Classification of diseases and injuries


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740 Anencephalus and similar anomalies

740.0 Anencephalus





740.1 Craniorachischisis

740.2 Iniencephaly

741 Spina bifida

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


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


Hydromeningocele (spinal)


Meningocele (spinal)





Spina bifida (aperta)


742 Other congenital anomalies of nervous system

Excludes: congenital central alveolar hypoventilation syndrome (327.25)

742.0 Encephalocele




Hydromeningocele, cranial

Meningocele, cerebral


742.1 Microcephalus



742.2 Reduction deformities of brain

Absence of part of brain

Agenesis of part of brain


Aplasia of part of brain



Hypoplasia of part of brain


742.3 Congenital hydrocephalus

Aqueduct of Sylvius:


obstruction, congenital


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




Multiple anomalies of brain NOS



742.5 Other specified anomalies of spinal cord

742.51 Diastematomyelia

742.53 Hydromyelia


742.59 Other



Congenital anomaly of spinal meninges

Defective development of cauda equina

Hypoplasia of spinal cord



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

743.0 Anophthalmos

743.00 Clinical anophthalmos, unspecified


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





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



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


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





743.45 Aniridia

743.46 Other specified anomalies of iris and ciliary body

Anisocoria, congenital

Atresia of pupil

Coloboma of iris


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


Absence of eyelid

Accessory eyelid




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


anomaly NOS of eye [any part]

deformity NOS of eye [any part]

744 Congenital anomalies of ear, face, and neck

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


Preauricular appendage




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


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


sinus (external) (internal)


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


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


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

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:



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


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

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:


arising from aorta or pulmonary trunk


746.86 Congenital heart block

Complete or incomplete atrioventricular [AV] block

746.87 Malposition of heart and cardiac apex

Abdominal heart


Ectopia cordis

Levocardia (isolated)


Excludes: dextrocardia with complete transposition of viscera (759.3)

746.89 Other

Atresia of cardiac vein

Hypoplasia of cardiac vein



diverticulum, left ventricle

pericardial defect

746.9 Unspecified anomaly of heart


anomaly of heart NOS

heart disease NOS

747 Other congenital anomalies of circulatory system

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


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


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]:



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)


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


aneurysm (peripheral)


stricture, artery


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

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


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:




748.3 Other anomalies of larynx, trachea, and bronchus

Absence or agenesis of:




Anomaly (of):

cricoid cartilage


thyroid cartilage

tracheal cartilage

Atresia (of):





Cleft thyroid, cartilage, congenital


dilation, trachea








Fissure of epiglottis


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

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


Congenital fissure of lip


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


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

750 Other congenital anomalies of upper alimentary tract

Excludes: dentofacial anomalies (524.0-524.9)

750.0 Tongue tie


750.1 Other anomalies of tongue

750.10 Anomaly of tongue, unspecified

750.11 Aglossia

750.12 Congenital adhesions of tongue

750.13 Fissure of tongue

Bifid tongue

Double tongue

750.15 Macroglossia

Congenital hypertrophy of tongue

750.16 Microglossia

Hypoplasia of tongue

750.19 Other

750.2 Other specified anomalies of mouth and pharynx

750.21 Absence of salivary gland

750.22 Accessory salivary gland

750.23 Atresia, salivary gland

Imperforate salivary duct

750.24 Congenital fistula of salivary gland

750.25 Congenital fistula of lip

Congenital (mucus) lip pits

750.26 Other specified anomalies of mouth

Absence of uvula

750.27 Diverticulum of pharynx

Pharyngeal pouch

750.29 Other specified anomalies of pharynx

Imperforate pharynx

750.3 Tracheoesophageal fistula, esophageal atresia and stenosis

Absent esophagus

Atresia of esophagus


esophageal ring

stenosis of esophagus

stricture of esophagus

Congenital fistula:



Imperforate esophagus

Webbed esophagus

750.4 Other specified anomalies of esophagus

Dilatation, congenital, of esophagus

Displacement, congenital, of esophagus

Diverticulum of esophagus

Duplication of esophagus

Giant esophagus

Esophageal pouch

Excludes: congenital hiatus hernia (750.6)

750.5 Congenital hypertrophic pyloric stenosis

Congenital or infantile:

constriction of pylorus

hypertrophy of pylorus

spasm of pylorus

stenosis of pylorus

stricture of pylorus

750.6 Congenital hiatus hernia

Displacement of cardia through esophageal hiatus

Excludes: congenital diaphragmatic hernia (756.6)

