740	Anencephalus and similar anomalies
740.0	Anencephalus
Acrania
Amyelencephalus
Hemianencephaly
Hemicephaly
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)
Hydromyelocele
Meningocele (spinal)
Meningomyelocele
Myelocele
Myelocystocele
Rachischisis
Spina bifida (aperta)
Syringomyelocele
742	Other congenital anomalies of nervous system
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 (237.7)
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
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
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
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
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
Uhl's disease
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
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
Agenesis of pulmonary artery
Anomaly of pulmonary artery
Atresia of pulmonary artery
Coarctation of pulmonary artery
Hypoplasia of pulmonary artery
Stenosis of pulmonary artery
Pulmonary arteriovenous aneurysm
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 (747.3)
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 (747.3)
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
Congenital:
deformity of nose
notching of tip of nose
performation 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
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
750	Other congenital anomalies of upper alimentary tract
Excludes:	dentofacial anomalies (524.0-524.9)
750.0	Tongue tie
Ankyloglossia
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
Congenital:
esophageal ring
stenosis of esophagus
stricture of esophagus
Congenital fistula:
esophagobronchial
esophagotracheal
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
Congenital:
cardiospasm
hourglass stomach
Displacement of stomach
Diverticulum of stomach, congenital
Duplication of stomach
Megalogastria
Microgastria
Transposition of stomach
750.8	Other specified anomalies of upper alimentary tract
750.9	Unspecified anomaly of upper alimentary tract
Congenital:
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)
Persistent:
omphalomesenteric duct
vitelline duct
751.1	Atresia and stenosis of small intestine
Atresia of:
duodenum
ileum
intestine NOS
Congenital:
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
Absence:
anus (congenital)
appendix, congenital
large instestine, congenital
rectum
Atresia of:
anus
colon
rectum
Congenital or infantile:
obstruction of large intestine
occlusion of anus
stricture of anus
Imperforate:
anus
rectum
Stricture of rectum, congenital
751.3	Hirschsprung's disease and other congenital functional disorders of colon
Aganglionosis
Congenital dilation of colon
Congenital megacolon
Marcrocolon
751.4	Anomalies of intestinal fixation
Congenital adhesions:
omental, anomalous
peritoneal
Jackson's membrane
Malrotation of colon
Rotation of cecum or colon:
failure of
incomplete
insufficient
Universal mesentery
751.5	Other anomalies of intestine
Congenital diverticulum, colon
Dolichocolon
Duplication of:
anus
appendix
cecum
intestine
Ectopic anus
Megaloappendix
Megaloduodenum
Microcolon
Persistent cloaca
Transposition of:
appendix
colon
intestine
751.6	Anomalies of gallbladder, bile ducts, and liver
751.60	Unspecified anomaly of gallbladder, bile ducts, and liver
751.61	Biliary atresia
Congenital:
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)
Accessory:
hepatic ducts
liver
Congenital:
choledochal cyst
hepatomegaly
Duplication of:
biliary duct
cystic duct
gallbladder
liver
Floating:
gallbladder
liver
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:
congenital (250.0-250.9)
neonatal (775.1)
fibrocystic disease of pancreas (277.00-277.09)
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
Congenital:
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)
testicular feminization syndrome (259.5)
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
Cyst:
epoophoron
fimbrial
parovarian
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
Absence, congenital, of uterus
Agenesis of uterus
Aplasia of uterus
Bicornuate uterus
Uterus unicornis
Uterus with only one functioning horn
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
752.49	Other anomalies of cervix, vagina, and external female genitalia
Absence of cervix, clitoris, vagina, or vulva
Agenesis of cervix, clitoris, vagina, or vulva
Congenital stenosis or stricture of:
cervical canal
vagina
Excludes:	double vagina associated with total duplication (752.2)
752.5	Undescended and retractile testicle
752.51	Undescended testis
Cryptorchism
Ectopic testis
752.52	Retractile testis
752.6	Hypospadias and epispadias and other penile anomalies
752.61	Hypospadias
752.62	Epispadias
Anaspadias
752.63	Congenital chordee
752.64	Micropenis
752.65	Hidden penis
752.69	Other penile anomalies
752.7	Indeterminate sex and pseudohermaphroditism
Gynandrism
