List of ICD-9 codes 740-759: congenital anomalies: Information from Answers.com

Neural tube defect	Anencephaly (Acephaly, Acrania, 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

central nervous system navs: anat/physio/dev, noncongen/congen/neoplasia, symptoms+signs/eponymous, proc

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

Eye

Entire eye	Anophthalmia · Microphthalmia

Lens	Ectopia lentis · Aphakia

Iris	Aniridia

Anterior segment	Axenfeld syndrome

Cornea	Keratoglobus · Megalocornea

Other	Buphthalmos · Coloboma · Hydrophthalmos · Norrie disease

eye navs: anat/adnexa anat/pathways/physio/dev, noncongen/congen/neoplasia, eponymous signs, proc

v • d • e Congenital malformations and deformations of ears (Q16-Q17, 744.0-744.3)

Ears	Macrotia · Microtia · Low-set ears

ear navs: anat/physio/dev, noncongen/congen, eponymous signs, proc

v • d • e Congenital malformations and deformations of face and neck (Q18, 744.4-744.9)

Face	jaw: Otocephaly mouth: Macrostomia · Microstomia lip: Macrocheilia · Microcheilia multiple: Hallermann-Streiff syndrome

Neck	Webbed neck

mouth navs: anat/dev, noncongen/congen face/congen GI/neoplasia, symptoms, proc

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

Left	aortic valves (stenosis, insufficiency, bicuspid) · mitral valves (stenosis, regurgitation) · Hypoplastic left heart syndrome

Other

Dextrocardia · Levocardia · Cor triatriatum · Crisscross heart · Brugada syndrome · Coronary artery anomaly · Ventricular inversion

heart navs: anat/physio/dev, noncongen/congen/neoplasia, symptoms+signs/eponymous, proc

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

Upper RT

Nose	Choanal atresia

Larynx	Laryngocele - Laryngomalacia

Lower RT

Trachea and bronchus	Tracheomalacia - Tracheal stenosis - Bronchomalacia - Mounier-Kuhn syndrome

Lung	Bronchiectasis - Pulmonary sequestration - Congenital cystic adenomatoid malformation

respiratory system navs: anat upper head,neck+lower/physio/dev, noncongen/congen/tumors, symptoms+signs/eponymous, proc

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/anus	Imperforate anus · Persistent cloaca

Accessory

Pancreas	Annular pancreas · Accessory pancreas · Johanson-Blizzard syndrome Pancreas divisum

Bile duct	Choledochal cysts (Caroli disease) · Biliary atresia

Liver	Alagille syndrome

mouth navs: anat/dev, noncongen/congen face/congen GI/neoplasia, symptoms, proc
digestive system navs: anat of tract,glands,perit/layers/physio/dev, noncongen/congen/congen of d+w/neoplasia, symptoms+signs/eponymous, proc

Congenital malformations and deformations of sex organs (Q50-Q56 · 752)

Female

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

Other	Vaginal septum · Clitoromegaly

Male

Testicle	Cryptorchidism · Polyorchidism · Monorchism · Anorchia · Sertoli cell-only syndrome

Penis	Hypospadias · Chordee · Micropenis · Macropenis · Penile agenesis · Diphallia

Vas deferens	Congenital absence of the vas deferens

SDS/DSD

Pseudohermaphroditism · True hermaphroditism · Mixed gonadal dysgenesis · Swyer syndrome · Persistent Mullerian duct syndrome

reproductive system navs: anat female,male/physio/dev, noncongen/congen/neoplasia, symptoms+signs/eponymous, proc

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 Dent's disease - Alport syndrome

Ureter	Ectopic ureter - Megaureter

Pelvic

Bladder	Bladder exstrophy

Urethra	Epispadias - Hypospadias

Vestigial

Urachus

Urachal cyst

urinary system navs: anat/physio/dev, noncongen/acid+base/congen/neoplasia, symptoms+signs/eponymous, proc

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

Limb/
dysmelia

Upper	clavicle/shoulder: Cleidocranial dysostosis · Sprengel's deformity · Wallis Zieff Goldblatt syndrome hand deformity: Madelung's deformity · Clinodactyly