750.7 Other specified anomalies of stomach



hourglass stomach

Displacement of stomach

Diverticulum of stomach, congenital

Duplication of stomach



Transposition of stomach

750.8 Other specified anomalies of upper alimentary tract

750.9 Unspecified anomaly of upper alimentary tract


anomaly NOS of upper alimentary tract [any part, except tongue]

deformity NOS of upper alimentary tract [any part, except tongue]

751 Other congenital anomalies of digestive system

751.0 Meckel's diverticulum

Meckel's diverticulum (displaced) (hypertrophic)


omphalomesenteric duct

vitelline duct

751.1 Atresia and stenosis of small intestine

Atresia of:



intestine NOS


absence of small intestine or intestine NOS

obstruction of small intestine or intestine NOS

stenosis of small intestine or intestine NOS

stricture of small intestine or intestine NOS

Imperforate jejunum

751.2 Atresia and stenosis of large intestine, rectum, and anal canal


anus (congenital)

appendix, congenital

large intestine, congenital


Atresia of:




Congenital or infantile:

obstruction of large intestine

occlusion of anus

stricture of anus




Stricture of rectum, congenital

751.3 Hirschsprung's disease and other congenital functional disorders of colon


Congenital dilation of colon

Congenital megacolon


751.4 Anomalies of intestinal fixation

Congenital adhesions:

omental, anomalous


Jackson's membrane

Malrotation of colon

Rotation of cecum or colon:

failure of



Universal mesentery

751.5 Other anomalies of intestine

Congenital diverticulum, colon


Duplication of:





Ectopic anus




Persistent cloaca

Transposition of:




751.6 Anomalies of gallbladder, bile ducts, and liver

751.60 Unspecified anomaly of gallbladder, bile ducts, and liver

751.61 Biliary atresia


absence of bile duct (common) or passage

hypoplasia of bile duct (common) or passage

obstruction of bile duct (common) or passage

stricture of bile duct (common) or passage

751.62 Congenital cystic disease of liver

Congenital polycystic disease of liver

Fibrocystic disease of liver

751.69 Other anomalies of gallbladder, bile ducts, and liver

Absence of:

gallbladder, congenital

liver (lobe)


hepatic ducts



choledochal cyst


Duplication of:

biliary duct

cystic duct






Intrahepatic gallbladder

751.7 Anomalies of pancreas

Absence of pancreas

Accessory pancreas

Agenesis of pancreas

Annular pancreas

Ectopic pancreatic tissue

Hypoplasia of pancreas

Pancreatic heterotopia

Excludes: diabetes mellitus (249.0-249.9, 250.0-250.9)

fibrocystic disease of pancreas (277.00-277.09)

neonatal diabetes mellitus (775.1)

751.8 Other specified anomalies of digestive system

Absence (complete) (partial) of alimentary tract NOS

Duplication of digestive organs NOS

Malposition, congenital of digestive organs NOS

Excludes: congenital diaphragmatic hernia (756.6)

congenital hiatus hernia (750.6)

751.9 Unspecified anomaly of digestive system


anomaly NOS of digestive system NOS

deformity NOS of digestive system NOS

752 Congenital anomalies of genital organs

Excludes: syndromes associated with anomalies in the number and form of chromosomes (758.0-758.9)

752.0 Anomalies of ovaries

Absence, congenital, of ovary

Accessory ovary

Ectopic ovary

Streak of ovary

752.1 Anomalies of fallopian tubes and broad ligaments

752.10 Unspecified anomaly of fallopian tubes and broad ligaments

752.11 Embryonic cyst of fallopian tubes and broad ligaments





752.19 Other

Absence of fallopian tube or broad ligament

Accessory fallopian tube or broad ligament

Atresia of fallopian tube or broad ligament

752.2 Doubling of uterus

Didelphic uterus

Doubling of uterus [any degree] (associated with doubling of cervix and vagina)

752.3 Other anomalies of uterus

752.31 Agenesis of uterus

Congenital absence of uterus

752.32 Hypoplasia of uterus

752.33 Unicornuate uterus

Unicornate uterus with or without a separate uterine horn

Uterus with only one functioning horn

752.34 Bicornuate uterus

Bicornuate uterus, complete or partial

752.35 Septate uterus

Septate uterus, complete or partial

752.36 Arcuate uterus

752.39 Other anomalies of uterus

Aplasia of uterus NOS

Müllerian anomaly of the uterus, NEC

Excludes: anomaly of uterus due to exposure to diethylstilbestrol [DES] in utero (760.76)

didelphic uterus (752.2)

doubling of uterus (752.2)

