22.-)

araneus nevus (I78.1)

melanocytic nevus (D22.-)

pigmented nevus (D22.-)

spider nevus (I78.1)

stellar nevus (I78.1)

  • Q82.6 Congenital sacral dimple

Parasacral dimple

Excludes2

pilonidal cyst with abscess (L05.01)

pilonidal cyst without abscess (L05.91)

  • Q82.8 Other specified congenital malformations of skin

Abnormal palmar creases

Accessory skin tags

Benign familial pemphigus (Hailey-Hailey)

Congenital poikiloderma

Cutis laxa (hyperelastica)

Dermatoglyphic anomalies

Inherited keratosis palmaris et plantaris

Keratosis follicularis (Darier-White)

Excludes1

Ehlers-Danlos syndrome (Q79.6-)

  • Q82.9 Congenital malformation of skin, unspecified
  • Q83 Congenital malformations of breast

Excludes2

absence of pectoral muscle (Q79.8)

hypoplasia of breast (N64.82)

micromastia (N64.82)

  • Q83.0 Congenital absence of breast with absent nipple

Supernumerary breast

Supernumerary nipple

  • Q83.8 Other congenital malformations of breast
  • Q83.9 Congenital malformation of breast, unspecified
  • Q84 Other congenital malformations of integument
  • Q84.0 Congenital alopecia

Congenital atrichosis

  • Q84.1 Congenital morphological disturbances of hair, not elsewhere classified

Beaded hair

Monilethrix

Pili annulati

Excludes1

Menkes' kinky hair syndrome (E83.0)

  • Q84.2 Other congenital malformations of hair

Congenital hypertrichosis

Congenital malformation of hair NOS

Persistent lanugo

Excludes1

nail patella syndrome (Q87.2)

  • Q84.4 Congenital leukonychia
  • Q84.5 Enlarged and hypertrophic nails

Congenital onychauxis

Pachyonychia

  • Q84.6 Other congenital malformations of nails

Congenital clubnail

Congenital koilonychia

Congenital malformation of nail NOS

  • Q84.8 Other specified congenital malformations of integument

Aplasia cutis congenita

  • Q84.9 Congenital malformation of integument, unspecified

Congenital anomaly of integument NOS

Congenital deformity of integument NOS

  • Q85 Phakomatoses, not elsewhere classified

Excludes1

ataxia telangiectasia (Louis-Bar) (G11.3)

familial dysautonomia (Riley-Day) (G90.1)

  • Q85.0 Neurofibromatosis (nonmalignant)
  • Q85.00 Neurofibromatosis, unspecified
  • Q85.01 Neurofibromatosis, type 1

Von Recklinghausen disease

  • Q85.02 Neurofibromatosis, type 2

Acoustic neurofibromatosis

  • Q85.09 Other neurofibromatosis

Bourneville's disease

Epiloia

  • Q85.8 Other phakomatoses, not elsewhere classified

Peutz-Jeghers Syndrome

Sturge-Weber(-Dimitri) syndrome

von Hippel-Lindau syndrome

Excludes1

Meckel-Gruber syndrome (Q61.9)

  • Q85.9 Phakomatosis, unspecified

Hamartosis NOS

  • Q86 Congenital malformation syndromes due to known exogenous causes, not elsewhere classified

Excludes2

iodine-deficiency-related hypothyroidism (E00-E02)

nonteratogenic effects of substances transmitted via placenta or breast milk (P04.-)

  • Q86.0 Fetal alcohol syndrome (dysmorphic)
  • Q86.1 Fetal hydantoin syndrome

Meadow's syndrome

  • Q86.2 Dysmorphism due to warfarin
  • Q86.8 Other congenital malformation syndromes due to known exogenous causes
  • Q87 Other specified congenital malformation syndromes affecting multiple systems Use additional

code(s) to identify all associated manifestations

  • Q87.0 Congenital malformation syndromes predominantly affecting facial appearance

Acrocephalopolysyndactyly

Acrocephalosyndactyly (Apert)

Cryptophthalmos syndrome

Cyclopia

Goldenhar syndrome

Moebius syndrome

Oro-facial-digital syndrome

Robin syndrome

Whistling face

  • Q87.1 Congenital malformation syndromes predominantly associated with short stature

Excludes1

Ellis-van Creveld syndrome (Q77.6)

Smith-Lemli-Opitz syndrome (E78.72)

  • Q87.19 Other congenital malformation syndromes predominantly associated with short stature

