List of ICD-9 codes 740–759: congenital anomalies

14. Congenital anomalies (740–759)

Nervous system (740-742)

(740) Anencephalus and similar anomalies
- (740.0) Anencephalus
(741) Spina bifida
(742) Other congenital anomalies of nervous system
- (742.1) Microcephalus
- (742.3) Hydrocephalus

Eye, ear, face and neck (743-744)

(743) Congenital anomalies of eye
- (743.0) Anophthalmos
  - (743.00) Clinical anophthalmos unspecified
  - (743.03) Cystic eyeball congenital
  - (743.06) Cryptophthalmos
- (743.1) Microphthalmos
- (743.2) Buphthalmos
- (743.3) Congenital cataract and lens anomalies
- (743.4) Coloboma and other anomalies of anterior segment
  - (743.45) Aniridia
- (743.5) Congenital anomalies of posterior segment
- (743.6) Congenital anomalies of eyelids, lacrimal system, and orbit
(744) Congenital anomalies of ear, face, and neck
- (744.0) Anomalies of ear causing impairment of hearing
- (744.1) Accessory auricle
- (744.2) Other specified congenital anomalies of ear
  - (744.22) Macrotia
  - (744.23) Microtia
- (744.3) Unspecified congenital anomaly of ear
- (744.4) Branchial cleft cyst or fistula; preauricular sinus
- (744.5) Webbing of neck
- (744.8) Other specified congenital anomalies of face and neck
  - (744.81) Macrocheilia
  - (744.82) Microcheilia
  - (744.83) Macrostomia
  - (744.84) Microstomia

Circulatory system (745-747)

(745) Bulbus cordis anomalies and anomalies of cardiac septal closure
- (745.0) Common truncus
- (745.1) Transposition of great vessels
- (745.2) Tetralogy of fallot
- (745.3) Common ventricle
- (745.4) Ventricular septal defect
- (745.5) Atrial septal defect
- (745.6) Endocardial cushion defects
- (745.7) Cor biloculare
(746) Other congenital anomalies of heart
- (746.1) Tricuspid atresia and stenosis congenital
- (746.2) Ebstein's anomaly
- (746.3) Congenital stenosis of aortic valve
- (746.4) Congenital insufficiency of aortic valve
- (746.5) Congenital mitral stenosis
- (746.6) Congenital mitral insufficiency
- (746.7) Hypoplastic left heart syndrome
- (746.8) Other specified congenital anomalies of heart
  - (746.81) Subaortic stenosis congenital
  - (746.82) Cor triatriatum
  - (746.83) Infundibular pulmonic stenosis congenital
  - (746.84) Congenital obstructive anomalies of heart not elsewhere classified
  - (746.85) Coronary artery anomaly congenital
  - (746.86) Congenital heart block
  - (746.87) Malposition of heart and cardiac apex
  - (746.89) Other specified congenital anomalies of heart
    - Brugada syndrome
(747) Other congenital anomalies of circulatory system
- (747.1) Coarctation of aorta
  - (747.11) Interruption of aortic arch
- (747.2) Other congenital anomalies of aorta
- (747.3) Congenital anomalies of pulmonary artery
- (747.4) Congenital anomalies of great veins
- (747.5) Absence or hypoplasia of umbilical artery
- (747.6) Arteriovenous malformation, unspec.
- (747.8) Other specified anomalies of circulatory system
  - (747.81) Congenital anomalies of cerebrovascular system
  - (747.82) Spinal vessel anomaly
  - (747.83) Persistent fetal circulation
- (747.9) Unspecified congenital anomaly of circulatory system

Respiratory system (748-748)

(748) Congenital anomalies of respiratory system
- (748.0) Choanal atresia

Digestive system (749-751)

