ICD-10 Chapter XVII: Congenital malformations, deformations and chromosomal abnormalities

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Q00-Q89 - Congenital malformations and deformations

(Q00-Q07) nervous system

• (Q00.) Anencephaly and similar malformations
  • (Q00.0) Anencephaly
    • Acephaly
  • (Q00.1) Craniorachischisis
  • (Q00.2) Iniencephaly

• (Q01.) Encephalocele
• (Q02.) Microcephaly
• (Q03.) Congenital hydrocephalus
  • (Q03.0) Malformations 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
  • (Q04.0) Congenital malformations of corpus callosum
  • (Q04.1) Arhinencephaly
  • (Q04.2) Holoprosencephaly
  • (Q04.3) Other reduction deformities of brain
    • Agyria and Lissencephaly (EUROCAT Q04.33)
    • Microgyria and Pachygyria (EUROCAT Q04.34)
    • Hydranencephaly (EUROCAT Q04.35)
  • (Q04.4) Septo-optic dysplasia
  • (Q04.5) Megalencephaly
  • (Q04.6) Congenital cerebral cysts
    • Porencephaly
    • Schizencephaly
  • (Q04.8) Other specified congenital malformations of brain
  • (Q04.9) Congenital malformation of brain, unspecified

• (Q05.) Spina bifida

• (Q06.) Other congenital malformations of spinal cord
  • (Q06.0) Amyelia
  • (Q06.1) Hypoplasia and dysplasia of spinal cord
  • (Q06.2) Diastematomyelia
  • (Q06.3) Other congenital cauda equina malformations
  • (Q06.4) Hydromyelia
  • (Q06.8) Other specified congenital malformations of spinal cord
  • (Q06.9) Congenital malformation of spinal cord, unspecified

• (Q07.) Other congenital malformations of nervous system
  • (Q07.0) Arnold-Chiari syndrome
  • (Q07.8) Other specified congenital malformations of nervous system
  • (Q07.9) Congenital malformation of nervous system, unspecified

(Q10-Q18) eye, ear, face and neck

• (Q10.) Congenital malformations of eyelid, lacrimal apparatus and orbit
  • (Q10.0) Congenital ptosis
  • (Q10.1) Congenital ectropion
  • (Q10.2) Congenital entropion
  • (Q10.3) Other congenital malformations of eyelid
  • (Q10.4) Absence and agenesis of lacrimal apparatus
  • (Q10.5) Congenital stenosis and stricture of lacrimal duct
  • (Q10.6) Other congenital malformations of lacrimal apparatus
  • (Q10.7) Congenital malformation of orbit

• (Q11.) Anophthalmos, microphthalmos and macrophthalmos
  • (Q11.0) Cystic eyeball
  • (Q11.1) Other anophthalmos
  • (Q11.2) Microphthalmos
  • (Q11.3) Macrophthalmos

• (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
  • (Q12.9) Congenital lens malformation, unspecified

• (Q13.) Congenital malformations of anterior segment of eye
  • (Q13.1) Absence of iris
    • Aniridia
• (Q14.) Congenital malformations of posterior segment of eye
• (Q15.) Other congenital malformations of eye
  • (Q15.0) Congenital glaucoma
    • Buphthalmos
    • Glaucoma of newborn
    • Hydrophthalmos
    • Keratoglobus, congenital, with glaucoma
    • Macrocornea with glaucoma
    • Macrophthalmos in congenital glaucoma
    • Megalocornea with glaucoma

• (Q16.) Congenital malformations of ear causing impairment of hearing

• (Q17.) Other congenital malformations of ear
  • (Q17.0) Accessory auricle
  • (Q17.1) Macrotia
  • (Q17.2) Microtia
  • (Q17.3) Other misshapen ear
  • (Q17.4) Misplaced ear
  • (Q17.5) Prominent ear
  • (Q17.8) Other specified congenital malformations of ear
  • (Q17.9) Congenital malformation of ear, unspecified

• (Q18.) Other congenital malformations of face and neck
  • (Q18.0) Sinus, fistula and cyst of branchial cleft
  • (Q18.1) Preauricular sinus and cyst
  • (Q18.2) Other branchial cleft malformations
    • Otocephaly
  • (Q18.3) Webbing of neck
  • (Q18.4) Macrostomia
  • (Q18.5) Microstomia
  • (Q18.6) Macrocheilia
  • (Q18.7) Microcheilia
  • (Q18.8) Other specified congenital malformations of face and neck
  • (Q18.9) Congenital malformation of face and neck, unspecified