Lower	hip: Dislocation of hip/Hip dysplasia · Upington disease · Coxa valga · Coxa vara knee: Genu valgum · Genu varum foot deformity: Club foot · Flat feet · Pes cavus · Rocker bottom foot

Either/both	dactyly/digit: Polydactyly/Syndactyly (Webbed toes) · Arachnodactyly · Cenani Lenz syndactylism · Ectrodactyly · Brachydactyly reduction deficits/limb: Acheiropodia · ectromelia (Phocomelia, Amelia, Hemimelia) multiple joints: Arthrogryposis · Larsen syndrome · Rapadilino syndrome

Craniofacial

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

Other axial

spine: spinal curvature (Scoliosis) · Klippel-Feil syndrome · Spondylolisthesis · Spina bifida occulta

ribs: Cervical rib · Bifid rib

sternum: Pectus excavatum · Pectus carinatum

joint navs: anat, non-congenital arthropathies/deformities/dorsopathies/soft tissue arthropathy/congenital, eponymous signs, proc

Osteochondrodysplasia (Q77-Q78, 756.4-756.5)

Osteodysplasia/
osteodystrophy

Diaphysis	Camurati-Engelmann disease/Engelmann syndrome

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) · Polyostotic fibrous dysplasia (McCune-Albright syndrome)

Chondrodysplasia/
chondrodystrophy
(including dwarfism)

bone and cartilage navs: anat/physio/dev, noncongen/congen/neoplasia, symptoms+signs/eponymous signs, proc

v • d • e Congenital diaphragm and abdominal wall defects (Q79.0-Q79.5, 756.6-756.7)

Thoracic diaphragm	Hernia: Congenital diaphragmatic hernia (Bochdalek hernia)

Abdominal wall	Omphalocele · Gastroschisis · Prune belly syndrome

digestive system navs: anat of tract,glands,perit/layers/physio/dev, noncongen/congen/congen of d+w/neoplasia, symptoms+signs/eponymous, proc

Congenital malformations and deformations of integument (Q80-Q82, 757.0-757.3)

Skin disease/
genodermatosis

Congenital ichthyosis

AD	Ichthyosis vulgaris

AR	Congenital ichthyosiform erythroderma: Epidermolytic hyperkeratosis · Ichthyosis lamellaris (Harlequin type ichthyosis) Netherton syndrome · Zunich-Kaye syndrome · Sjögren-Larsson syndrome

XR	X-linked ichthyosis

Epidermolysis bullosa

Epidermolysis bullosa simplex · Junctional epidermolysis bullosa · Epidermolysis bullosa dystrophica

Pigmentation disorder

Incontinentia pigmenti · Urticaria pigmentosa · Lelis syndrome

DNA repair-deficiency disorder

Bloom syndrome · Rothmund-Thomson syndrome · Xeroderma pigmentosum

Ectodermal dysplasia

Naegeli syndrome/Dermatopathia pigmentosa reticularis · Hay-Wells syndrome · Hypohidrotic ectodermal dysplasia · Focal dermal hypoplasia · Ellis-van Creveld syndrome

Hyperkeratosis/keratinopathy

Meleda disease · Keratosis pilaris · Darier's disease · Dyskeratosis congenita · Lelis syndrome

Nevus

Capillary hemangioma · Port-wine stain (Nevus flammeus nuchae)

Other

Familial cutaneous papillomatosis · Kindler syndrome · cadherin (EEM syndrome) · immune system (Hereditary lymphedema, Mastocytosis) · Hailey Hailey · Birthmark

Elastic fiber/Connective tissue

Cutis laxa (Gerodermia osteodysplastica) · Pseudoxanthoma elasticum

integument navs: anat/physio, noncongen/congen/neoplasia, symptoms+signs/eponymous, proc

v • d • e Congenital malformations and deformations of skin appendages (Q84, 757.4-757.5)

Nail disease	Anonychia · Leukonychia · Pachyonychia congenita/Onychauxis · Koilonychia