(749) Cleft palate
- (749.0) Cleft palate, unspec.
- (749.2) Cleft palate w/ cleft lip
(750) Other congenital anomalies of upper alimentary tract
- (750.0) Tongue tie
- (750.1) Other congenital anomalies of tongue
- (750.2) Other specified congenital anomalies of mouth and pharynx
- (750.3) Congenital tracheoesophageal fistula esophageal atresia and stenosis
- (750.4) Other specified congenital anomalies of esophagus
- (750.5) Congenital hypertrophic pyloric stenosis
- (750.6) Congenital hiatus hernia
- (750.7) Other specified congenital anomalies of stomach
- (750.8) Other specified congenital anomalies of upper alimentary tract
- (750.9) Unspecified congenital anomaly of upper alimentary tract
(751) Other congenital anomalies of digestive system
- (751.0) Meckel's diverticulum
- (751.1) Congenital atresia and stenosis of small intestine
- (751.2) Imperforate anus
- (751.3) Hirschsprung's disease
- (751.4) Congenital anomalies of intestinal fixation
- (751.5) Other congenital anomalies of intestine
- (751.6) Anomalies of gallbladder bile ducts and liver
- (751.7) Congenital anomalies of pancreas
- (751.8) Other specified congenital anomalies of digestive system
- (751.9) Unspecified congenital anomaly of digestive system

Neural tube defect	Anencephaly Acephaly Acrania Acalvaria Iniencephaly Encephalocele Arnold–Chiari malformation

Other	Microcephaly Congenital hydrocephalus Dandy–Walker syndrome other reduction deformities Holoprosencephaly Lissencephaly Pachygyria Hydranencephaly Septo-optic dysplasia Megalencephaly CNS cyst Porencephaly Schizencephaly Polymicrogyria Bilateral frontoparietal polymicrogyria

Spinal cord

Neural tube defect	Spina bifida Rachischisis

Other	Currarino syndrome Diastomatomyelia Syringomyelia

Congenital malformations and deformations of eyes (Q10–Q15, 743)

Adnexa

Eyelid	Ptosis Ectropion Entropion Distichia Blepharophimosis Ablepharon Marcus Gunn phenomenon

Lacrimal apparatus	Congenital lacrimal duct obstruction

Globe

Entire eye	Anophthalmia (Cystic eyeball, Cryptophthalmos) Microphthalmia

Lens	Ectopia lentis Aphakia

Iris	Aniridia

Anterior segment	Axenfeld syndrome

Cornea	Keratoglobus Megalocornea

Other	Buphthalmos Coloboma (Coloboma of optic nerve) Hydrophthalmos Norrie disease

Congenital malformations and deformations of ears (Q16–Q17, 744.0–744.3)

Size	Macrotia Microtia Anotia

Position	Low-set ears

Other	Accessory auricle Mondini dysplasia

Congenital malformations and deformations of face and neck (Q18, 744.4–744.9)

Face	jaw: Otocephaly mouth: Macrostomia Microstomia lip: Macrocheilia Microcheilia chin: Microgenia multiple/other: Hallermann–Streiff syndrome Branchial cleft cyst

Neck	Webbed neck

Ungrouped	Preauricular sinus and cyst

Congenital heart defects (Q20–Q24, 745–746)

Cardiac shunt/
heart septal defect

Aortopulmonary septal defect	R→L: Double outlet right ventricle Taussig–Bing syndrome Transposition of the great vessels dextro levo Persistent truncus arteriosus Aortopulmonary window

Atrial septal defect	L→R: Sinus venosus atrial septal defect Lutembacher's syndrome

Ventricular septal defect	L→R and R→L: Eisenmenger's syndrome R→L, with other conditions: Tetralogy of Fallot

Atrioventricular septal defect	L→R: Ostium primum

Valvular heart disease/
heart chambers

Right	pulmonary valves stenosis insufficiency tricuspid valves stenosis atresia Ebstein's anomaly Hypoplastic right heart syndrome Uhl anomaly

Left	aortic valves stenosis insufficiency bicuspid mitral valves stenosis regurgitation Hypoplastic left heart syndrome

Other

Congenital vascular defects / Vascular malformation (Q25–Q28, 747)

Great arteries/
other arteries

Aorta	Patent ductus arteriosus Coarctation of the aorta Interrupted aortic arch Double aortic arch Right-sided aortic arch Overriding aorta Aneurysm of sinus of Valsalva Vascular ring

Pulmonary artery	Pulmonary atresia Stenosis of pulmonary artery

Subclavian artery	Aberrant subclavian artery

Umbilical artery	Single umbilical artery

Great veins

Superior/inferior vena cava	Congenital stenosis of vena cava Persistent left superior vena cava

