14. CONGENITAL ANOMALIES ICD 9 CODES(740-759)
Acrania
Amyelencephalus
Hemianencephaly
Hemicephaly
Excludes: spina bifida occulta (756.17)
The following fifth-digit subclassification is for use with category 741:
0 unspecified region
1 cervical region
2 dorsal (thoracic) region
3 lumbar region
[0-3]
Arnold-Chiari syndrome, type II
Chiari malformation, type II
Any condition classifiable to 741.9 with any condition classifiable to 742.3
[0-3]
Hydromeningocele (spinal)
Hydromyelocele
Meningocele (spinal)
Meningomyelocele
Myelocele
Myelocystocele
Rachischisis
Spina bifida (aperta)
Syringomyelocele
Excludes: congenital central alveolar hypoventilation syndrome (327.25)
Encephalocystocele
Encephalomyelocele
Hydroencephalocele
Hydromeningocele, cranial
Meningocele, cerebral
Meningoencephalocele
Hydromicrocephaly
Micrencephaly
Absence of part of brain
Agenesis of part of brain
Agyria
Aplasia of part of brain
Arhinencephaly
Holoprosencephaly
Hypoplasia of part of brain
Microgyria
Aqueduct of Sylvius:
anomaly
obstruction, congenital
stenosis
Atresia of foramina of Magendie and Luschka
Hydrocephalus in newborn
Excludes: hydrocephalus:
acquired (331.3-331.4)
due to congenital toxoplasmosis (771.2)
with any condition classifiable to 741.9 (741.0)
Congenital cerebral cyst
Macroencephaly
Macrogyria
Megalencephaly
Multiple anomalies of brain NOS
Porencephaly
Ulegyria
Hydrorhachis
Amyelia
Atelomyelia
Congenital anomaly of spinal meninges
Defective development of cauda equina
Hypoplasia of spinal cord
Myelatelia
Myelodysplasia
Agenesis of nerve
Displacement of brachial plexus
Familial dysautonomia
Jaw-winking syndrome
Marcus-Gunn syndrome
Riley-Day syndrome
Excludes: neurofibromatosis (237.70-237.79)
Anomaly of brain, nervous system, and spinal cord
Congenital, of brain, nervous system, and spinal cord:
disease of brain, nervous system, and spinal cord
lesion of brain, nervous system, and spinal cord
Deformity of brain, nervous system, and spinal cord
Agenesis
Congenital absence of eye
Anophthalmos NOS
Dysplasia of eye
Hypoplasia of eye
Rudimentary eye
Glaucoma:
congenital
newborn
Hydrophthalmos
Excludes: glaucoma of childhood (365.14)
traumatic glaucoma due to birth injury (767.8)
Keratoglobus, congenital, associated with buphthalmos
Megalocornea associated with buphthalmos
Excludes: infantile cataract (366.00-366.09)
Congenital absence of lens
Microphakia
Spherophakia
Microcornea
Excludes: that associated with buphthalmos (743.22)
Anomaly:
Axenfeld's
Peters'
Rieger's
Anisocoria, congenital
Atresia of pupil
Coloboma of iris
Corectopia
Congenital vitreous opacity
Coloboma of optic disc (congenital)
Congenital retinal aneurysm
Ablepharon
Absence of eyelid
Accessory eyelid
Congenital:
ectropion
entropion
Absence, agenesis, of cilia
Absence, agenesis of:
lacrimal apparatus
punctum lacrimale
Accessory lacrimal canal
Accessory eye muscles
Excludes: congenital nystagmus (379.51)
ocular albinism (270.2)
optic nerve hypoplasia (377.43)
retinitis pigmentosa (362.74)
Congenital:
anomaly NOS of eye [any part]
deformity NOS of eye [any part]
Excludes: anomaly of:
cervical spine (754.2, 756.10-756.19)
larynx (748.2-748.3)
nose (748.0-748.1)
parathyroid gland (759.2)
thyroid gland (759.2)
cleft lip (749.10-749.25)
Excludes: congenital deafness without mention of cause (380.0-389.9)
Absence of:
auditory canal (external)
auricle (ear) (with stenosis or atresia of auditory canal)
Atresia or stricture of auditory canal (external)
Atresia or stricture of osseous meatus (ear)
Fusion of ear ossicles
Congenital anomaly of:
membranous labyrinth
organ of Corti
Absence of ear, congenital
Accessory tragus
Polyotia
Preauricular appendage
Supernumerary:
ear
lobule
Excludes: that with impairment of hearing (744.00-744.09)
Absence of Eustachian tube
Bat ear
Darwin's tubercle
Pointed ear
Prominence of auricle
Ridge ear
Excludes: preauricular sinus (744.46)
Congenital:
anomaly NOS of ear, NEC
deformity NOS of ear, NEC
Branchial:
sinus (external) (internal)
vestige
Fistula (of):
auricle, congenital
cervicoaural
Pterygium colli
Hypertrophy of lip, congenital
Excludes: congenital fistula of lip (750.25)
musculoskeletal anomalies (754.0-754.1, 756.0)
Congenital:
anomaly NOS of face [any part] or neck [any part]
deformity NOS of face [any part] or neck [any part]
Absent septum between aorta and pulmonary artery
Communication (abnormal) between aorta and pulmonary artery
Aortic septal defect
Common aortopulmonary trunk
Persistent truncus arteriosus
Transposition of great vessels:
NOS
classical
Dextratransposition of aorta
Incomplete transposition of great vessels
Origin of both great vessels from right ventricle
Taussig-Bing syndrome or defect
Fallot's pentalogy
Ventricular septal defect with pulmonary stenosis or atresia, dextraposition of aorta, and hypertrophy of right ventricle
Excludes: Fallot's triad (746.09)
Cor triloculare biatriatum
Single ventricle
Eisenmenger's defect or complex
Gerbode defect
Interventricular septal defect
Left ventricular-right atrial communication
Roger's disease
Excludes: common atrioventricular canal type (745.69)
single ventricle (745.3)
Defect:
atrium secundum
fossa ovalis
Lutembacher's syndrome
Patent or persistent:
foramen ovale
ostium secundum
Persistent ostium primum
Absence of atrial septum
Atrioventricular canal type ventricular septal defect
Common atrioventricular canal
Common atrium
Absence of atrial and ventricular septa
Septal defect NOS
Excludes: endocardial fibroelastosis (425.3)
Excludes: infundibular or subvalvular pulmonic stenosis (746.83)
tetralogy of Fallot (745.2)
Congenital absence of pulmonary valve
Congenital insufficiency of pulmonary valve
Fallot's triad or trilogy
Absence of tricuspid valve
Congenital aortic stenosis
Excludes: congenital:
subaortic stenosis (746.81)
supravalvular aortic stenosis (747.22)
Bicuspid aortic valve
Congenital aortic insufficiency
Fused commissure of mitral valve
Parachute deformity of mitral valve
Supernumerary cusps of mitral valve
Atresia, or marked hypoplasia, of aortic orifice or valve, with hypoplasia of ascending aorta and defective development of left ventricle (with mitral valve atresia)
Subvalvular pulmonic stenosis
Shone's syndrome
Uhl's disease
Use additional code: for associated anomalies, such as:
coarctation of aorta (747.10)
congenital mitral stenosis (746.5)
subaortic stenosis (746.81)
Anomalous origin or communication of coronary artery
Arteriovenous malformation of coronary artery
Coronary artery:
absence
arising from aorta or pulmonary trunk
single
Complete or incomplete atrioventricular [AV] block
Abdominal heart
Dextrocardia
Ectopia cordis
Levocardia (isolated)
Mesocardia
Excludes: dextrocardia with complete transposition of viscera (759.3)
Atresia of cardiac vein
Hypoplasia of cardiac vein
Congenital:
cardiomegaly
diverticulum, left ventricle
pericardial defect
Congenital:
anomaly of heart NOS
heart disease NOS
Patent ductus Botalli
Persistent ductus arteriosus
Hypoplasia of aortic arch
Anomalous origin, right subclavian artery
Dextraposition of aorta
Double aortic arch
Kommerell's diverticulum
