International Classification of Diseases

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Introduction

ICD-11 planning overview

The International Classification of Diseases (ICD) World Health Organization's classification used worldwide as the standard diagnostic tool for epidemiology, health management and clinical purposes. This includes the analysis of the general health situation of population groups. It is used to monitor the incidence and prevalence of diseases and other health problems. The release date for next version update (ICD-11) is 2018. Abnormality pages begin with the ICD coding, and Embryology site content is currently being updated from ICD-10 to ICD-11 coding. The 10th revision, Australian Modification (ICD-10-AM) is currently in use in Australian hospitals for admitted patients.


The ICD is the international standard diagnostic classification for all general epidemiological, many health management purposes and clinical use.


Note - this is not a full listing of the classifications, only the major sub-headings that relate to development (use the "Detailed" links to see additional sub-sub-headings).

ICD-11 Links: ICD-11 | Anomalies one body system | Multiple anomalies or syndromes | Chromosomal anomalies | Intellectual development | Abnormal Development | Prenatal Diagnosis | Birth | Neonatal Diagnosis | ICD-10 | Reports

ICD-11 Chapter 20 Developmental anomalies

20 Developmental anomalies This chapter includes conditions caused by failure of a particular body site or body system to develop correctly during the antenatal period. Exclusions Inborn errors of metabolism (5C50-5C5Z)

International Classification of Diseases ICD-11 20 Developmental anomalies (beta draft)  
ICD-11 Beta Draft - NOT FINAL, updated on a daily basis, It is not approved by WHO, NOT TO BE USED for CODING except for agreed FIELD TRIALS.

Chapter 20 Developmental anomalies, only a few examples of the draft ICD-11 Beta coding and tree structure for "structural developmental anomalies" within this section are shown in the table below.