Pulmonary vein	Anomalous pulmonary venous connection (Total, Partial) Scimitar syndrome

Arteriovenous malformation

Cerebral arteriovenous malformation

Congenital malformations and deformations of respiratory system (Q30–Q34, 748)

Upper RT

Nose	Choanal atresia Arrhinia

Larynx	Laryngeal cyst Laryngocele Laryngomalacia

Lower RT

Trachea and bronchus	Tracheomalacia Tracheal stenosis Bronchomalacia Tracheobronchomegaly

Lung	Bronchiectasis Pulmonary hypoplasia Pulmonary sequestration Congenital cystic adenomatoid malformation

Congenital malformations and deformations of digestive system (Q35–Q45, 749–751)

Upper GI tract

Tongue, mouth and pharynx	Cleft lip and palate Van der Woude syndrome tongue Ankyloglossia Macroglossia Hypoglossia

Esophagus	EA/TEF Esophageal atresia: types A, B, C, and D Tracheoesophageal fistula: types B, C, D and E esophageal rings Esophageal web (upper) Schatzki ring (lower)

Stomach	Pyloric stenosis Hiatus hernia

Lower GI tract

Intestines	Intestinal atresia Duodenal atresia Meckel's diverticulum Hirschsprung's disease Intestinal malrotation Dolichocolon Enteric duplication cyst

Rectum/anal canal	Imperforate anus Rectovestibular fistula Persistent cloaca Rectal atresia

Accessory

Pancreas	Annular pancreas Accessory pancreas Johanson–Blizzard syndrome Pancreas divisum

Bile duct	Choledochal cysts Caroli disease Biliary atresia

Liver	Alagille syndrome Polycystic liver disease

Female congenital anomalies of the genitalia, including Intersex and DSD: (Q50–Q52; 752.0–752.4)

Internal

Uterine malformation	Müllerian agenesis Cervical agenesis Unicornuate uterus Uterus didelphys Bicornuate uterus Uterine septum Arcuate uterus

Vagina	Vaginal septum Vaginal hypoplasia

External

Male congenital anomalies of the genitalia, including Intersex and DSD: (Q53–Q56 752.5–752.7)

Internal

Testicle

Cryptorchidism Polyorchidism Monorchism Anorchia Sertoli cell-only syndrome

True hermaphroditism Mixed gonadal dysgenesis Swyer syndrome

Vas deferens

Congenital absence of the vas deferens

Other

Persistent Müllerian duct syndrome

External

Penis	Hypospadias Epispadias Chordee Micropenis Penile agenesis Diphallia

Other	Pseudohermaphroditism

Congenital malformations and deformations of urinary system (Q60–Q64, 753)

Abdominal

Kidney	Renal agenesis/Potter sequence, Papillorenal syndrome cystic Polycystic kidney disease Meckel syndrome Multicystic dysplastic kidney Medullary sponge kidney Horseshoe kidney Renal ectopia Nephronophthisis Supernumerary kidney Dent's disease Alport syndrome

Ureter	Ectopic ureter Megaureter Duplicated ureter

Pelvic

Bladder	Bladder exstrophy

Urethra	Epispadias Hypospadias Posterior urethral valves

Vestigial

Urachus	Urachal cyst Urachal fistula

Congenital malformations and deformations of musculoskeletal system / musculoskeletal abnormality (Q65–Q76, 754–756.3)

Appendicular
limb / dysmelia

Arms

clavicle / shoulder:	Cleidocranial dysostosis Sprengel's deformity Wallis–Zieff–Goldblatt syndrome

hand deformity:	Madelung's deformity Clinodactyly Oligodactyly Polydactyly

Leg

hip:	Dislocation of hip / Hip dysplasia Upington disease Coxa valga Coxa vara

knee:	Genu valgum Genu varum Genu recurvatum Discoid meniscus Congenital patellar dislocation Congenital knee dislocation

foot deformity:	varus Club foot Pigeon toe valgus Flat feet Pes cavus Rocker bottom foot Hammer toe

