ICD-10-CM (2010)/CHAPTER 17

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)

Q00 Anencephaly and similar malformations

Q00.0 Anencephaly

Acephaly

Acrania

Amyelencephaly

Hemianencephaly

Hemicephaly

Q00.1 Craniorachischisis

Q00.2 Iniencephaly

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

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

Q04.1 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 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 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)

larynx (Q31.-)

lip NEC (Q38.0)

nose (Q30.-)

parathyroid gland (Q89.2)

thyroid gland (Q89.2)

Q10 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 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 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

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 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)

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 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 (Q25.7)

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)

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 (Q64.7)

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 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

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 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 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 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

Q55.69 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 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

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 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)

Q65 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 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 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) (Q65.8)

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 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 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 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 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 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) (Q65.8)

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 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-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 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)

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 (Q87.1)

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 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 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)

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 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

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 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 (E88.3-)

Q90 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 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

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 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 (Q87.1)

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 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