(Q20-Q28) circulatory system

• (Q20.) Congenital malformations of cardiac chambers and connections
  • (Q20.0) Common arterial trunk
    • Persistent truncus arteriosus
  • (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
  • (Q20.5) Discordant atrioventricular connection
    • Corrected transposition
  • (Q20.6) Isomerism of atrial appendages

• (Q21.) Congenital malformations of cardiac septa
  • (Q21.0) Ventricular septal defect
  • (Q21.1) Atrial septal defect
  • (Q21.2) Atrioventricular septal defect
  • (Q21.3) Tetralogy of Fallot
  • (Q21.8) Other congenital malformations of cardiac septa
    • Eisenmenger's syndrome

• (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
  • (Q22.3) Other congenital malformations of pulmonary valve
  • (Q22.4) Congenital tricuspid stenosis
    • 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
  • (Q23.1) Congenital insufficiency of aortic valve
  • (Q23.2) Congenital mitral stenosis
  • (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
  • (Q24.0) Dextrocardia
  • (Q24.1) Laevocardia
  • (Q24.2) Cor triatriatum
  • (Q24.3) Pulmonary infundibular stenosis
  • (Q24.4) Congenital subaortic stenosis
  • (Q24.5) Malformation of coronary vessels
  • (Q24.6) Congenital heart block
  • (Q24.8) Other specified congenital malformations of heart
  • (Q24.9) Congenital malformation of heart, unspecified

• (Q25.) Congenital malformations of great arteries
  • (Q25.0) Patent ductus arteriosus
  • (Q25.1) Coarctation of aorta
  • (Q25.2) Atresia of aorta
  • (Q25.3) Stenosis of aorta
  • (Q25.4) Other congenital malformations of aorta
    • Overriding aorta (EUROCAT Q25.42)
    • Aneurysm of sinus of Valsalva (ruptured) (EUROCAT Q25.43)
    • Double aortic arch (vascular ring of aorta)
  • (Q25.5) Atresia of pulmonary artery
  • (Q25.6) Stenosis of pulmonary artery
  • (Q25.7) Other congenital malformations of pulmonary artery

• (Q26.) Congenital malformations of great veins
  • (Q26.0) Congenital stenosis of vena cava
  • (Q26.1) Persistent left superior vena cava
  • (Q26.2) Total anomalous pulmonary venous connection
  • (Q26.3) Partial anomalous pulmonary venous connection
  • (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
    • Scimitar syndrome
  • (Q26.9) Congenital malformation of great vein, unspecified

• (Q27.) Other congenital malformations of peripheral vascular system
  • (Q27.0) Congenital absence and hypoplasia of umbilical artery
  • (Q27.1) Congenital renal artery stenosis
  • (Q27.2) Other congenital malformations of renal artery
  • (Q27.3) Peripheral arteriovenous malformation
  • (Q27.4) Congenital phlebectasia
  • (Q27.8) Other specified congenital malformations of peripheral vascular system
  • (Q27.9) Congenital malformation of peripheral vascular system, unspecified

• (Q28.) Other congenital malformations of circulatory system
  • (Q28.0) Arteriovenous malformation of precerebral vessels
  • (Q28.1) Other malformations of precerebral vessels
  • (Q28.2) Arteriovenous malformation of cerebral vessels
  • (Q28.3) Other malformations of cerebral vessels
  • (Q28.8) Other specified congenital malformations of circulatory system
  • (Q28.9) Congenital malformation of circulatory system, unspecified

(Q30-Q34) respiratory system

• (Q30.) [[Congenital malformations of nose
  • (Q30.0) Choanal atresia
  • (Q30.1) Agenesis and underdevelopment of nose
  • (Q30.2) Fissured, notched and cleft nose
  • (Q30.3) Congenital perforated nasal septum
  • (Q30.8) Other congenital malformations of nose
  • (Q30.9) Congenital malformation of nose, unspecified

• (Q31.) Congenital malformations of larynx
  • (Q31.0) Web of larynx
  • (Q31.1) Congenital subglottic stenosis
  • (Q31.2) Laryngeal hypoplasia
  • (Q31.3) Laryngocele
  • (Q31.5) Congenital laryngomalacia
  • (Q31.8) Other congenital malformations of larynx
  • (Q31.9) Congenital malformation of larynx, unspecified

• (Q32.) Congenital malformations of trachea and bronchus
  • (Q32.0) Congenital tracheomalacia
  • (Q32.1) Other congenital malformations of trachea
  • (Q32.2) Congenital bronchomalacia
  • (Q32.3) Congenital stenosis of bronchus
  • (Q32.4) Other congenital malformations of bronchus