Either / both

fingers and toes	Polydactyly / Syndactyly webbed toes Arachnodactyly Cenani–Lenz syndactylism Ectrodactyly Brachydactyly Clubbed thumb

reduction deficits / limb:	Acheiropodia ectromelia Phocomelia Amelia Hemimelia

multiple joints:	Arthrogryposis Larsen syndrome Rapadilino syndrome

Axial

Skull and face

Craniosynostosis:	Scaphocephaly Oxycephaly Trigonocephaly

Craniofacial dysostosis:	Crouzon syndrome Hypertelorism Hallermann–Streiff syndrome Treacher Collins syndrome

other:	Macrocephaly Platybasia Craniodiaphyseal dysplasia Dolichocephaly Greig cephalopolysyndactyly syndrome Plagiocephaly Saddle nose

Vertebral column

Thoracic skeleton

ribs:	Cervical Bifid

sternum:	Pectus excavatum Pectus carinatum

Osteochondrodysplasia (Q77–Q78, 756.4–756.5)

Osteodysplasia//
osteodystrophy

Diaphysis	Camurati–Engelmann disease

Metaphysis	Metaphyseal dysplasia Jansen's metaphyseal chondrodysplasia Schmid metaphyseal chondrodysplasia

Epiphysis	Spondyloepiphyseal dysplasia congenita Multiple epiphyseal dysplasia Otospondylomegaepiphyseal dysplasia

Osteosclerosis	Raine syndrome Osteopoikilosis Osteopetrosis

Other/ungrouped	FLNB Boomerang dysplasia Opsismodysplasia Polyostotic fibrous dysplasia McCune–Albright syndrome

Chondrodysplasia/
chondrodystrophy
(including dwarfism)

Osteochondroma

osteochondromatosis
- Hereditary multiple exostoses

Chondroma/enchondroma

enchondromatosis
- Ollier disease
- Maffucci syndrome

Growth factor receptor

FGFR2:	Antley–Bixler syndrome

FGFR3:	Achondroplasia Hypochondroplasia Thanatophoric dysplasia

COL2A1 collagen disease

SLC26A2 sulfation defect

Chondrodysplasia punctata

Other dwarfism

Congenital diaphragm and abdominal wall defects, abdominopelvic cavity (Q79.0–Q79.5, 756.6–756.7)

Thoracic diaphragm	Hernia Congenital diaphragmatic hernia Bochdalek hernia

Abdominal wall	Omphalocele Gastroschisis Prune belly syndrome

Congenital malformations and deformations of integument / skin disease (Q80–Q82, 757.0–757.3)

Genodermatosis

Congenital ichthyosis/
erythrokeratodermia

AD	Ichthyosis vulgaris

AR	Congenital ichthyosiform erythroderma: Epidermolytic hyperkeratosis Lamellar ichthyosis Harlequin type ichthyosis Netherton syndrome Zunich–Kaye syndrome Sjögren–Larsson syndrome

XR	X-linked ichthyosis

Ungrouped	Ichthyosis bullosa of Siemens Ichthyosis follicularis Ichthyosis prematurity syndrome Ichthyosis–sclerosing cholangitis syndrome Nonbullous congenital ichthyosiform erythroderma Ichthyosis linearis circumflexa Ichthyosis hystrix

EB
and related

EBS
- EBS-K
- EBS-WC
- EBS-DM
- EBS-OG
- EBS-MD
- EBS-MP

DEB
- DDEB
- RDEB

Ectodermal dysplasia

Elastic/Connective

Hyperkeratosis/
keratinopathy

PPK	diffuse: Diffuse epidermolytic palmoplantar keratoderma Diffuse nonepidermolytic palmoplantar keratoderma Palmoplantar keratoderma of Sybert Mal de Meleda syndromic connexin Bart–Pumphrey syndrome Clouston's hidrotic ectodermal dysplasia Vohwinkel syndrome Corneodermatoosseous syndrome plakoglobin Naxos syndrome Scleroatrophic syndrome of Huriez Olmsted syndrome Cathepsin C Papillon–Lefèvre syndrome Haim–Munk syndrome Camisa disease focal: Focal palmoplantar keratoderma with oral mucosal hyperkeratosis Focal palmoplantar and gingival keratosis Howel–Evans syndrome Pachyonychia congenita Pachyonychia congenita type I Pachyonychia congenita type II Striate palmoplantar keratoderma Tyrosinemia type II punctate: Acrokeratoelastoidosis of Costa Focal acral hyperkeratosis Keratosis punctata palmaris et plantaris Keratosis punctata of the palmar creases Schöpf–Schulz–Passarge syndrome Porokeratosis plantaris discreta Spiny keratoderma ungrouped: Palmoplantar keratoderma and spastic paraplegia desmoplakin Carvajal syndrome connexin Erythrokeratodermia variabilis HID/KID