752.4 Anomalies of cervix, vagina, and external female genitalia

752.40 Unspecified anomaly of cervix, vagina, and external female genitalia

752.41 Embryonic cyst of cervix, vagina, and external female genitalia

Cyst of:

canal of Nuck, congenital

Gartner's duct

vagina, embryonal

vulva, congenital

752.42 Imperforate hymen

752.43 Cervical agenesis

Cervical hypoplasia

752.44 Cervical duplication

752.45 Vaginal agenesis

Agenesis of vagina, total or partial

752.46 Transverse vaginal septum

752.47 Longitudinal vaginal septum

Longitudinal vaginal septum with or without obstruction

752.49 Other anomalies of cervix, vagina, and external female genitalia

Absence of clitoris or vulva

Agenesis of clitoris or vulva

Anomalies of cervix, NEC

Anomalies of hymen, NEC

Congenital stenosis or stricture of:

cervical canal


Müllerian anomalies of the cervix and vagina, NEC

Excludes: double vagina associated with total duplication (752.2)

752.5 Undescended and retractile testicle

752.51 Undescended testis


Ectopic testis

752.52 Retractile testis

752.6 Hypospadias and epispadias and other penile anomalies

752.61 Hypospadias

752.62 Epispadias


752.63 Congenital chordee

752.64 Micropenis

752.65 Hidden penis

752.69 Other penile anomalies

752.7 Indeterminate sex and pseudohermaphroditism




Pseudohermaphroditism (male) (female)

Pure gonadal dysgenesis

Excludes: androgen insensitivity (259.50-259.52)


female, with adrenocortical disorder (255.2)

male, with gonadal disorder (257.8)

with specified chromosomal anomaly (758.0-758.9)

testicular feminization syndrome (259.50-259.52)

752.8 Other specified anomalies of genital organs

Excludes: congenital hydrocele (778.6)

penile anomalies (752.61-752.69)

phimosis or paraphimosis (605)

752.81 Scrotal transposition

752.89 Other specified anomalies of genital organs

Absence of:


spermatic cord

vas deferens


Aplasia (congenital) of:


round ligament


Atresia of:

ejaculatory duct

vas deferens

Fusion of testes

Hypoplasia of testis



752.9 Unspecified anomaly of genital organs


anomaly NOS of genital organ, NEC

deformity NOS of genital organ, NEC

753 Congenital anomalies of urinary system

753.0 Renal agenesis and dysgenesis

Atrophy of kidney:



Congenital absence of kidney(s)

Hypoplasia of kidney(s)

753.1 Cystic kidney disease

Excludes: acquired cyst of kidney (593.2)

753.10 Cystic kidney disease, unspecified

753.11 Congenital single renal cyst

753.12 Polycystic kidney, unspecified type

753.13 Polycystic kidney, autosomal dominant

753.14 Polycystic kidney, autosomal recessive

753.15 Renal dysplasia

753.16 Medullary cystic kidney


753.17 Medullary sponge kidney

753.19 Other specified cystic kidney disease

Multicystic kidney

753.2 Obstructive defects of renal pelvis and ureter

753.20 Unspecified obstructive defect of renal pelvis and ureter

753.21 Congenital obstruction of ureteropelvic junction

753.22 Congenital obstruction of ureterovesical junction

Adynamic ureter

Congenital hydroureter

753.23 Congenital ureterocele

753.29 Other

753.3 Other specified anomalies of kidney

Accessory kidney


calculus of kidney

displaced kidney

Discoid kidney

Double kidney with double pelvis

Ectopic kidney

Fusion of kidneys

Giant kidney

Horseshoe kidney

Hyperplasia of kidney

Lobulation of kidney

Malrotation of kidney

Trifid kidney (pelvis)