Hair disease	keratin disease (Monilethrix) · Sabinas brittle hair syndrome · Pili annulati · Uncombable hair syndrome

skin appendage navs: anat, noncongen/congen/neoplasia, symptoms+signs/eponymous, proc

v • d • e Congenital malformations and deformations of the breast (Q83, 757.6)

Breast	Amastia · Polymastia · Micromastia

Nipple	Athelia · Polythelia

breast navs: anat/physio, noncongen/congenital/neoplasia, proc

Pathology: chromosome abnormalities (Q90-Q99 · 758)

Autosomal

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

Monosomies/deletions	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) · Di George's syndrome (22) genomic imprinting (Angelman syndrome/Prader-Willi syndrome (15))

X/Y linked

Monosomy	Turner syndrome (XO)

Trisomy/tetrasomy, other karyotypes/mosaics	Klinefelter's 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 46,XX/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)

Gonadal dysgenesis

Mixed gonadal dysgenesis · XX gonadal dysgenesis

Other

Fragile X syndrome · Uniparental disomy

v • d • e Lymphatic disease: splenic disease (D73/Q89.0, 289.4-289.5/759.0)

Acquired	Acquired asplenia/hyposplenism · Wandering spleen · Splenomegaly (Banti's syndrome)

Congenital	Congenital asplenia/hyposplenism · Accessory spleen · Polysplenia

lymphatic organ navs: anat, physio, lymphatic disease/splenic disease/thymus disorders/thymus neoplasia/vascular tumors, proc

Congenital endocrine disease (Q89.1-Q89.2, 759.1-759.2)

Pancreas

see congenital digestive

Hypothalamic/
pituitary axes
+parathyroid

Pituitary	Congenital hypopituitarism

Thyroid	Persistent thyroglossal duct · Thyroglossal cyst Congenital hypothyroidism: Thyroid dysgenesis · Thyroid dyshormonogenesis (Pendred syndrome)

Parathyroid	Congenital absence of parathyroid

Adrenal	Absent adrenal gland

Gonads	see congenital reproductive

endocrine navs: anat/physio/dev/hormones, noncongen/congen/neoplasia, symptoms+signs/eponymous, proc

v • d • e Twin conditions (Q89.3-Q89.4, 759.3-759.4)

Zygosity	Monozygotic · Dizygotic · 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

v • d • e Phakomatoses (Q85, 759.5-759.6)

Neurofibromatosis	Type I · Type II

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)

Other	Abdallat Davis Farrage syndrome · Ataxia telangiectasia · Incontinentia pigmenti · Peutz-Jeghers syndrome

v • d • e Congenital abnormality · multiple abnormalities (Q87, 759.7)

Craniofacial	Acrocephalosyndactylia (Apert syndrome/Pfeiffer syndrome, Saethre-Chotzen syndrome, Carpenter syndrome, Sakati-Nyhan-Tisdale syndrome) other: Möbius syndrome · Goldenhar syndrome · Cyclopia

Short stature	Aarskog-Scott syndrome · Cockayne syndrome · Cornelia de Lange Syndrome · Dubowitz syndrome · Noonan syndrome · Robinow syndrome · Silver-Russell dwarfism · Seckel syndrome · Smith-Lemli-Opitz syndrome-Turner syndrome

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	Beckwith-Wiedemann syndrome · Sotos syndrome · Weaver syndrome

Laurence-Moon-Bardet-Biedl	Bardet-Biedl syndrome · Laurence-Moon syndrome

Combined/other, known locus	3 (Zimmerman-Laband syndrome) · 4/13 (Fraser syndrome) · 8 (Branchio-oto-renal syndrome) · 12 (Keutel syndrome, Timothy syndrome) · 15 (Marfan syndrome) · 19 (Donohue syndrome)

Combined/other, unknown locus	Ablepharon macrostomia syndrome · Fountain syndrome · Urban-Rogers-Meyer syndrome · Yunis-Varon syndrome

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

Contents

14. Congenital anomalies (740-759)

nervous system

eye, ear, face and neck

circulatory system

respiratory system

digestive system

genital organs

urinary system

musculoskeletal system

integument

chromosomal anomalies

Other

See also