Overriding aorta
Persistent:
convolutions, aortic arch
right aortic arch
Vascular ring
Excludes: hypoplasia of aortic arch (747.10)
Absence of aorta
Aplasia of aorta
Hypoplasia of aorta
Stricture of aorta
Supra (valvular)-aortic stenosis
Excludes: congenital aortic (valvular) stenosis or stricture, so stated (746.3)
hypoplasia of aorta in hypoplastic left heart syndrome (746.7)
Aneurysm of sinus of Valsalva
Congenital:
aneurysm of aorta
dilation of aorta
Agenesis of pulmonary artery
Atresia of pulmonary artery
Coarctation of pulmonary artery
Hypoplasia of pulmonary artery
Stenosis of pulmonary artery
Pulmonary arteriovenous aneurysm
Excludes: acquired pulmonary arteriovenous fistula (417.0)
Anomaly of pulmonary artery
Anomaly NOS of:
pulmonary veins
vena cava
Total anomalous pulmonary venous return [TAPVR]:
subdiaphragmatic
supradiaphragmatic
Partial anomalous pulmonary venous return
Absence of vena cava (inferior) (superior)
Congenital stenosis of vena cava (inferior) (superior)
Persistent:
left posterior cardinal vein
left superior vena cava
Scimitar syndrome
Transposition of pulmonary veins NOS
Single umbilical artery
Absence of artery or vein, NEC
Anomaly of artery or vein, NEC
Atresia of artery or vein, NEC
Arteriovenous aneurysm (peripheral)
Arteriovenous malformation of the peripheral vascular system
Congenital:
aneurysm (peripheral)
phlebectasia
stricture, artery
varix
Multiple renal arteries
Excludes: anomalies of:
cerebral vessels (747.81)
pulmonary artery (747.39)
congenital retinal aneurysm (743.58)
hemangioma (228.00-228.09)
lymphangioma (228.1)
Arteriovenous malformation of brain
Cerebral arteriovenous aneurysm, congenital
Congenital anomalies of cerebral vessels
Excludes: ruptured cerebral (arteriovenous) aneurysm (430)
Arteriovenous malformation of spinal vessel
Persistent pulmonary hypertension
Primary pulmonary hypertension of newborn
Aneurysm, congenital, specified site not elsewhere classified
Excludes: congenital aneurysm:
coronary (746.85)
peripheral (747.6)
pulmonary (747.39)
arteriovenous (747.32)
retinal (743.58)
Excludes: congenital central alveolar hypoventilation syndrome (327.25)
congenital defect of diaphragm (756.6)
Atresia of nares (anterior) (posterior)
Congenital stenosis of nares (anterior) (posterior)
Absent nose
Accessory nose
Cleft nose
Deformity of wall of nasal sinus
Congenital:
deformity of nose
notching of tip of nose
perforation of wall of nasal sinus
Excludes: congenital deviation of nasal septum (754.0)
Web of larynx:
NOS
glottic
subglottic
Absence or agenesis of:
bronchus
larynx
trachea
Anomaly (of):
cricoid cartilage
epiglottis
thyroid cartilage
tracheal cartilage
Atresia (of):
epiglottis
glottis
larynx
trachea
Cleft thyroid, cartilage, congenital
Congenital:
dilation, trachea
stenosis:
larynx
trachea
tracheocele
Diverticulum:
bronchus
trachea
Fissure of epiglottis
Laryngocele
Posterior cleft of cricoid cartilage (congenital)
Rudimentary tracheal bronchus
Stridor, laryngeal, congenital
Disease, lung:
cystic, congenital
polycystic, congenital
Honeycomb lung, congenital
Excludes: acquired or unspecified cystic lung (518.89)
Absence of lung (fissures) (lobe)
Aplasia of lung
Hypoplasia of lung (lobe)
Sequestration of lung
Accessory lung (lobe)
Azygos lobe (fissure), lung
Abnormal communication between pericardial and pleural sacs
Anomaly, pleural folds
Atresia of nasopharynx
Congenital cyst of mediastinum
Anomaly of respiratory system NOS
Cleft uvula
Cheiloschisis
Congenital fissure of lip
Harelip
Labium leporinum
Cheilopalatoschisis
