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

    Note: CODES FROM THIS CHAPTER ARE NOT FOR USE ON MATERNAL OR FETAL RECORDS
    Excludes1: inborn errors of metabolism (E70-E90)
    This chapter contains the following blocks:
    Q00-Q07 Congenital malformations of the nervous system
    Q10-Q18 Congenital malformations of eye, ear, face and neck
    Q20-Q28 Congenital malformations of the circulatory system
    Q30-Q34 Congenital malformations of the respiratory system
    Q35-Q37 Cleft lip and cleft palate
    Q38-Q45 Other congenital malformations of the digestive system
    Q50-Q56 Congenital malformations of genital organs
    Q60-Q64 Congenital malformations of the urinary system
    Q65-Q79 Congenital malformations and deformations of the musculoskeletal system
    Q80-Q89 Other congenital malformations
    Q90-Q99 Chromosomal abnormalities, not elsewhere classified

# Congenital malformations of the nervous system (Q00-Q07)Congenital malformations of the nervous system (Q00-Q07)
## Q00 Anencephaly and similar malformationsQ00 Anencephaly and similar malformations

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

## Q01 EncephaloceleQ01 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 MicrocephalyQ02 Microcephaly

    Includes: hydromicrocephaly
       micrencephalon
    Excludes1: Meckel-Gruber syndrome (Q61.9)

## Q03 Congenital hydrocephalusQ03 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 brainQ04 Other congenital malformations of brain

    Excludes1: cyclopia (Q87.0)
       macrocephaly (Q75.3)
    Q04.0 Congenital malformations of corpus callosum
       Agenesis of corpus callosum
    Q04.1 Arhinencephaly
    Q04.2 Holoprosencephaly
    Q04.3 Other reduction deformities of brain
       Absence 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
    Q04.5 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
       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 cordQ06 Other congenital malformations of spinal cord

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

## Q07 Other congenital malformations of nervous systemQ07 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)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)
       larynx (Q31.-)
       lip NEC (Q38.0)
       nose (Q30.-)
       parathyroid gland (Q89.2)
       thyroid gland (Q89.2)

## Q10 Congenital malformations of eyelid, lacrimal apparatus and orbitQ10 Congenital malformations of eyelid, lacrimal apparatus and orbit

    Excludes1: cryptophthalmos NOS (Q11.2)
       cryptophthalmos syndrome (Q87.0)
    Q10.0 Congenital ptosis
    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 macrophthalmosQ11 Anophthalmos, microphthalmos and macrophthalmos

    Q11.0 Cystic eyeball
    Q11.1 Other anophthalmos
       Anophthalmos NOS
       Agenesis of eye
       Aplasia of eye
    Q11.2 Microphthalmos
       Cryptophthalmos NOS
       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 malformationsQ12 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 eyeQ13 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 eyeQ14 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 eyeQ15 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 earQ17 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 neckQ18 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)Congenital malformations of the circulatory system (Q20-Q28)
## Q20 Congenital malformations of cardiac chambers and connectionsQ20 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 septaQ21 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
       Excludes1: Eisenmenger's complex (I27.8)
          Eisenmenger's syndrome (I27.8)
    Q21.9 Congenital malformation of cardiac septum, unspecified
       Septal (heart) defect NOS

## Q22 Congenital malformations of pulmonary and tricuspid valvesQ22 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 valvesQ23 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 heartQ24 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 arteriesQ25 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)
    Q25.2 Atresia of aorta
    Q25.3 Supravalvular aortic stenosis
       Excludes1: congenital aortic stenosis NOS (Q23.0)
          congenital aortic valve stenosis (Q23.0)
    Q25.4 Other congenital malformations of aorta
       Absence of aorta
       Aneurysm of sinus of Valsalva (ruptured)
       Aplasia of aorta
       Congenital aneurysm of aorta
       Congenital malformations of aorta
       Congenital dilatation of aorta
       Double aortic arch [vascular ring of aorta]
       Hypoplasia of aorta
       Persistent convolutions of aortic arch
       Persistent right aortic arch
       Excludes1: hypoplasia of aorta in hypoplastic left heart syndrome (Q23.4)
    Q25.5 Atresia of pulmonary artery
    Q25.6 Stenosis of pulmonary artery
       Supravalvular pulmonary stenosis
    Q25.7 Other congenital malformations of pulmonary artery
       Aberrant pulmonary artery
       Agenesis of pulmonary artery
       Congenital aneurysm of pulmonary artery
       Congenital anomaly of pulmonary artery
       Congenital pulmonary arteriovenous aneurysm
       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 veinsQ26 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 systemQ27 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 systemQ28 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 (Q25.7)
       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
    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)Congenital malformations of the respiratory system (Q30-Q34)
## Q30 Congenital malformations of noseQ30 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 larynxQ31 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 bronchusQ32 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 lungQ33 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 systemQ34 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)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 palateQ35 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 lipQ36 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 lipQ37 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)Other congenital malformations of the digestive system (Q38-Q45)
## Q38 Other congenital malformations of tongue, mouth and pharynxQ38 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 esophagusQ39 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 intestineQ41 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 intestineQ42 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 intestineQ43 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 (Q64.7)
          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 liverQ44 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 systemQ45 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)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 ligamentsQ50 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 cervixQ51 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
    Q51.3 Bicornate uterus
    Q51.4 Unicornate uterus
    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
       Hypoplasia of uterus and cervix
    Q51.9 Congenital malformation of uterus and cervix, unspecified