Mortality and Morbidity Statistics - 20 Developmental Anomalies  
Structural Developmental Anomalies  
  • Structural developmental anomalies of the nervous system  
    • LA00 Anencephaly or similar anomalies
    • LA01 Cephalocele
    • LA02 Spina bifida
    • LA03 Arnold-Chiari malformation type II
    • LA04 Congenital hydrocephalus
    • LA05 Cerebral structural developmental anomalies
    • LA06 Cerebellar structural developmental anomalies
    • LA07 Structural developmental anomalies of the neurenteric canal, spinal cord or vertebral column
    • LA0Y Other specified structural developmental anomalies of the nervous system
    • LA0Z Structural developmental anomalies of the nervous system, unspecified
  • Structural developmental anomalies of the eye, eyelid or lacrimal apparatus
    • LA10 Structural developmental anomalies of ocular globes
    • LA11 Structural developmental anomalies of the anterior segment of eye
    • LA30 Structural developmental anomalies of lens or zonula
    • LA31 Structural developmental anomalies of the posterior segment of eye
    • LA32 Structural developmental anomalies of eyelid, lacrimal apparatus or orbit
    • LA3Y Other specified structural developmental anomalies of the eye, eyelid or lacrimal apparatus
    • LA3Z Structural developmental anomalies of the eye, eyelid or lacrimal apparatus, unspecified
  • Structural developmental anomalies of the ear  
    • LA40 Structural anomaly of eustachian apparatus
    • LA41 Minor anomalies of pinnae
    • LA42 Structural developmental anomalies of ear causing hearing impairment
    • LA43 Otocephaly
    • LA44 Accessory auricle
    • LA4Y Other specified structural developmental anomalies of the ear
    • LA4Z Structural developmental anomalies of the ear, unspecified
  • Structural developmental anomalies of the face, mouth or teeth
    • LA50 Structural developmental anomalies of teeth and periodontal tissues
    • LA51 Structural developmental anomalies of mouth or tongue
    • Clefts of lip, alveolus or palate
    • LA70 Congenital velopharyngeal incompetence
    • LA71 Facial clefts
    • LA72 Facial asymmetry
    • LA73 Macrocheilia
    • LA74 Microcheilia
    • LA75 Compression facies
    • LA76 Pierre Robin syndrome
    • LC20 Dermoid cyst
    • LA7Y Other specified structural developmental anomalies of the face, mouth or teeth
    • LA7Z Structural developmental anomalies of the face, mouth or teeth, unspecified
  • Structural developmental anomalies of the neck  
  • Structural developmental anomalies of the respiratory system  
  • Structural developmental anomalies of the circulatory system  
    • Structural developmental anomaly of heart and great vessels
      • LB00 Congenital heart or great vessel related acquired abnormality
      • LB01 Congenital anomaly of atrioventricular or ventriculo-arterial connections
      • LB02 Congenital anomaly of the mediastinal veins Congenital anomaly of atria or atrial septum
      • LB20 Congenital anomaly of atrioventricular valves or septum
      • LB21 Congenital anomaly of ventricles and ventricular septum
      • LB22 Functionally univentricular heart
      • LB23 Congenital anomaly of ventriculo-arterial valves and adjacent regions
      • LB24 Congenital anomaly of great arteries including arterial duct
      • LB25 Anomalous position-orientation of heart
      • LB26 Total mirror imagery
      • LB27 Left isomerism
      • LB28 Congenital anomaly of coronary arteries
      • LB29 Structural developmental anomalies of the pericardium
      • LB2Y Other specified structural developmental anomaly of heart and great vessels
      • LB2Z Structural developmental anomaly of heart and great vessels, unspecified
    • LB30 Structural developmental anomalies of the peripheral vascular system
      • LB30.1 Capillary malformations
      • LB30.2 Lymphatic malformations
        • LB30.21 Macrocystic lymphatic malformation
        • LB30.22 Microcystic lymphatic malformation
        • LB30.23 Cystic hygroma in fetus
        • BD23.1 Primary lymphoedema
            • EK91 Yellow nail syndrome
            • LC5F.26 Noonan syndrome
        • LB30.2Y Other specified lymphatic malformations
        • LB30.2Z Lymphatic malformations, unspecified
      • LB30.3 Peripheral venous malformations
      • LB30.4 Peripheral arteriovenous malformations
      • LB30.5 Peripheral arterial malformations
      • LB30.6 Pulmonary arteriovenous fistula
      • LB30.Y Other specified structural developmental anomalies of the peripheral vascular system
      • LB30.Z Structural developmental anomalies of the peripheral vascular system, unspecified
    • LB3Y Other specified structural developmental anomalies of the circulatory system
    • LB3Z Structural developmental anomalies of the circulatory system, unspecified
  • Structural developmental anomalies of the diaphragm, abdominal wall or umbilical cord  
  • Structural developmental anomalies of the digestive tract  
  • Structural developmental anomalies of the liver, biliary tract, pancreas or spleen  
  • Structural developmental anomalies of the urinary system  
  • Structural developmental anomalies of the female genital system  
  • Structural developmental anomalies of the male genital system  
  • Structural developmental anomalies of the breast  
  • Structural developmental anomalies of the skeleton  
  • Structural developmental anomalies of the skin  
  • Structural developmental anomalies of the adrenal glands  
Multiple developmental anomalies or syndromes
Chromosomal anomalies, excluding gene mutations
Conditions with disorders of intellectual development as a relevant clinical feature
LD6Y Other specified developmental anomalies

LD6Z Developmental anomalies, unspecified

CD-11 Beta Draft - NOT FINAL, updated on a daily basis, It is not approved by WHO, NOT TO BE USED for CODING except for agreed FIELD TRIALS.


See also International Classification of Diseases
ICD-10

ICD-11 Structural developmental anomalies primarily affecting one body system  
  • Structural developmental anomalies of the nervous system
  • Structural developmental anomalies of the eye, eyelid or lacrimal apparatus
  • Structural developmental anomalies of the ear
  • Structural developmental anomalies of the face, mouth or teeth
  • Structural developmental anomalies of the neck
  • Structural developmental anomalies of the respiratory system
  • Structural developmental anomalies of the circulatory system
  • Structural developmental anomalies of the diaphragm, abdominal wall or umbilical cord
  • Structural developmental anomalies of the digestive tract
  • Structural developmental anomalies of the liver, biliary tract, pancreas or spleen
  • Structural developmental anomalies of the urinary system
  • Structural developmental anomalies of the female genital system
  • Structural developmental anomalies of the male genital system
  • Structural developmental anomalies of the breast
  • Structural developmental anomalies of the skeleton
  • Structural developmental anomalies of the skin
  • Structural developmental anomalies of the adrenal glands
LD0Y Other specified Structural developmental anomalies primarily affecting one body system

