Congenital Anomalies ICD 9 Codes (740-759)


14.  CONGENITAL ANOMALIES ICD 9 CODES(740-759)
740       Anencephalus and similar anomalies  (ICD 9 CM Codes)
740.0    Anencephalus
Acrania
Amyelencephalus
Hemianencephaly
Hemicephaly
740.1    Craniorachischisis
740.2    Iniencephaly
741       Spina bifida   (ICD 9 CM Codes)
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
741.0    With hydrocephalus
[0-3]
Arnold-Chiari syndrome, type II
Chiari malformation, type II
Any condition classifiable to 741.9 with any condition classifiable to 742.3
741.9    Without mention of hydrocephalus
[0-3]
Hydromeningocele (spinal)
Hydromyelocele
Meningocele (spinal)
Meningomyelocele
Myelocele
Myelocystocele
Rachischisis
Spina bifida (aperta)
Syringomyelocele
742       Other congenital anomalies of nervous system    (ICD 9 CM Codes)
Excludes:    congenital central alveolar hypoventilation syndrome (327.25)
742.0    Encephalocele
Encephalocystocele
Encephalomyelocele
Hydroencephalocele
Hydromeningocele, cranial
Meningocele, cerebral
Meningoencephalocele
742.1    Microcephalus
Hydromicrocephaly
Micrencephaly
742.2    Reduction deformities of brain
Absence of part of brain
Agenesis of part of brain
Agyria
Aplasia of part of brain
Arhinencephaly
Holoprosencephaly
Hypoplasia of part of brain
Microgyria
742.3    Congenital hydrocephalus
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)
742.4    Other specified anomalies of brain
Congenital cerebral cyst
Macroencephaly
Macrogyria
Megalencephaly
Multiple anomalies of brain NOS
Porencephaly
Ulegyria
742.5    Other specified anomalies of spinal cord
742.51  Diastematomyelia
742.53  Hydromyelia
Hydrorhachis
742.59  Other
Amyelia
Atelomyelia
Congenital anomaly of spinal meninges
Defective development of cauda equina
Hypoplasia of spinal cord
Myelatelia
Myelodysplasia
742.8    Other specified anomalies of nervous system
Agenesis of nerve
Displacement of brachial plexus
Familial dysautonomia
Jaw-winking syndrome
Marcus-Gunn syndrome
Riley-Day syndrome
Excludes: neurofibromatosis (237.70-237.79)
742.9    Unspecified anomaly of brain, spinal cord, and nervous system
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
743       Congenital anomalies of eye    (ICD 9 CM Codes)
743.0    Anophthalmos
743.00  Clinical anophthalmos, unspecified
Agenesis
Congenital absence of eye
Anophthalmos NOS
743.03  Cystic eyeball, congenital
743.06  Cryptophthalmos
743.1    Microphthalmos
Dysplasia of eye
Hypoplasia of eye
Rudimentary eye
743.10  Microphthalmos, unspecified
743.11  Simple microphthalmos
743.12  Microphthalmos associated with other anomalies of eye and adnexa
743.2    Buphthalmos
Glaucoma:
congenital
newborn
Hydrophthalmos
Excludes: glaucoma of childhood (365.14)
traumatic glaucoma due to birth injury (767.8)
743.20  Buphthalmos, unspecified
743.21  Simple buphthalmos
743.22  Buphthalmos associated with other ocular anomalies
Keratoglobus, congenital, associated with buphthalmos
Megalocornea associated with buphthalmos
743.3    Congenital cataract and lens anomalies
Excludes: infantile cataract (366.00-366.09)
743.30  Congenital cataract, unspecified
743.31  Capsular and subcapsular cataract
743.32  Cortical and zonular cataract
743.33  Nuclear cataract
743.34  Total and subtotal cataract, congenital
743.35  Congenital aphakia
Congenital absence of lens
743.36  Anomalies of lens shape
Microphakia
Spherophakia
743.37  Congenital ectopic lens
743.39  Other
743.4    Coloboma and other anomalies of anterior segment
743.41  Anomalies of corneal size and shape
Microcornea
Excludes: that associated with buphthalmos (743.22)
743.42  Corneal opacities, interfering with vision, congenital
743.43  Other corneal opacities, congenital
743.44  Specified anomalies of anterior chamber, chamber angle, and related structures
Anomaly:
Axenfeld's
Peters'
Rieger's
743.45  Aniridia
743.46  Other specified anomalies of iris and ciliary body
Anisocoria, congenital
Atresia of pupil
Coloboma of iris
Corectopia
743.47  Specified anomalies of sclera