## Q52 Other congenital malformations of female genitaliaQ52 Other congenital malformations of female genitalia

    Q52.0 Congenital absence of vagina
    Q52.1 Doubling of vagina
       Septate vagina
       Excludes1: doubling of vagina with doubling of uterus and cervix (Q51.1-)
    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 testicleQ53 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
       Q53.12 Ectopic perineal testis, unilateral
    Q53.2 Undescended testicle, bilateral
       Q53.20 Undescended testicle, unspecified, bilateral
       Q53. ​21 Abdominal testis, bilateral
       Q53.22 Ectopic perineal testis, bilateral
    Q53.9 Undescended testicle, unspecified
       Cryptorchism NOS

## Q54 HypospadiasQ54 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 organsQ55 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
       ​Q55.69 Other congenital malformation of penis NOS
          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 pseudohermaphroditismQ56 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)Congenital malformations of the urinary system (Q60-Q64)
## Q60 Renal agenesis and other reduction defects of kidneyQ60 Renal agenesis and other reduction defects of kidney

    Includes: congenital absence 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 diseaseQ61 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 ureterQ62 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 kidneyQ63 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 systemQ64 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 extrophy 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)Congenital malformations and deformations of the musculoskeletal system (Q65-Q79)
## Q65 Congenital deformities of hipQ65 Congenital deformities of hip

    Excludes1: clicking hip (R29.4)
    Q65.0 Congenital dislocation of hip, unilateral
       Q65.00 Congenital dislocation of hip, unilateral, unspecified side
       Q65.01 Congenital dislocation of right hip
       Q65.02 Congenital dislocation of left hip
    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 hip, unilateral, unspecified side
       Q65.31 Congenital partial dislocation of right hip
       Q65.32 Congenital partial dislocation of left hip
    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
       Anteversion ​of femoral neck
       Congenital acetabular dysplasia
       Congenital coxa valga
       Congenital coxa vara
    Q65.9 Congenital deformity of hip, unspecified

## Q66 Congenital deformities of feetQ66 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
    Q66.1 Congenital talipes calcaneovarus
    Q66.2 Congenital metatarsus (primus) varus
    Q66.3 Other congenital varus deformities of feet
       Hallux varus, congenital
    Q66.4 Congenital talipes calcaneovalgus
    Q66.5 Congenital pes planus
       Congenital flat foot
       Congenital rigid flat foot
       Congenital spastic (everted) flat foot
       Excludes1: pes planus, acquired (M21.4)
    Q66.6 Other congenital valgus deformities of feet
       Congenital metatarsus valgus
    Q66.7 Congenital pes cavus
    Q66.8 Other congenital deformities of feet
       Congenital asymmetric talipes
       Congenital clubfoot NOS
       Congenital talipes NOS
       Congenital tarsal coalition
       Congenital vertical talus
       Hammer toe, congenital
    Q66.9 Congenital deformity of feet, unspecified

## Q67 Congenital musculoskeletal deformities of head, face, spine and chestQ67 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 deformitiesQ68 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) (Q65.8)
    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 PolydactylyQ69 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 SyndactylyQ70 Syndactyly

    Q70.0 Fused fingers
       Complex syndactyly of fingers with synostosis
       Q70.00 Fused fingers, unspecified fingers
       Q70.01 Fused right fingers
       Q70.02 Fused left fingers
       Q70.03 Fused fingers, bilateral
    Q70.1 Webbed fingers
       Simple syndactyly of fingers without synostosis
       Q70.10 Webbed fingers, unspecified side
       Q70.11 Webbed right fingers
       Q70.12 Webbed left fingers
       Q70.13 Webbed fingers, bilateral
    Q70.2 Fused toes
       Complex syndactyly of toes with synostosis
    Q70.3 Webbed toes
       Simple syndactyly of toes without synostosis
    Q70.4 Polysyndactyly
    Q70.9 Syndactyly, unspecified
       Symphalangy NOS