LD0Z Structural developmental anomalies primarily affecting one body system, unspecified

Abnormality Links: abnormal development | abnormal genetic | abnormal environmental | Unknown | teratogens | ectopic pregnancy | cardiovascular abnormalities | Coelomic Cavity | endocrine abnormalities | gastrointestinal abnormalities | genital abnormalities | head abnormalities | integumentary abnormalities | musculoskeletal abnormalities | limb abnormalities | neural abnormalities | neural crest abnormalities | renal abnormalities] | respiratory abnormalities | placenta abnormalities | hearing abnormalities | vision abnormalities | twinning | Developmental Origins of Health and Disease | ICD-11
Historic Embryology  
1915 Congenital Cardiac Disease | 1917 Frequency of Anomalies in Human Embryos | 1920 Hydatiform Degeneration Tubal Pregnancy | 1921 Anencephalic Embryo | 1921 Rat and Man | 1966 Congenital Malformations
ICD-11 Links: ICD-11 | Anomalies one body system | Multiple anomalies or syndromes | Chromosomal anomalies | Intellectual development | Abnormal Development | Prenatal Diagnosis | Birth | Neonatal Diagnosis | ICD-10 | Reports
ICD-11 Multiple developmental anomalies or syndromes  
  • LD20 Syndromes with central nervous system anomalies as a major feature
  • LD21 Syndromes with eye anomalies as a major feature
  • LD22 Syndromes with dental anomalies as a major feature
  • LD23 Syndromes with vascular anomalies as a major feature
  • LD24 Syndromes with skeletal anomalies as a major feature
  • LD25 Syndromes with face or limb anomalies as a major feature
  • LD26 Syndromes with limb anomalies as a major feature
  • LD27 Syndromes with skin or mucosal anomalies as a major feature
  • LD28 Syndromes with connective tissue involvement as a major feature
  • LD29 Syndromes with obesity as a major feature
  • LD2A Malformative disorders of sex development
  • LD2B Syndromes with premature ageing appearance as a major feature
  • LD2C Overgrowth syndromes
  • LD2D Phakomatoses or hamartoneoplastic syndromes
  • LD2E Syndromes with structural anomalies due to inborn errors of metabolism
  • LD2F Syndromes with multiple structural anomalies, without predominant body system involvement
  • LD2G Conjoined twins
  • LD2H Syndromic genetic deafness
LD2Y Other specified multiple developmental anomalies or syndromes

LD2Z Multiple developmental anomalies or syndromes, unspecified

ICD-11 Links: ICD-11 | Anomalies one body system | Multiple anomalies or syndromes | Chromosomal anomalies | Intellectual development | Abnormal Development | Prenatal Diagnosis | Birth | Neonatal Diagnosis | ICD-10 | Reports
ICD-11 Chromosomal anomalies excluding gene mutations  
  • LD40 Complete trisomies of the autosomes
  • LD41 Duplications of the autosomes
  • LD42 Polyploidies
  • LD43 Complete monosomies of the autosomes
  • LD44 Deletions of the autosomes
  • LD45 Uniparental disomies
  • LD46 Imprinting errors
  • LD47 Balanced rearrangements or structural markers
Sex chromosome anomalies
  • LD50 Number anomalies of chromosome X  
  • LD51 Structural anomalies of chromosome X, excluding Turner syndrome
  • LD52 Number anomalies of chromosome Y
  • LD53 Structural anomalies of chromosome Y
  • LD54 Male with sex chromosome mosaicism
  • LD55 Fragile X chromosome
  • LD56 Chimaera 46, XX, 46, XY  
LD5Y Other specified sex chromosome anomalies  
LD5Z Sex chromosome anomalies, unspecified  
LD7Y Other specified chromosomal anomalies, excluding gene mutations