Aarskog syndrome

Cockayne syndrome

De Lange syndrome

Dubowitz syndrome

Noonan syndrome

Robinow-Silverman-Smith syndrome

Russell-Silver syndrome

Seckel syndrome

  • Q87.2 Congenital malformation syndromes predominantly involving limbs

Holt-Oram syndrome

Klippel-Trenaunay-Weber syndrome

Nail patella syndrome

Rubinstein-Taybi syndrome

Sirenomelia syndrome

Thrombocytopenia with absent radius (TAR) syndrome

VATER syndrome

  • Q87.3 Congenital malformation syndromes involving early overgrowth

Beckwith-Wiedemann syndrome

Sotos syndrome

Weaver syndrome

  • Q87.40 Marfan's syndrome, unspecified
  • Q87.41 Marfan's syndrome with cardiovascular manifestations
  • Q87.410 Marfan's syndrome with aortic dilation
  • Q87.418 Marfan's syndrome with other cardiovascular manifestations
  • Q87.42 Marfan's syndrome with ocular manifestations
  • Q87.43 Marfan's syndrome with skeletal manifestation
  • Q87.5 Other congenital malformation syndromes with other skeletal changes
  • Q87.8 Other specified congenital malformation syndromes, not elsewhere classified

Excludes1

Zellweger syndrome (E71.510)

  • Q87.81 Alport syndrome Use additional

code to identify stage of chronic kidney disease (N18.1-N18.6)

  • Q87.82 Arterial tortuosity syndrome
  • Q87.89 Other specified congenital malformation syndromes, not elsewhere classified

Laurence-Moon (-Bardet)-Biedl syndrome

  • Q89 Other congenital malformations, not elsewhere classified
  • Q89.0 Congenital absence and malformations of spleen

Excludes1

isomerism of atrial appendages (with asplenia or polysplenia) (Q20.6)

  • Q89.09 Congenital malformations of spleen

Congenital splenomegaly

  • Q89.1 Congenital malformations of adrenal gland

Excludes1

adrenogenital disorders (E25.-)

congenital adrenal hyperplasia (E25.0)

  • Q89.2 Congenital malformations of other endocrine glands

Congenital malformation of parathyroid or thyroid gland

Persistent thyroglossal duct

Thyroglossal cyst

Excludes1

congenital goiter (E03.0)

congenital hypothyroidism (E03.1)

Dextrocardia with situs inversus

Mirror-image atrial arrangement with situs inversus

Situs inversus or transversus abdominalis

Situs inversus or transversus thoracis

Transposition of abdominal viscera

Transposition of thoracic viscera

Excludes1

dextrocardia NOS (Q24.0)

Craniopagus

Dicephaly

Pygopagus

Thoracopagus

  • Q89.7 Multiple congenital malformations, not elsewhere classified

Multiple congenital anomalies NOS

Multiple congenital deformities NOS

Excludes1

congenital malformation syndromes affecting multiple systems (Q87.-)

  • Q89.8 Other specified congenital malformations Use additional

code(s) to identify all associated manifestations

  • Q89.9 Congenital malformation, unspecified

Congenital anomaly NOS

Congenital deformity NOS

Chromosomal abnormalities, not elsewhere classified (Q90-Q99)

Excludes2

mitochondrial metabolic disorders (E88.4-)

  • Q90 Down syndrome Use additional

code(s) to identify any associated physical conditions and degree of intellectual disabilities (F70-F79)

  • Q90.0 Trisomy 21, nonmosaicism (meiotic nondisjunction)
  • Q90.1 Trisomy 21, mosaicism (mitotic nondisjunction)
  • Q90.2 Trisomy 21, translocation
  • Q90.9 Down syndrome, unspecified

Trisomy 21 NOS

  • Q91 Trisomy 18 and Trisomy 13
  • Q91.0 Trisomy 18, nonmosaicism (meiotic nondisjunction)
  • Q91.1 Trisomy 18, mosaicism (mitotic nondisjunction)
  • Q91.2 Trisomy 18, translocation
  • Q91.3 Trisomy 18, unspecified
  • Q91.4 Trisomy 13, nonmosaicism (meiotic nondisjunction)
  • Q91.5 Trisomy 13, mosaicism (mitotic nondisjunction)
  • Q91.6 Trisomy 13, translocation
  • Q91.7 Trisomy 13, unspecified
  • Q92 Other trisomies and partial trisomies of the autosomes, not elsewhere classified

Includes

unbalanced translocations and insertions

Excludes1

trisomies of chromosomes 13, 18, 21 (Q90-Q91)

  • Q92.0 Whole chromosome trisomy, nonmosaicism (meiotic nondisjunction)
  • Q92.1 Whole chromosome trisomy, mosaicism (mitotic nondisjunction)

Less than whole arm duplicated

Whole arm or more duplicated

Excludes1

partial trisomy due to unbalanced translocation (Q92.5)

  • Q92.5 Duplications with other complex rearrangements

Partial trisomy due to unbalanced translocations Code also

any associated deletions due to unbalanced translocations, inversions and insertions (Q93.7)