753.4 Other specified anomalies of ureter

Absent ureter

Accessory ureter

Deviation of ureter

Displaced ureteric orifice

Double ureter

Ectopic ureter

Implantation, anomalous, of ureter

753.5 Exstrophy of urinary bladder

Ectopia vesicae

Extroversion of bladder

753.6 Atresia and stenosis of urethra and bladder neck

Congenital obstruction:

bladder neck


Congenital stricture of:

urethra (valvular)

urinary meatus

vesicourethral orifice

Imperforate urinary meatus

Impervious urethra

Urethral valve formation

753.7 Anomalies of urachus

Cyst (of) urachus

Fistula (of) urachus

Patent (of) urachus

Persistent umbilical sinus

753.8 Other specified anomalies of bladder and urethra

Absence, congenital of:







diverticulum of bladder

hernia of bladder

Congenital urethrorectal fistula

Congenital prolapse of:

bladder (mucosa)




urinary meatus

753.9 Unspecified anomaly of urinary system


anomaly NOS of urinary system [any part, except urachus]

deformity NOS of urinary system [any part, except urachus]

754 Certain congenital musculoskeletal deformities

Includes: nonteratogenic deformities which are considered to be due to intrauterine malposition and pressure

754.0 Of skull, face, and jaw

Asymmetry of face

Compression facies

Depressions in skull

Deviation of nasal septum, congenital



Potter's facies

Squashed or bent nose, congenital

Excludes: dentofacial anomalies (524.0-524.9)

syphilitic saddle nose (090.5)

754.1 Of sternocleidomastoid muscle

Congenital sternomastoid torticollis

Congenital wryneck

Contracture of sternocleidomastoid (muscle)

Sternomastoid tumor

754.2 Of spine

Congenital postural:



754.3 Congenital dislocation of hip

754.30 Congenital dislocation of hip, unilateral

Congenital dislocation of hip NOS

754.31 Congenital dislocation of hip, bilateral

754.32 Congenital subluxation of hip, unilateral

Congenital flexion deformity, hip or thigh

Predislocation status of hip at birth

Preluxation of hip, congenital

754.33 Congenital subluxation of hip, bilateral

754.35 Congenital dislocation of one hip with subluxation of other hip

754.4 Congenital genu recurvatum and bowing of long bones of leg

754.40 Genu recurvatum

754.41 Congenital dislocation of knee (with genu recurvatum)

754.42 Congenital bowing of femur

754.43 Congenital bowing of tibia and fibula

754.44 Congenital bowing of unspecified long bones of leg

754.5 Varus deformities of feet

Excludes: acquired (736.71, 736.75, 736.79)

754.50 Talipes varus

Congenital varus deformity of foot, unspecified

Pes varus

754.51 Talipes equinovarus

Equinovarus (congenital)

754.52 Metatarsus primus varus

754.53 Metatarsus varus

754.59 Other

Talipes calcaneovarus

754.6 Valgus deformities of feet

Excludes: valgus deformity of foot (acquired) (736.79)

754.60 Talipes valgus

Congenital valgus deformity of foot, unspecified

754.61 Congenital pes planus

Congenital rocker bottom flat foot

Flat foot, congenital

Excludes: pes planus (acquired) (734)

754.62 Talipes calcaneovalgus

754.69 Other




754.7 Other deformities of feet

Excludes: acquired (736.70-736.79)

754.70 Talipes, unspecified

Congenital deformity of foot NOS

754.71 Talipes cavus

Cavus foot (congenital)

754.79 Other

Asymmetric talipes




754.8 Other specified nonteratogenic anomalies

754.81 Pectus excavatum

Congenital funnel chest

754.82 Pectus carinatum

Congenital pigeon chest [breast]

754.89 Other

Club hand (congenital)


deformity of chest wall

dislocation of elbow

Generalized flexion contractures of lower limb joints, congenital

Spade-like hand (congenital)

755 Other congenital anomalies of limbs

Excludes: those deformities classifiable to 754.0-754.8

755.0 Polydactyly

755.00 Polydactyly, unspecified digits

Supernumerary digits

755.01 Of fingers

Accessory fingers

755.02 Of toes

Accessory toes

755.1 Syndactyly


Webbing of digits

755.10 Of multiple and unspecified sites

755.11 Of fingers without fusion of bone

755.12 Of fingers with fusion of bone

755.13 Of toes without fusion of bone

755.14 Of toes with fusion of bone

755.2 Reduction deformities of upper limb

755.20 Unspecified reduction deformity of upper limb

Ectromelia NOS of upper limb

Hemimelia NOS of upper limb

Shortening of arm, congenital

755.21 Transverse deficiency of upper limb

Amelia of upper limb

Congenital absence of:

fingers, all (complete or partial)

forearm, including hand and fingers

upper limb, complete

Congenital amputation of upper limb

Transverse hemimelia of upper limb

755.22 Longitudinal deficiency of upper limb, NEC

Phocomelia NOS of upper limb

Rudimentary arm

755.23 Longitudinal deficiency, combined, involving humerus, radius, and ulna (complete or incomplete)

Congenital absence of arm and forearm (complete or incomplete) with or without metacarpal deficiency and/or phalangeal deficiency, incomplete