LD7Z Chromosomal anomalies, excluding gene mutations, unspecified

genetic abnormalities
ICD-11 Links: ICD-11 | Anomalies one body system | Multiple anomalies or syndromes | Chromosomal anomalies | Intellectual development | Abnormal Development | Prenatal Diagnosis | Birth | Neonatal Diagnosis | ICD-10 | Reports
ICD-11 Conditions with disorders of intellectual development as a relevant clinical feature  
LD90 Conditions with disorders of intellectual development as a relevant clinical feature  
  • LD90.0 Angelman syndrome  
  • LD90.1 Early-onset parkinsonism - intellectual deficit  
  • LD90.2 Pelizaeus-Merzbacher-like disease  
  • LD90.3 Prader-Willi syndrome  
  • LD90.4 Rett syndrome  
  • 5C55.01 Lesch-Nyhan syndrome  
  • LA04.0 Hydrocephalus with stenosis of the aqueduct of Sylvius  
  • 8A44.0 Pelizaeus-Merzbacher disease  
  • 8C21.2 Hereditary sensory or autonomic neuropathy type IV  
  • LD20.00 Joubert syndrome  
  • 5C50.0 Phenylketonuria  
  • 5C50.12 Tyrosinaemia type 2  
  • 5C50.A1 Carbamoylphosphate synthetase deficiency  
  • 5C50.F1 Carnosinaemia  
  • 5C50.F2 Homocarnosinosis  
  • LD20.1 Syndromes with lissencephaly as a major feature  
  • 5C52.03 Sjögren-Larsson syndrome  
  • LA05.50 Polymicrogyria  
  • LA05.60 Porencephaly  
  • 5C53.02 Pyruvate dehydrogenase complex deficiency  
  • CB04.5 Brain-lung-thyroid syndrome  
  • 5C56.02 Metachromatic leukodystrophy  
  • 5C56.1 Neuronal ceroid lipofuscinosis  
  • 5C56.31 Mucopolysaccharidosis type 2  
  • 5C56.33 Mucopolysaccharidosis type 6  
  • 5C60.0 Oculocerebrorenal syndrome  
  • LD44.N0 CATCH 22 phenotype  
  • LD24.80 Langer-Giedion syndrome  
  • 5C58.00 Crigler-Najjar syndrome  
  • LD55 Fragile X chromosome  
  • LD27.00 Incontinentia pigmenti  
  • LD2D.2 Tuberous sclerosis  
  • LD2F.15 Noonan syndrome  
  • KA62.8 Congenital rubella syndrome  
  • KA62.3 Congenital cytomegalovirus infection  
  • LD40.0 Complete trisomy 21  
  • LD50.31 Klinefelter syndrome, male with more than two X chromosomes  
LD90.Y Other specified conditions with disorders of intellectual development as a relevant clinical feature  
LD90.Z Conditions with disorders of intellectual development as a relevant clinical feature, unspecified  
LD9Y Other specified developmental anomalies

LD9Z Developmental anomalies, unspecified

neural abnormalities
ICD-11 Links: ICD-11 | Anomalies one body system | Multiple anomalies or syndromes | Chromosomal anomalies | Intellectual development | Abnormal Development | Prenatal Diagnosis | Birth | Neonatal Diagnosis | ICD-10 | Reports
  • LD9Y Other specified developmental anomalies
  • LD9Z Developmental anomalies, unspecified

Circulatory system structural anomalies

ICD-11 Structural developmental anomalies of the circulatory system (draft) 
ICD-11 Beta Draft - NOT FINAL, updated on a daily basis, It is not approved by WHO, NOT TO BE USED for CODING except for agreed FIELD TRIALS.

20 Developmental Anomalies - Structural Developmental Anomalies

Beta coding and tree structure for "structural developmental anomalies" within this section are shown in the table below.

