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ICD-10-CM (2010)/CHAPTER 17

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Contents

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

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)[edit]

Q00 Anencephaly and similar malformations[edit]

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

Q01 Encephalocele[edit]

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[edit]

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

Q03 Congenital hydrocephalus[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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)[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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)[edit]

Q20 Congenital malformations of cardiac chambers and connections[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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)[edit]

Q30 Congenital malformations of nose[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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)[edit]

Use additional code to identify associated malformation of the nose (Q30.2)
Excludes1: Robin's syndrome (Q87.0)

Q35 Cleft palate[edit]

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[edit]

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[edit]

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)[edit]

Q38 Other congenital malformations of tongue, mouth and pharynx[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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)[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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)[edit]

Q60 Renal agenesis and other reduction defects of kidney[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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)[edit]

Q65 Congenital deformities of hip[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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)[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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)[edit]

Q80 Congenital ichthyosis[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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)[edit]

Excludes2: mitochondrial metabolic disorders (E88.3-)

Q90 Down syndrome[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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