Phocomelia, complete, of upper limb

755.24 Longitudinal deficiency, humeral, complete or partial (with or without distal deficiencies, incomplete)

Congenital absence of humerus (with or without absence of some [but not all] distal elements)

Proximal phocomelia of upper limb

755.25 Longitudinal deficiency, radioulnar, complete or partial (with or without distal deficiencies, incomplete)

Congenital absence of radius and ulna (with or without absence of some [but not all] distal elements)

Distal phocomelia of upper limb

755.26 Longitudinal deficiency, radial, complete or partial (with or without distal deficiencies, incomplete)

Agenesis of radius

Congenital absence of radius (with or without absence of some [but not all] distal elements)

755.27 Longitudinal deficiency, ulnar, complete or partial (with or without distal deficiencies, incomplete)

Agenesis of ulna

Congenital absence of ulna (with or without absence of some [but not all] distal elements)

755.28 Longitudinal deficiency, carpals or metacarpals, complete or partial (with or without incomplete phalangeal deficiency)

755.29 Longitudinal deficiency, phalanges, complete or partial

Absence of finger, congenital

Aphalangia of upper limb, terminal, complete or partial

Excludes: terminal deficiency of all five digits (755.21)

transverse deficiency of phalanges (755.21)

755.3 Reduction deformities of lower limb

755.30 Unspecified reduction deformity of lower limb

Ectromelia NOS of lower limb

Hemimelia NOS of lower limb

Shortening of leg, congenital

755.31 Transverse deficiency of lower limb

Amelia of lower limb

Congenital absence of:


leg, including foot and toes

lower limb, complete

toes, all, complete

Transverse hemimelia of lower limb

755.32 Longitudinal deficiency of lower limb, NEC

Phocomelia NOS of lower limb

755.33 Longitudinal deficiency, combined, involving femur, tibia, and fibula (complete or incomplete)

Congenital absence of thigh and (lower) leg (complete or incomplete) with or without metacarpal deficiency and/or phalangeal deficiency, incomplete

Phocomelia, complete, of lower limb

755.34 Longitudinal deficiency, femoral, complete or partial (with or without distal deficiencies, incomplete)

Congenital absence of femur (with or without absence of some [but not all] distal elements)

Proximal phocomelia of lower limb

755.35 Longitudinal deficiency, tibiofibular, complete or partial (with or without distal deficiencies, incomplete)

Congenital absence of tibia and fibula (with or without absence of some [but not all] distal elements)

Distal phocomelia of lower limb

755.36 Longitudinal deficiency, tibia, complete or partial (with or without distal deficiencies, incomplete)

Agenesis of tibia

Congenital absence of tibia (with or without absence of some [but not all] distal elements)

755.37 Longitudinal deficiency, fibular, complete or partial (with or without distal deficiencies, incomplete)

Agenesis of fibula

Congenital absence of fibula (with or without absence of some [but not all] distal elements)

755.38 Longitudinal deficiency, tarsals or metatarsals, complete or partial (with or without incomplete phalangeal deficiency)

755.39 Longitudinal deficiency, phalanges, complete or partial

Absence of toe, congenital

Aphalangia of lower limb, terminal, complete or partial

Excludes: terminal deficiency of all five digits (755.31)

transverse deficiency of phalanges (755.31)

755.4 Reduction deformities, unspecified limb

Absence, congenital (complete or partial) of limb NOS

Amelia of unspecified limb

Ectromelia of unspecified limb

Hemimelia of unspecified limb

Phocomelia of unspecified limb

755.5 Other anomalies of upper limb, including shoulder girdle

755.50 Unspecified anomaly of upper limb

755.51 Congenital deformity of clavicle

755.52 Congenital elevation of scapula

Sprengel's deformity

755.53 Radioulnar synostosis

755.54 Madelung's deformity

755.55 Acrocephalosyndactyly

Apert's syndrome

755.56 Accessory carpal bones

755.57 Macrodactylia (fingers)

755.58 Cleft hand, congenital

Lobster-claw hand

755.59 Other

Cleidocranial dysostosis


valgus, congenital

varus, congenital

Excludes: club hand (congenital) (754.89)

congenital dislocation of elbow (754.89)