Structural developmental anomalies of the circulatory system  
  • Structural developmental anomaly of heart and great vessels
    • LB00 Congenital heart or great vessel related acquired abnormality
    • LB01 Congenital anomaly of atrioventricular or ventriculo-arterial connections
      • LB01.1 Transposition of the great arteries
      • LB01.2 Double outlet right ventricle
      • LB01.3 Double outlet left ventricle
      • LB01.4 Common arterial trunk
      • LB01.Y Other specified congenital anomaly of atrioventricular or ventriculo-arterial connections
      • LB01.Z Congenital anomaly of atrioventricular or ventriculo-arterial connections, unspecified
    • LB02 Congenital anomaly of the mediastinal veins Congenital anomaly of atria or atrial septum
    • LB20 Congenital anomaly of atrioventricular valves or septum
    • LB21 Congenital anomaly of ventricles and ventricular septum
      • LB21.1 Congenital right ventricular outflow tract obstruction  
      • LB21.2 Double-chambered right ventricle  
      • LB21.3 Tetralogy of Fallot
      • LB21.4 Congenital left ventricular outflow tract obstruction  
      • LB21.5 Congenital ventricular septal defects 
      • LB21.Y Other specified congenital anomaly of ventricles and ventricular septum
      • LB21.Z Congenital anomaly of ventricles and ventricular septum, unspecified  
    • LB22 Functionally univentricular heart
    • LB23 Congenital anomaly of ventriculo-arterial valves and adjacent regions
    • LB24 Congenital anomaly of great arteries including arterial duct
      • LB.1 Congenital aorto-pulmonary window
      • LB.2 Congenital anomaly of pulmonary arterial tree
      • LB.3 Congenital anomaly of aorta and its branches
      • LB.4 Tracheo-oesophageal compressive syndrome
      • LB.5 Patent arterial duct
      • LB.Y Other specified congenital anomaly of great arteries including arterial duct
      • LB.Z Congenital anomaly of great arteries including arterial duct, unspecified
    • LB25 Anomalous position-orientation of heart
    • LB26 Total mirror imagery
    • LB27 Left isomerism
    • LB28 Congenital anomaly of coronary arteries
    • LB29 Structural developmental anomalies of the pericardium
    • LB2Y Other specified structural developmental anomaly of heart and great vessels
    • LB2Z Structural developmental anomaly of heart and great vessels, unspecified
  • LB30 Structural developmental anomalies of the peripheral vascular system
    • LB30.1 Capillary malformations
    • LB30.2 Lymphatic malformations
      • LB30.21 Macrocystic lymphatic malformation
      • LB30.22 Microcystic lymphatic malformation
      • LB30.23 Cystic hygroma in fetus
      • BD23.1 Primary lymphoedema
          • EK91 Yellow nail syndrome
          • LC5F.26 Noonan syndrome
      • LB30.2Y Other specified lymphatic malformations
      • LB30.2Z Lymphatic malformations, unspecified
    • LB30.3 Peripheral venous malformations
    • LB30.4 Peripheral arteriovenous malformations
    • LB30.5 Peripheral arterial malformations
    • LB30.6 Pulmonary arteriovenous fistula
    • LB30.Y Other specified structural developmental anomalies of the peripheral vascular system
    • LB30.Z Structural developmental anomalies of the peripheral vascular system, unspecified
  • LB3Y Other specified structural developmental anomalies of the circulatory system
  • LB3Z Structural developmental anomalies of the circulatory system, unspecified
[CD-11 Beta Draft - NOT FINAL, updated on a daily basis, It is not approved by WHO, NOT TO BE USED for CODING except for agreed FIELD TRIALS.


See also International Classification of Diseases | Abnormalities


KA62 Viral infection in the foetus or newborn

Infections of the foetus or newborn - KA62 Viral infection in the foetus or newborn

  • KA62.0 Congenital Zika virus infection
  • KA62.1 Congenital Epstein-Barr virus infection
  • KA62.2 Congenital Varicella Zoster virus infection
  • KA62.3 Congenital cytomegalovirus infection
  • KA62.4 Congenital echovirus infection
  • KA62.5 Congenital enterovirus infection
  • KA62.6 Congenital human immunodeficiency virus infection
  • KA62.7 Congenital parvovirus syndrome
  • KA62.8 Congenital rubella syndrome
  • KA62.9 Congenital viral hepatitis
  • KA62.A Perinatal Herpes simplex infection
  • KA62.Y Other specified viral infection in the foetus or newborn
  • KA62.Z Viral infection in the foetus or newborn, unspecified