Other	Meleda disease Keratosis pilaris ATP2A2 Darier's disease Dyskeratosis congenita Lelis syndrome Dyskeratosis congenita Keratolytic winter erythema Keratosis follicularis spinulosa decalvans Keratosis linearis with ichthyosis congenital and sclerosing keratoderma syndrome Keratosis pilaris atrophicans faciei Keratosis pilaris

Other

see also Template:Congenital malformations and deformations of skin appendages, Template:Phakomatoses, Template:Pigmentation disorders, Template:DNA replication and repair-deficiency disorder

Developmental
anomalies

Midline	Dermoid cyst Encephalocele Nasal glioma PHACE association Sinus pericranii

Nevus	Capillary hemangioma Port-wine stain Nevus flammeus nuchae

Other/ungrouped	Aplasia cutis congenita Amniotic band syndrome Branchial cyst Cavernous venous malformation Accessory nail of the fifth toe Bronchogenic cyst Congenital cartilaginous rest of the neck Congenital hypertrophy of the lateral fold of the hallux Congenital lip pit Congenital malformations of the dermatoglyphs Congenital preauricular fistula Congenital smooth muscle hamartoma Cystic lymphatic malformation Median raphe cyst Melanotic neuroectodermal tumor of infancy Mongolian spot Nasolacrimal duct cyst Omphalomesenteric duct cyst Poland anomaly Rapidly involuting congenital hemangioma Rosenthal–Kloepfer syndrome Skin dimple Superficial lymphatic malformation Thyroglossal duct cyst Verrucous vascular malformation Birthmark

Congenital malformations and deformations of skin appendages (Q84, 757.4–757.5)

Nail disease	Anonychia Leukonychia Pachyonychia congenita/Onychauxis Koilonychia

Hair disease	hypotrichosis/abnormalities: keratin disease Monilethrix IBIDS syndrome Sabinas brittle hair syndrome Pili annulati Pili torti Uncombable hair syndrome Björnstad syndrome Giant axonal neuropathy with curly hair hypertrichosis: Zimmermann–Laband syndrome

Congenital malformations and deformations of the breast (Q83, 757.6)

Breast	Amastia Polymastia Micromastia Symmastia

Nipple	Athelia Polythelia

Chromosome abnormalities (Q90–Q99, 758)

Autosomal

Trisomies	Down syndrome 21 Edwards syndrome 18 Patau syndrome 13 Trisomy 9 Warkany syndrome 2 8 Cat eye syndrome/Trisomy 22 22 Trisomy 16

Monosomies/deletions	1q21.1 deletion syndrome/1q21.1 duplication syndrome/TAR syndrome 1 Wolf–Hirschhorn syndrome 4 Cri du chat/Chromosome 5q deletion syndrome 5 Williams syndrome 7 Jacobsen syndrome 11 Miller–Dieker syndrome/Smith–Magenis syndrome 17 DiGeorge syndrome 22 22q11.2 distal deletion syndrome 22 22q13 deletion syndrome 22 genomic imprinting Angelman syndrome/Prader–Willi syndrome (15) Distal 18q-/Proximal 18q-

X/Y linked

Monosomy	Turner syndrome (45,X)

Trisomy/tetrasomy, other karyotypes/mosaics	Klinefelter syndrome (47,XXY) 48,XXYY 48,XXXY 49,XXXYY 49,XXXXY Triple X syndrome (47,XXX) 48,XXXX 49,XXXXX 47,XYY 48,XYYY 49,XYYYY 45,X/46,XY

Translocations

Leukemia/lymphoma

Lymphoid	Burkitt's lymphoma t(8 MYC;14 IGH) Follicular lymphoma t(14 IGH;18 BCL2) Mantle cell lymphoma/Multiple myeloma t(11 CCND1:14 IGH) Anaplastic large-cell lymphoma t(2 ALK;5 NPM1) Acute lymphoblastic leukemia