755.6 Other anomalies of lower limb, including pelvic girdle

755.60 Unspecified anomaly of lower limb

755.61 Coxa valga, congenital

755.62 Coxa vara, congenital

755.63 Other congenital deformity of hip (joint)

Congenital anteversion of femur (neck)

Excludes: congenital dislocation of hip (754.30-754.35)

755.64 Congenital deformity of knee (joint)


absence of patella

genu valgum [knock-knee]

genu varum [bowleg]

Rudimentary patella

755.65 Macrodactylia of toes

755.66 Other anomalies of toes


hallux valgus

hallux varus

hammer toe

755.67 Anomalies of foot, NEC

Astragaloscaphoid synostosis

Calcaneonavicular bar

Coalition of calcaneus

Talonavicular synostosis

Tarsal coalitions

755.69 Other


angulation of tibia

deformity (of):

ankle (joint)

sacroiliac (joint)

fusion of sacroiliac joint

755.8 Other specified anomalies of unspecified limb

755.9 Unspecified anomaly of unspecified limb


anomaly NOS of unspecified limb

deformity NOS of unspecified limb

Excludes: reduction deformity of unspecified limb (755.4)

756 Other congenital musculoskeletal anomalies

Excludes: congenital myotonic chondrodystrophy (359.23)

those deformities classifiable to 754.0-754.8

756.0 Anomalies of skull and face bones

Absence of skull bones


Congenital deformity of forehead


Crouzon's disease


Imperfect fusion of skull



Premature closure of cranial sutures

Tower skull


Excludes: acrocephalosyndactyly [Apert's syndrome] (755.55)

dentofacial anomalies (524.0-524.9)

skull defects associated with brain anomalies, such as:

anencephalus (740.0)

encephalocele (742.0)

hydrocephalus (742.3)

microcephalus (742.1)

756.1 Anomalies of spine

756.10 Anomaly of spine, unspecified

756.11 Spondylolysis, lumbosacral region

Prespondylolisthesis (lumbosacral)

756.12 Spondylolisthesis

756.13 Absence of vertebra, congenital

756.14 Hemivertebra

756.15 Fusion of spine [vertebra], congenital

756.16 Klippel-Feil syndrome

756.17 Spina bifida occulta

Excludes: spina bifida (aperta) (741.0-741.9)

756.19 Other


Supernumerary vertebra

756.2 Cervical rib

Supernumerary rib in the cervical region

756.3 Other anomalies of ribs and sternum

Congenital absence of:




fissure of sternum

fusion of ribs

Sternum bifidum

Excludes: nonteratogenic deformity of chest wall (754.81-754.89)

756.4 Chondrodystrophy


Chondrodystrophia (fetalis)



Ollier's disease

Excludes: congenital myotonic chondrodystrophy (359.23)

lipochondrodystrophy [Hurler's syndrome] (277.5)

Morquio's disease (277.5)

756.5 Osteodystrophies

756.50 Osteodystrophy, unspecified

756.51 Osteogenesis imperfecta

Fragilitas ossium


756.52 Osteopetrosis

756.53 Osteopoikilosis

756.54 Polyostotic fibrous dysplasia of bone

756.55 Chondroectodermal dysplasia

Ellis-van Creveld syndrome

756.56 Multiple epiphyseal dysplasia

756.59 Other

Albright (-McCune)-Sternberg syndrome

756.6 Anomalies of diaphragm

Absence of diaphragm

Congenital hernia:


foramen of Morgagni

Eventration of diaphragm

Excludes: congenital hiatus hernia (750.6)

756.7 Anomalies of abdominal wall

756.70 Anomaly of abdominal wall, unspecified

756.71 Prune belly syndrome

Eagle-Barrett syndrome

Prolapse of bladder mucosa

756.72 Omphalocele


756.73 Gastroschisis

756.79 Other congenital anomalies of abdominal wall

Excludes: umbilical hernia (551-553 with.1)

756.8 Other specified anomalies of muscle, tendon, fascia, and connective tissue

756.81 Absence of muscle and tendon

Absence of muscle (pectoral)