ICD-11 Chapter 19 Certain conditions originating in the perinatal period

  • Foetus or newborn affected by maternal factors or by complications of pregnancy, labour or delivery
  • Disorders of newborn related to length of gestation or foetal growth
  • Birth injury
  • Infections of the foetus or newborn
  • Haemorrhagic or haematological disorders of foetus or newborn
  • Neurological disorders specific to the perinatal or neonatal period
  • Respiratory disorders specific to the perinatal or neonatal period
  • Cardiovascular disorders present in the perinatal or neonatal period
  • Transitory endocrine or metabolic disorders specific to foetus or newborn
  • Digestive system disorders of foetus or newborn
  • Genitourinary system disorders specific to the perinatal or neonatal period
  • Disorders involving the integument of foetus or newborn
  • Disturbances of temperature regulation of newborn
  • Certain disorders originating in the perinatal period
  • KD5Z Conditions originating in the perinatal or neonatal period, unspecified

Changes ICD-10 to ICD-11

Below is a summary of changes from ICD 10 to ICD-11 as listed in the Nov 2015 newsletter.[1]

New Chapters

  • Chapter 3 Diseases of the Blood and Blood forming Organs
  • Chapter 4 Disorders of the Immune System.
  • Chapter 5 Conditions related to Sexual Health.
  • Chapter 8 Sleep-Wake Disorders
  • Chapter 26 Extension codes
  • Chapter 27 Traditional Medicine

New Concepts

  • Foundation: Everything in ICD
  • Entity: Each element in the foundation
  • Linearization: also known as a Classification
  • Stem code: Category (includes former ‘dagger’ codes)
  • Extension code: Additional information
  • Linearization parents: Classification hierarchy, Chapter, Block, Category Content Model
  • ICD-11 categories have a short and a long definition.
  • All ICD-11 categories include separate information on anatomy, aetiology, and other aspects. These can be accessed through searches, or when browsing in the tabular list.

New Coding Scheme

  • The chapter numbering: now arabic numbers, not roman numerals
  • The coding scheme for categories: now minimum 4 characters, 2 levels of subcategories
  • Asterisk codes become Clinical forms or Extension codes. Additional sub-classifications become Extension codes

Terminology

  • ICD-10 had a range of expressions to describe a causal relationship between conditions in a code title. In ICD 11, the preferred term is “due to”.
  • ICD-10 had a range of expressions indicating the coincidence of two conditions in a code title (e.g. “in” or “with”). In ICD-11, the preferred term is “associated with”.


ICD-10 Chapter XVII Congenital malformations, deformations and chromosomal abnormalities

Within ICD-10 classification "congenital malformations, deformations and chromosomal abnormalities" are (Q00-Q99) but excludes "inborn errors of metabolism" (E70-E90).

(ICD) ICD-10 was endorsed by the Forty-third World Health Assembly in May 1990 and came into use in WHO Member States as from 1994. The classification is the latest in a series which has its origins in the 1850s. The first edition, known as the International List of Causes of Death, was adopted by the International Statistical Institute in 1893. WHO took over the responsibility for the ICD at its creation in 1948 when the Sixth Revision, which included causes of morbidity for the first time, was published. The World Health Assembly adopted in 1967 the WHO Nomenclature Regulations that stipulate use of ICD in its most current revision for mortality and morbidity statistics by all Member States.

ICD-10 Links: XVII Congenital Malformations | System Tables | XVI Perinatal Period | XV Pregnancy Childbirth | Abnormal Development | Prenatal Diagnosis | Birth | Neonatal Diagnosis | ICD-11 | Reports

Congenital malformations of the nervous system (Q00-Q07)

Links: Q00-Q07 Detailed | Neural System - Abnormalities
  • Q00 Anencephaly and similar malformations
  • Q01 Encephalocele Incl.: encephalomyelocele, hydroencephalocele, hydromeningocele, cranial meningocele, cerebral meningoencephalocele Excl.: Meckel-Gruber syndrome (Q61.9)
  • Q02 Microcephaly Incl.: Hydromicrocephaly Micrencephalon Excl.: Meckel-Gruber syndrome (Q61.9)
  • Q03 Congenital hydrocephalus Incl.: hydrocephalus in newborn Excl.: Arnold-Chiari syndrome (Q07.0) hydrocephalus: acquired (G91.-) due to congenital toxoplasmosis (P37.1) with spina bifida (Q05.0-Q05.4)
  • Q04 Other congenital malformations of brain Excl.: cyclopia (Q87.0) macrocephaly (Q75.3)
  • Q05 Spina bifida Incl.: hydromeningocele (spinal), meningocele (spinal), meningomyelocele, myelocele, myelomeningocele, rachischisis, spina bifida (aperta)(cystica), syringomyelocele Excl.: Arnold-Chiari syndrome (Q07.0), spina bifida occulta (Q76.0)
  • Q06 Other congenital malformations of spinal cord
  • Q07 Other congenital malformations of nervous system Excl.: familial dysautonomia [Riley-Day] (G90.1), neurofibromatosis (nonmalignant) (Q85.0)