743.48  Multiple and combined anomalies of anterior segment
743.49  Other
743.5    Congenital anomalies of posterior segment
743.51  Vitreous anomalies
Congenital vitreous opacity
743.52  Fundus coloboma
743.53  Chorioretinal degeneration, congenital
743.54  Congenital folds and cysts of posterior segment
743.55  Congenital macular changes
743.56  Other retinal changes, congenital
743.57  Specified anomalies of optic disc
Coloboma of optic disc (congenital)
743.58  Vascular anomalies
Congenital retinal aneurysm
743.59  Other
743.6    Congenital anomalies of eyelids, lacrimal system, and orbit
743.61  Congenital ptosis
743.62  Congenital deformities of eyelids
Ablepharon
Absence of eyelid
Accessory eyelid
Congenital:
ectropion
entropion
743.63  Other specified congenital anomalies of eyelid
Absence, agenesis, of cilia
743.64  Specified congenital anomalies of lacrimal gland
743.65  Specified congenital anomalies of lacrimal passages
Absence, agenesis of:
lacrimal apparatus
punctum lacrimale
Accessory lacrimal canal
743.66  Specified congenital anomalies of orbit
743.69  Other
Accessory eye muscles
743.8    Other specified anomalies of eye
Excludes: congenital nystagmus (379.51)
ocular albinism (270.2)
optic nerve hypoplasia (377.43)
retinitis pigmentosa (362.74)
743.9    Unspecified anomaly of eye
Congenital:
anomaly NOS of eye [any part]
deformity NOS of eye [any part]
744       Congenital anomalies of ear, face, and neck    (ICD 9 CM Codes)
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)
744.0    Anomalies of ear causing impairment of hearing
Excludes: congenital deafness without mention of cause (380.0-389.9)
744.00  Unspecified anomaly of ear with impairment of hearing
744.01  Absence of external ear
Absence of:
auditory canal (external)
auricle (ear) (with stenosis or atresia of auditory canal)
744.02  Other anomalies of external ear with impairment of hearing
Atresia or stricture of auditory canal (external)
744.03  Anomaly of middle ear, except ossicles
Atresia or stricture of osseous meatus (ear)
744.04  Anomalies of ear ossicles
Fusion of ear ossicles
744.05  Anomalies of inner ear
Congenital anomaly of:
membranous labyrinth
organ of Corti
744.09  Other
Absence of ear, congenital
744.1    Accessory auricle
Accessory tragus
Polyotia
Preauricular appendage
Supernumerary:
ear
lobule
744.2    Other specified anomalies of ear
Excludes: that with impairment of hearing (744.00-744.09)
744.21  Absence of ear lobe, congenital
744.22  Macrotia
744.23  Microtia
744.24  Specified anomalies of Eustachian tube
Absence of Eustachian tube
744.29  Other
Bat ear
Darwin's tubercle
Pointed ear
Prominence of auricle
Ridge ear
Excludes: preauricular sinus (744.46)
744.3    Unspecified anomaly of ear
Congenital:
anomaly NOS of ear, NEC
deformity NOS of ear, NEC
744.4    Branchial cleft cyst or fistula; preauricular sinus
744.41  Branchial cleft sinus or fistula
Branchial:
sinus (external) (internal)
vestige
744.42  Branchial cleft cyst
744.43  Cervical auricle
744.46  Preauricular sinus or fistula
744.47  Preauricular cyst
744.49  Other
Fistula (of):
auricle, congenital
cervicoaural
744.5    Webbing of neck
Pterygium colli
744.8    Other specified anomalies of face and neck
744.81  Macrocheilia
Hypertrophy of lip, congenital
744.82  Microcheilia
744.83  Macrostomia
744.84  Microstomia
744.89  Other
Excludes: congenital fistula of lip (750.25)
musculoskeletal anomalies (754.0-754.1, 756.0)
744.9    Unspecified anomalies of face and neck
Congenital:
anomaly NOS of face [any part] or neck [any part]
deformity NOS of face [any part] or neck [any part]
745       Bulbus cordis anomalies and anomalies of cardiac septal closure   (ICD 9 CM Codes)
745.0    Common truncus
Absent septum between aorta and pulmonary artery
Communication (abnormal) between aorta and pulmonary artery
Aortic septal defect
Common aortopulmonary trunk
Persistent truncus arteriosus
745.1    Transposition of great vessels
745.10  Complete transposition of great vessels
Transposition of great vessels:
NOS
classical
745.11  Double outlet right ventricle
Dextratransposition of aorta
Incomplete transposition of great vessels
Origin of both great vessels from right ventricle
Taussig-Bing syndrome or defect
745.12  Corrected transposition of great vessels
745.19  Other
745.2    Tetralogy of Fallot
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)
745.3    Common ventricle
Cor triloculare biatriatum
Single ventricle
745.4    Ventricular septal defect
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)
745.5    Ostium secundum type atrial septal defect
Defect:
atrium secundum
fossa ovalis
Lutembacher's syndrome
Patent or persistent:
foramen ovale
ostium secundum
745.6    Endocardial cushion defects
745.60  Endocardial cushion defect, unspecified type
745.61  Ostium primum defect
Persistent ostium primum
745.69  Other
Absence of atrial septum
Atrioventricular canal type ventricular septal defect
Common atrioventricular canal
Common atrium
745.7    Cor biloculare
Absence of atrial and ventricular septa
745.8    Other
745.9    Unspecified defect of septal closure
Septal defect NOS
746       Other congenital anomalies of heart   (ICD 9 CM Codes)
Excludes:    endocardial fibroelastosis (425.3)
746.0    Anomalies of pulmonary valve
Excludes: infundibular or subvalvular pulmonic stenosis (746.83)
tetralogy of Fallot (745.2)
746.00  Pulmonary valve anomaly, unspecified
746.01  Atresia, congenital
Congenital absence of pulmonary valve
746.02  Stenosis, congenital
746.09  Other
Congenital insufficiency of pulmonary valve
Fallot's triad or trilogy
746.1    Tricuspid atresia and stenosis, congenital
Absence of tricuspid valve
746.2    Ebstein's anomaly
746.3    Congenital stenosis of aortic valve
Congenital aortic stenosis
Excludes: congenital:
subaortic stenosis (746.81)
supravalvular aortic stenosis (747.22)
746.4    Congenital insufficiency of aortic valve
Bicuspid aortic valve
Congenital aortic insufficiency
746.5    Congenital mitral stenosis
Fused commissure of mitral valve
Parachute deformity of mitral valve
Supernumerary cusps of mitral valve
746.6    Congenital mitral insufficiency
746.7    Hypoplastic left heart syndrome
Atresia, or marked hypoplasia, of aortic orifice or valve, with hypoplasia of ascending aorta and defective development of left ventricle (with mitral valve atresia)
746.8    Other specified anomalies of heart
746.81  Subaortic stenosis
746.82  Cor triatriatum
746.83  Infundibular pulmonic stenosis
Subvalvular pulmonic stenosis
746.84  Obstructive anomalies of heart, NEC
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)
746.85  Coronary artery anomaly
Anomalous origin or communication of coronary artery
Arteriovenous malformation of coronary artery
Coronary artery:
absence
arising from aorta or pulmonary trunk
single
746.86  Congenital heart block
Complete or incomplete atrioventricular [AV] block
746.87  Malposition of heart and cardiac apex
Abdominal heart
Dextrocardia
Ectopia cordis
Levocardia (isolated)
Mesocardia
Excludes: dextrocardia with complete transposition of viscera (759.3)
746.89  Other
Atresia of cardiac vein
Hypoplasia of cardiac vein
Congenital:
cardiomegaly
diverticulum, left ventricle
pericardial defect
746.9    Unspecified anomaly of heart
Congenital:
anomaly of heart NOS
heart disease NOS
747       Other congenital anomalies of circulatory system    (ICD 9 CM Codes)
747.0    Patent ductus arteriosus
Patent ductus Botalli
Persistent ductus arteriosus
747.1    Coarctation of aorta
747.10  Coarctation of aorta (preductal) (postductal)
Hypoplasia of aortic arch
747.11  Interruption of aortic arch
747.2    Other anomalies of aorta
747.20  Anomaly of aorta, unspecified
747.21  Anomalies 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)
747.22  Atresia and stenosis of aorta
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)
747.29  Other
Aneurysm of sinus of Valsalva
Congenital:
aneurysm of aorta
dilation of aorta
747.3    Anomalies of pulmonary artery
747.31  Pulmonary artery coarctation and atresia