Myeloid	Philadelphia chromosome t(9 ABL; 22 BCR) Acute myeloblastic leukemia with maturation t(8 RUNX1T1;21 RUNX1) Acute promyelocytic leukemia t(15 PML,17 RARA) Acute megakaryoblastic leukemia t(1 RBM15;22 MKL1)

Other

Ewing's sarcoma t(11 FLI1; 22 EWS)
Synovial sarcoma t(x SYT;18 SSX)
Dermatofibrosarcoma protuberans t(17 COL1A1;22 PDGFB)
Myxoid liposarcoma t(12 DDIT3; 16 FUS)
Desmoplastic small-round-cell tumor t(11 WT1; 22 EWS)
Alveolar rhabdomyosarcoma t(2 PAX3; 13 FOXO1) t (1 PAX7; 13 FOXO1)

Other

Lymphatic disease: Congenital lymphatic organ disorders (Q89.0/Q89.2, 759.0/759.2)

Thymus	Thymic hypoplasia DiGeorge syndrome Ectopic thymus

Spleen	Congenital asplenia/hyposplenism Asplenia with cardiovascular anomalies Accessory spleen Polysplenia

Congenital endocrine disorders (Q89.1–Q89.2, 759.1–759.2)

Pituitary	Congenital hypopituitarism

Thyroid	Thyroid disease Persistent thyroglossal duct Thyroglossal cyst Congenital hypothyroidism Thyroid dysgenesis Thyroid dyshormonogenesis Pendred syndrome

Parathyroid	Congenital absence of parathyroid

Adrenal	Absent adrenal gland

Twin conditions (Q89.3–Q89.4, 759.3–759.4)

Zygosity	Twin Half twin

Monochorionic twins	Twin-to-twin transfusion syndrome Twin reversed arterial perfusion Monoamniotic twins

Conjoined twins	Parasitic twin (Craniopagus parasiticus, Fetus in fetu)

Other	Vanishing twin Chimerism Mixed twins

Phakomatosis (Q85, 759.5–759.6)

Angiomatosis	Sturge–Weber syndrome Von Hippel–Lindau disease

Hamartoma	Tuberous sclerosis Hypothalamic hamartoma (Pallister–Hall syndrome) Multiple hamartoma syndrome Proteus syndrome Cowden syndrome Bannayan–Riley–Ruvalcaba syndrome Lhermitte–Duclos disease

Neurofibromatosis	Type I Type II

Other	Abdallat–Davis–Farrage syndrome Ataxia telangiectasia Incontinentia pigmenti Peutz–Jeghers syndrome

Congenital abnormality syndromes (Q87, 759.7)

Craniofacial

Acrocephalosyndactylia Apert syndrome Carpenter syndrome Pfeiffer syndrome Saethre–Chotzen syndrome Sakati–Nyhan–Tisdale syndrome

other:	Baller–Gerold syndrome Cyclopia Goldenhar syndrome Möbius syndrome

Short stature

Limbs

Adducted thumb syndrome Holt–Oram syndrome Klippel–Trénaunay–Weber syndrome Nail–patella syndrome Rubinstein–Taybi syndrome

Gastrulation/mesoderm:	Caudal regression syndrome Ectromelia Sirenomelia VACTERL association

Overgrowth

Laurence–Moon–Bardet–Biedl

Combined/other,
known locus

2 (Feingold syndrome)
3 (Zimmermann–Laband syndrome)
4/13 (Fraser syndrome)
8 (Branchio-oto-renal syndrome, CHARGE syndrome)
12 (Keutel syndrome, Timothy syndrome)
15 (Marfan syndrome)
19 (Donohue syndrome)
Multiple
- Fryns syndrome

This article is issued from Wikipedia - version of the 7/20/2016. The text is available under the Creative Commons Attribution/Share Alike but additional terms may apply for the media files.

List of ICD-9 codes 740–759: congenital anomalies

14. Congenital anomalies (740–759)

Nervous system (740-742)

Eye, ear, face and neck (743-744)

Circulatory system (745-747)

Respiratory system (748-748)

Digestive system (749-751)

Genital organs (752-752)

Urinary system (753-753)

Musculoskeletal system (754-756)

Integument (757-757)

Chromosomal anomalies (758-758)

Other (759-759)

See also