• (Q33.) Congenital malformations of lung
  • (Q33.0) Congenital cystic lung
  • (Q33.1) Accessory lobe of lung
  • (Q33.2) Sequestration of lung
  • (Q33.3) Agenesis of lung
  • (Q33.4) Congenital bronchiectasis
  • (Q33.5) Ectopic tissue in lung
  • (Q33.6) Hypoplasia and dysplasia of lung
  • (Q33.8) Other congenital malformations of lung
  • (Q33.9) Congenital malformation of lung, unspecified

• (Q34.) Other congenital malformations of respiratory system
  • (Q34.0) Anomaly of pleura
  • (Q34.1) Congenital cyst of mediastinum
  • (Q34.8) Other specified congenital malformations of respiratory system
  • (Q34.9) Congenital malformation of respiratory system, unspecified

(Q35-Q45) digestive system

• (Q35.) Cleft palate

• (Q36.) Cleft lip

• (Q37.) Cleft palate with cleft lip

• (Q38.) Other congenital malformations of tongue, mouth and pharynx
  • (Q38.0) Congenital malformations of lips, not elsewhere classified
    • Van der Woude's syndrome
  • (Q38.1) Ankyloglossia
  • (Q38.2) Macroglossia
  • (Q38.3) Other congenital malformations of tongue
  • (Q38.4) Congenital malformations of salivary glands and ducts
  • (Q38.5) Congenital malformations of palate, not elsewhere classified
  • (Q38.6) Other congenital malformations of mouth
  • (Q38.7) Pharyngeal pouch

• (Q39.) Congenital malformations of oesophagus
  • (Q39.0) Atresia of oesophagus without fistula
  • (Q39.1) Atresia of oesophagus with tracheo-oesophageal fistula
  • (Q39.2) Congenital tracheo-oesophageal fistula without atresia
  • (Q39.3) Congenital stenosis and stricture of oesophagus
  • (Q39.4) Oesophageal web
  • (Q39.5) Congenital dilatation of oesophagus
  • (Q39.6) Diverticulum of oesophagus
  • (Q39.8) Other congenital malformations of oesophagus
  • (Q39.9) Congenital malformation of oesophagus, unspecified

• (Q40.) Other congenital malformations of upper alimentary tract
  • (Q40.0) Congenital hypertrophic pyloric stenosis
  • (Q40.1) Congenital hiatus hernia

• (Q41.) Congenital absence, atresia and stenosis of small intestine
  • (Q41.0) Congenital absence, atresia and stenosis of duodenum
  • (Q41.1) Congenital absence, atresia and stenosis of 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

• (Q42.) Congenital absence, atresia and stenosis 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
  • (Q43.1) Hirschsprung's disease
  • (Q43.2) Other congenital functional disorders of colon
  • (Q43.3) Congenital malformations of intestinal fixation
  • (Q43.4) Duplication of intestine
  • (Q43.5) Ectopic anus
  • (Q43.6) Congenital fistula of rectum and anus
  • (Q43.7) Persistent cloaca
  • (Q43.8) Other specified congenital malformations of intestine

• (Q44.) Congenital malformations of gallbladder, bile ducts and liver
  • (Q44.0) Agenesis, aplasia and hypoplasia of gallbladder
  • (Q44.1) Other congenital malformations of 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
  • (Q44.6) Cystic disease of liver
  • (Q44.7) Other congenital malformations of liver
    • Accessory liver
    • Alagille's syndrome

• (Q45.) Other congenital malformations of digestive system
  • (Q45.0) Agenesis, aplasia and hypoplasia of pancreas
  • (Q45.1) Annular pancreas
  • (Q45.2) Congenital pancreatic cyst
  • (Q45.3) Other congenital malformations of pancreas and pancreatic duct
  • (Q45.8) Other specified congenital malformations of digestive system
  • (Q45.9) Congenital malformation of digestive system, unspecified]]

(Q50-Q56) genital organs

• (Q50.) Congenital malformations of ovaries, fallopian tubes and broad ligaments
• (Q51.) Congenital malformations of uterus and cervix
  • (Q51.0) Agenesis and aplasia of uterus
  • (Q51.1) Doubling of uterus with doubling of cervix and vagina
  • (Q51.2) Other doubling of uterus
  • (Q51.3) Bicornate uterus
  • (Q51.4) Unicornate uterus
  • (Q51.5) Agenesis and aplasia of cervix
  • (Q51.6) Embryonic cyst of cervix
  • (Q51.7) Congenital fistulae between uterus and digestive and urinary tracts
  • (Q51.8) Other congenital malformations of uterus and cervix
  • (Q51.9) 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
  • (Q52.2) Congenital rectovaginal fistula
  • (Q52.3) Imperforate hymen
  • (Q