Agenesis of pulmonary artery
Atresia of pulmonary artery
Coarctation of pulmonary artery
Hypoplasia of pulmonary artery
Stenosis of pulmonary artery
747.32  Pulmonary arteriovenous malformation
Pulmonary arteriovenous aneurysm
Excludes: acquired pulmonary arteriovenous fistula (417.0)
747.39  Other anomalies of pulmonary artery and pulmonary circulation
Anomaly of pulmonary artery
747.4    Anomalies of great veins
747.40  Anomaly of great veins, unspecified
Anomaly NOS of:
pulmonary veins
vena cava
747.41  Total anomalous pulmonary venous connection
Total anomalous pulmonary venous return [TAPVR]:
subdiaphragmatic
supradiaphragmatic
747.42  Partial anomalous pulmonary venous connection
Partial anomalous pulmonary venous return
747.49  Other anomalies of great veins
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
747.5    Absence or hypoplasia of umbilical artery
Single umbilical artery
747.6    Other anomalies of peripheral vascular system
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)
747.60  Anomaly of the peripheral vascular system, unspecified site
747.61  Gastrointestinal vessel anomaly
747.62  Renal vessel anomaly
747.63  Upper limb vessel anomaly
747.64  Lower limb vessel anomaly
747.69  Anomalies of other specified sites of peripheral vascular system
747.8    Other specified anomalies of circulatory system
747.81  Anomalies of cerebrovascular system
Arteriovenous malformation of brain
Cerebral arteriovenous aneurysm, congenital
Congenital anomalies of cerebral vessels
Excludes: ruptured cerebral (arteriovenous) aneurysm (430)
747.82  Spinal vessel anomaly
Arteriovenous malformation of spinal vessel
747.83  Persistent fetal circulation
Persistent pulmonary hypertension
Primary pulmonary hypertension of newborn
747.89  Other
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)
747.9    Unspecified anomaly of circulatory system
748       Congenital anomalies of respiratory system   (ICD 9 CM Codes)
Excludes:    congenital central alveolar hypoventilation syndrome (327.25)
congenital defect of diaphragm (756.6)
748.0    Choanal atresia
Atresia of nares (anterior) (posterior)
Congenital stenosis of nares (anterior) (posterior)
748.1    Other anomalies of nose
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)
748.2    Web of larynx
Web of larynx:
NOS
glottic
subglottic
748.3    Other anomalies of larynx, trachea, and bronchus
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
748.4    Congenital cystic lung
Disease, lung:
cystic, congenital
polycystic, congenital
Honeycomb lung, congenital
Excludes: acquired or unspecified cystic lung (518.89)
748.5    Agenesis, hypoplasia, and dysplasia of lung
Absence of  lung (fissures) (lobe)
Aplasia of lung
Hypoplasia of lung (lobe)
Sequestration of lung
748.6    Other anomalies of lung
748.60  Anomaly of lung, unspecified
748.61  Congenital bronchiectasis
748.69  Other
Accessory lung (lobe)
Azygos lobe (fissure), lung
748.8    Other specified anomalies of respiratory system
Abnormal communication between pericardial and pleural sacs
Anomaly, pleural folds
Atresia of nasopharynx
Congenital cyst of mediastinum
748.9    Unspecified anomaly of respiratory system
Anomaly of respiratory system NOS
749       Cleft palate and cleft lip    (ICD 9 CM Codes)
749.0    Cleft palate
749.00  Cleft palate, unspecified
749.01  Unilateral, complete
749.02  Unilateral, incomplete
Cleft uvula
749.03  Bilateral, complete
749.04  Bilateral, incomplete
749.1    Cleft lip
Cheiloschisis
Congenital fissure of lip
Harelip
Labium leporinum
749.10  Cleft lip, unspecified
749.11  Unilateral, complete
749.12  Unilateral, incomplete
749.13  Bilateral, complete
749.14  Bilateral, incomplete
749.2    Cleft palate with cleft lip
Cheilopalatoschisis
749.20  Cleft palate with cleft lip, unspecified
749.21  Unilateral, complete
749.22  Unilateral, incomplete
749.23  Bilateral, complete
749.24  Bilateral, incomplete
749.25  Other combinations

(ICD 9 CM Codes)

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