Hermaphroditism
Ovotestis
Pseudohermaphroditism (male) (female)
Pure gonadal dysgenesis
Excludes:	pseudohermaphroditism:
female, with adrenocortical disorder (255.2)
male, with gonadal disorder (257.8)
with specified chromosomal anomaly (758.0-758.9)
testicular feminization syndrome (259.5)
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:
prostate
spermatic cord
vas deferens
Anorchism
Aplasia (congenital) of:
prostate
round ligament
testicle
Atresia of:
ejaculatory duct
vas deferens
Fusion of testes
Hypoplasia of testis
Monorchism
Polyorchism
752.9	Unspecified anomaly of genital organs
Congenital:
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
infantile
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
Nephronopthisis
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
Congenital:
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
Deviaton 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
urethra
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:
bladder
urethra
Accessory:
bladder
urethra
Congenital:
diverticulum of bladder
hernia of bladder
Congenital urethrorectal fistula
Congenital prolapse of:
bladder (mucosa)
urethra
Double:
urethra
urinary meatus
753.9	Unspecified anomaly of urinary system
Congenital:
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
Dolichocephaly
Plagiocephaly
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:
lordosis
scoliosis
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
Talipes:
equinovalgus
planovalgus
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
Talipes:
calcaneus
equinus
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)
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
Symphalangy
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:
foot
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
Cubitus:
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)
Congenital:
absence of patella
genu valgum [knock-knee]
genu varum [bowleg]
Rudimentary patella
755.65	Macrodactylia of toes
755.66	Other anomalies of toes
Congenital:
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
Congenital:
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
Congenital:
anomaly NOS of unspecified limb
deformity NOS of unspecified limb
Excludes:	reduction deformity of unspecified limb (755.4)
756	Other congenital musculoskeletal anomalies
Excludes:	those deformities classifiable to 754.0-754.8
756.0	Anomalies of skull and face bones
Absence of skull bones
Acrocephaly
Congenital deformity of forehead
Craniosynostosis
Crouzon's disease
Hypertelorism
Imperfect fusion of skull
Oxycephaly
Platybasia
Premature closure of cranial sutures
Tower skull
Trigonocephaly
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
Platyspondylia
Supernumerary vertebra
756.2	Cervical rib
Supernumerary rib in the cervical region
756.3	Other anomalies of ribs and sternum
Congenital absence of:
rib
sternum
Congenital:
fissure of sternum
fusion of ribs
Sternum bifidum
Excludes:	nonteratogenic deformity of chest wall (754.81-754.89)
756.4	Chondrodystrophy
Achondroplasia
Chondrodystrophia (fetalis)
Dyschondroplasia
Enchondromatosis
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
Osteopsathyrosis
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:
diaphragmatic
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
756.79	Other congenital anomalies of abdominal wall
Exomphalos
Gastroschisis
Omphalocele
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
Congenital:
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
Birthmarks
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
Congenital:
alopecia
atrichosis
beaded hair
hypertrichosis
monilethrix
Persistent lanugo
757.5	Specified anomalies of nails
Anonychia
Congenital:
clubnail
koilonychia
leukonychia
onychauxis
pachyonychia
757.6	Specified anomalies of breast
Absent breast or nipple
Accessory breast or nipple
Supernumerary breast or nipple
Hypoplasia of breast
Excludes:	absence of pectoral muscle (756.81)
757.8	Other specified anomalies of the integument
757.9	Unspecified anomaly of the integument
Congenital:
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
Mongolism
Translocation Down's syndrome
Trisomy:
21 or 22
G
758.1	Patau's syndrome
Trisomy:
13
D1
758.2	Edward's syndrome
Trisomy:
18
E3
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:
abdominalis
thoracis
Transposition of viscera:
abdominal
thoracic
Excludes:	dextrocardia without mention of complete transposition (746.87)
759.4	Conjoined twins
Craniopagus
Dicephalus
Pygopagus
Thoracopagus
Xiphopagus
759.5	Tuberous sclerosis
Bourneville's disease
Epiloia
759.6	Other hamartoses, NEC
Syndrome:
Peutz-Jeghers
Sturge-Weber (-Dimitri)
von Hippel-Lindau
Excludes:	neurofibromatosis (237.7)
759.7	Multiple congenital anomalies, so described
Congenital:
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