## Q71 Reduction defects of upper limbQ71 Reduction defects of upper limb

    Q71.0 Congenital complete absence of upper limb
       Q71.00 Congenital complete absence of upper limb, unspecified side
       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 upper arm and forearm with hand present, unspecified side
       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 side
       Q71.21 Congenital absence of both right forearm and hand
       Q71.22 Congenital absence of both left forearm and hand
       Q71.23 Congenital absence of both forearm and hand, bilateral
    Q71.3 Congenital absence of hand and finger
       Q71. ​30 Congenital absence of hand and finger, unspecified side
       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 radius, unspecified side
       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 ulna, unspecified side
       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 side
       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
       Congenital shortening of upper limb
       Q71.80 Other reduction defects of upper limb, unspecified side
       Q71.81 Other reduction defects of right upper limb
       Q71.82 Other reduction defects of left upper limb
       Q71.83 Other reduction defects of upper limb, bilateral
    Q71.9 Reduction defect of upper limb, unspecified
       Q71.90 Reduction defect of upper limb, unspecified, side unspecified
       Q71.91 Reduction defect of right upper limb, unspecified
       Q71.92 Reduction defect of left upper limb, unspecified
       Q71.93 Reduction defect of upper limb, unspecified, bilateral

## Q72 Reduction defects of lower limbQ72 Reduction defects of lower limb

    Q72.0 Congenital complete absence of lower limb
       Q72.00 Congenital complete absence of lower limb, unspecified side
       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 thigh and lower leg with foot present, unspecified side
       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 side
       Q72.21 Congenital absence of both right lower leg and foot
       Q72.22 Congenital absence of both left lower leg and foot
       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 foot and toe(s), unspecified side
       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 femur, unspecified side
       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 tibia, unspecified side
       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 fibula, unspecified side
       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 side
       Q72.71 Right split foot
       Q72.72 Left split foot
       Q72.73 Split foot, bilateral
    Q72.8 Other reduction defects of lower limb
       Congenital shortening of lower limb(s)
       Q72.80 Other reduction defects of lower limb, unspecified side
       Q72.81 Other reduction defects of right lower limb
       Q72. ​82 Other reduction defects of left lower limb
       Q72.83 Other reduction defects of lower limb, bilateral
    Q72.9 Reduction defect of lower limb, unspecified
       Q72.90 Reduction defect of lower limb, unspecified, unspecified side
       Q72.91 Reduction defect of right lower limb, unspecified
       Q72.92 Reduction defect of left lower limb, unspecified
       Q72.93 Reduction defect of lower limb, unspecified, bilateral

## Q73 Reduction defects of unspecified limbQ73 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)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) (Q65.8)
    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 bonesQ75 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 thoraxQ76 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 spineQ77 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 osteochondrodysplasiasQ78 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-Sch?nberg 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 classifiedQ79 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 syndrome
    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's 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)Other congenital malformations (Q80-Q89)
## Q80 Congenital ichthyosisQ80 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 bullosaQ81 Epidermolysis bullosa

    Q81.0 Epidermolysis bullosa simplex
    Excludes1: Cockayne's syndrome (Q87.1)
    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 skinQ82 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 Mastocytosis
       Urticaria pigmentosa
       Excludes1: malignant mastocytosis (C96.2)
    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: caf? 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)
    Q82.8 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 breastQ83 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 integumentQ84 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 classifiedQ85 Phakomatoses, not elsewhere classified

    Excludes1: ataxia telangiectasia [Louis-Bar] (G11.3)
       familial dysautonomia [Riley-Day] (G90.1)
    Q85.0 Neurofibromatosis (nonmalignant)
       Von Recklinghausen's disease
    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 classifiedQ86 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 systemsQ87 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
       Aarskog ​syndrome
       Cockayne syndrome
       De Lange syndrome
       Dubowitz syndrome
       Noonan syndrome
       Prader-Willi syndrome
       Robinow-Silverman-Smith syndrome
       Russell-Silver syndrome
       Seckel syndrome
       Smith-Lemli-Opitz syndrome
       Excludes1: Ellis-van Creveld syndrome (Q77.6)
    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)
       Q87.89 Other specified congenital malformation syndromes, not elsewhere classified
          Laurence-Moon (-Bardet)-Biedl syndrome

## Q89 Other congenital malformations, not elsewhere classifiedQ89 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)Chromosomal abnormalities, not elsewhere classified (Q90-Q99)

    Excludes2: mitochondrial metabolic disorders (E88.3-)

## Q90 Down syndromeQ90 Down syndrome

    Use ​additional code(s) to identify any associated physical conditions and degree of mental retardation (F70-F79)
    Q90.0 Trisomy 21, nonmosaicism (meiotic nondisjunction)
    Q90.1 Trisomy 21, mosaicism (mitotic nondisjunction)
    Q90.2 Trisomy 21, translocation
    Q90.9 Down's syndrome, unspecified
       Trisomy 21 NOS

## Q91 Trisomy 18 and Trisomy 13Q91 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 classifiedQ92 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
       Angelman syndrome
    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
       Q93.88 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 classifiedQ95 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 syndromeQ96 Turner's syndrome

    Excludes1: Noonan syndrome (Q87.1)
    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 classifiedQ97 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 classifiedQ98 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 classifiedQ99 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