Trisomies due to dicentrics

Trisomies due to extra rings

Trisomies due to isochromosomes

Individual with marker heterochromatin

  • Q92.61 Marker chromosomes in normal individual
  • Q92.62 Marker chromosomes in abnormal individual
  • Q92.7 Triploidy and polyploidy
  • Q92.8 Other specified trisomies and partial trisomies of autosomes

Duplications identified by fluorescence in situ hybridization (FISH)

Duplications identified by in situ hybridization (ISH)

Duplications seen only at prometaphase

  • Q92.9 Trisomy and partial trisomy of autosomes, unspecified
  • Q93 Monosomies and deletions from the autosomes, not elsewhere classified
  • Q93.0 Whole chromosome monosomy, nonmosaicism (meiotic nondisjunction)
  • Q93.1 Whole chromosome monosomy, mosaicism (mitotic nondisjunction)
  • Q93.2 Chromosome replaced with ring, dicentric or isochromosome
  • Q93.3 Deletion of short arm of chromosome 4

Wolff-Hirschorn syndrome

  • Q93.4 Deletion of short arm of chromosome 5

Cri-du-chat syndrome

  • Q93.5 Other deletions of part of a chromosome
  • Q93.59 Other deletions of part of a chromosome
  • Q93.7 Deletions with other complex rearrangements

Deletions due to unbalanced translocations, inversions and insertions Code also

any associated duplications due to unbalanced translocations, inversions and insertions (Q92.5)

  • Q93.8 Other deletions from the autosomes
  • Q93.81 Velo-cardio-facial syndrome

Deletion 22q11.2

Miller-Dieker syndrome

Smith-Magenis syndrome

  • Q93.89 Other deletions from the autosomes

Deletions identified by fluorescence in situ hybridization (FISH)

Deletions identified by in situ hybridization (ISH)

Deletions seen only at prometaphase

  • Q93.9 Deletion from autosomes, unspecified
  • Q95 Balanced rearrangements and structural markers, not elsewhere classified

Includes

Robertsonian and balanced reciprocal translocations and insertions

  • Q95.0 Balanced translocation and insertion in normal individual
  • Q95.1 Chromosome inversion in normal individual
  • Q95.2 Balanced autosomal rearrangement in abnormal individual
  • Q95.3 Balanced sex/autosomal rearrangement in abnormal individual
  • Q95.5 Individual with autosomal fragile site
  • Q95.8 Other balanced rearrangements and structural markers
  • Q95.9 Balanced rearrangement and structural marker, unspecified
  • Q96 Turner's syndrome

Excludes1

Noonan syndrome (Q87.19)

  • Q96.1 Karyotype 46, X iso (Xq)

Karyotype 46, isochromosome Xq

  • Q96.2 Karyotype 46, X with abnormal sex chromosome, except iso (Xq)

Karyotype 46, X with abnormal sex chromosome, except isochromosome Xq

  • Q96.3 Mosaicism, 45, X/46, XX or XY
  • Q96.4 Mosaicism, 45, X/other cell line(s) with abnormal sex chromosome
  • Q96.8 Other variants of Turner's syndrome
  • Q96.9 Turner's syndrome, unspecified
  • Q97 Other sex chromosome abnormalities, female phenotype, not elsewhere classified

Excludes1

Turner's syndrome (Q96.-)

  • Q97.1 Female with more than three X chromosomes
  • Q97.2 Mosaicism, lines with various numbers of X chromosomes
  • Q97.3 Female with 46, XY karyotype
  • Q97.8 Other specified sex chromosome abnormalities, female phenotype
  • Q97.9 Sex chromosome abnormality, female phenotype, unspecified
  • Q98 Other sex chromosome abnormalities, male phenotype, not elsewhere classified
  • Q98.0 Klinefelter syndrome karyotype 47, XXY
  • Q98.1 Klinefelter syndrome, male with more than two X chromosomes
  • Q98.3 Other male with 46, XX karyotype
  • Q98.4 Klinefelter syndrome, unspecified
  • Q98.6 Male with structurally abnormal sex chromosome
  • Q98.7 Male with sex chromosome mosaicism
  • Q98.8 Other specified sex chromosome abnormalities, male phenotype
  • Q98.9 Sex chromosome abnormality, male phenotype, unspecified
  • Q99 Other chromosome abnormalities, not elsewhere classified
  • Q99.0 Chimera 46, XX/46, XY

Chimera 46, XX/46, XY true hermaphrodite

  • Q99.1 46, XX true hermaphrodite

46, XX with streak gonads

46, XY with streak gonads

Pure gonadal dysgenesis

  • Q99.2 Fragile X chromosome

Fragile X syndrome

  • Q99.8 Other specified chromosome abnormalities
  • Q99.9 Chromosomal abnormality, unspecified

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