756.82 Accessory muscle

756.83 Ehlers-Danlos syndrome

756.89 Other

Amyotrophia congenita

Congenital shortening of tendon

756.9 Other and unspecified anomalies of musculoskeletal system


anomaly NOS of musculoskeletal system, NEC

deformity NOS of musculoskeletal system, NEC

757 Congenital anomalies of the integument

Includes: anomalies of skin, subcutaneous tissue, hair, nails, and breast

Excludes: hemangioma (228.00-228.09)

pigmented nevus (216.0-216.9)

757.0 Hereditary edema of legs

Congenital lymphedema

Hereditary trophedema

Milroy's disease

757.1 Ichthyosis congenita

Congenital ichthyosis

Harlequin fetus

Ichthyosiform erythroderma

757.2 Dermatoglyphic anomalies

Abnormal palmar creases

757.3 Other specified anomalies of skin

757.31 Congenital ectodermal dysplasia

757.32 Vascular hamartomas


Port-wine stain

Strawberry nevus

757.33 Congenital pigmentary anomalies of skin

Congenital poikiloderma

Urticaria pigmentosa

Xeroderma pigmentosum

Excludes: albinism (270.2)

757.39 Other

Accessory skin tags, congenital

Congenital scar

Epidermolysis bullosa

Keratoderma (congenital)

Excludes: pilonidal cyst (685.0-685.1)

757.4 Specified anomalies of hair




beaded hair



Persistent lanugo

757.5 Specified anomalies of nails








757.6 Specified congenital anomalies of breast

Accessory breast or nipple

Congenital absent breast or nipple

Supernumerary breast or nipple

Excludes: absence of pectoral muscle (756.81)

hypoplasia of breast (611.82)

micromastia (611.82)

757.8 Other specified anomalies of the integument

757.9 Unspecified anomaly of the integument


anomaly NOS of integument

deformity NOS of integument

758 Chromosomal anomalies

Includes: syndromes associated with anomalies in the number and form of chromosomes

Use additional codes for conditions associated with the chromosomal anomalies

758.0 Down's syndrome


Translocation Down's syndrome


21 or 22


758.1 Patau's syndrome




758.2 Edward's syndrome




758.3 Autosomal deletion syndromes

758.31 Cri-du-chat syndrome

Deletion 5p

758.32 Velo-cardio-facial syndrome

Deletion 22q11.2

758.33 Other microdeletions

Miller-Dieker syndrome

Smith-Magenis syndrome

758.39 Other autosomal deletions

758.4 Balanced autosomal translocation in normal individual

758.5 Other conditions due to autosomal anomalies

Accessory autosomes NEC

758.6 Gonadal dysgenesis

Ovarian dysgenesis

Turner's syndrome

XO syndrome

Excludes: pure gonadal dysgenesis (752.7)

758.7 Klinefelter's syndrome

XXY syndrome

758.8 Other conditions due to chromosome anomalies

758.81 Other conditions due to sex chromosome anomalies

758.89 Other

758.9 Conditions due to anomaly of unspecified chromosome

759 Other and unspecified congenital anomalies

759.0 Anomalies of spleen

Aberrant spleen

Absent spleen

Accessory spleen

Congenital splenomegaly

Ectopic spleen

Lobulation of spleen

759.1 Anomalies of adrenal gland

Aberrant adrenal gland

Absent adrenal gland

Accessory adrenal gland

Excludes: adrenogenital disorders (255.2)

congenital disorders of steroid metabolism (255.2)

759.2 Anomalies of other endocrine glands

Absent parathyroid gland

Accessory thyroid gland

Persistent thyroglossal or thyrolingual duct

Thyroglossal (duct) cyst

Excludes: congenital:

goiter (246.1)

hypothyroidism (243)

759.3 Situs inversus

Situs inversus or transversus:



Transposition of viscera:



Excludes: dextrocardia without mention of complete transposition (746.87)

759.4 Conjoined twins






759.5 Tuberous sclerosis

Bourneville's disease


759.6 Other hamartoses, NEC



Sturge-Weber (-Dimitri)

von Hippel-Lindau

Excludes: neurofibromatosis (237.70-237.79)

759.7 Multiple congenital anomalies, so described


anomaly, multiple NOS

deformity, multiple NOS

759.8 Other specified anomalies

759.81 Prader-Willi syndrome

759.82 Marfan syndrome

759.83 Fragile X syndrome

759.89 Other

Congenital malformation syndromes affecting multiple systems, NEC

Laurence-Moon-Biedl syndrome

759.9 Congenital anomaly, unspecified

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