Links: Q00-Q07 Detailed | Neural System - Abnormalities

Congenital malformations of eye, ear, face and neck (Q10-Q18)

Links: Q10-Q18 Detailed | Head Abnormalities | Hearing Abnormalities | Vision Abnormalities | Smell Abnormalities

Excl.: cleft lip and cleft palate (Q35-Q37) congenital malformation of: cervical spine (Q05.0, Q05.5, Q67.5, Q76.0-Q76.4) larynx (Q31.-) lip NEC (Q38.0) nose (Q30.-) parathyroid gland (Q89.2) thyroid gland (Q89.2)

  • Q10 Congenital malformations of eyelid, lacrimal apparatus and orbit Excl.: cryptophthalmos: NOS (Q11.2) syndrome (Q87.0)
  • Q11 Anophthalmos, microphthalmos and macrophthalmos
  • Q12 Congenital lens malformations
  • Q13 Congenital malformations of anterior segment of eye
  • Q14 Congenital malformations of posterior segment of eye
  • Q15 Other congenital malformations of eye Excl.: congenital nystagmus (H55) ocular albinism (E70.3) retinitis pigmentosa (H35.5)
  • Q16 Congenital malformations of ear causing impairment of hearing Excl.: congenital deafness (H90.-)
  • Q17 Other congenital malformations of ear Excl.: preauricular sinus (Q18.1)
  • Q18 Other congenital malformations of face and neck Excl.: cleft lip and cleft palate (Q35-Q37) conditions classified to


Links: Q10-Q18 Detailed | Head Abnormalities | Hearing Abnormalities | Vision Abnormalities | Smell Abnormalities

Congenital malformations of the circulatory system (Q20-Q28)

Links: Q20-Q28 Detailed | Cardiovascular System - Abnormalities
  • Q20 Congenital malformations of cardiac chambers and connections Excl.: dextrocardia with situs inversus (Q89.3) mirror-image atrial arrangement with situs inversus (Q89.3)
  • Q21 Congenital malformations of cardiac septa Excl.: acquired cardiac septal defect (I51.0)
  • Q22 Congenital malformations of pulmonary and tricuspid valves
  • Q23 Congenital malformations of aortic and mitral valves
  • Q24 Other congenital malformations of heart Excl.: endocardial fibroelastosis (I42.4)
  • Q25 Congenital malformations of great arteries
  • Q26 Congenital malformations of great veins
  • Q27 Other congenital malformations of peripheral vascular system Excl.: anomalies of: cerebral and precerebral vessels (Q28.0-Q28.3) coronary vessels (Q24.5) pulmonary artery (Q25.5-Q25.7) congenital retinal aneurysm (Q14.1) haemangioma and lymphangioma (D18.-)
  • Q28 Other congenital malformations of circulatory system Excl.: congenital aneurysm: NOS (Q27.8) coronary (Q24.5) peripheral (Q27.8) pulmonary (Q25.7) retinal (Q14.1) ruptured: cerebral arteriovenous malformation (I60.8) malformation of precerebral vessels (I72.-)


ICD-11 Structural developmental anomalies of the circulatory system (draft) 
ICD-11 Beta Draft - NOT FINAL, updated on a daily basis, It is not approved by WHO, NOT TO BE USED for CODING except for agreed FIELD TRIALS.

20 Developmental Anomalies - Structural Developmental Anomalies

Beta coding and tree structure for "structural developmental anomalies" within this section are shown in the table below.

Structural developmental anomalies of the circulatory system  
  • Structural developmental anomaly of heart and great vessels
    • LB00 Congenital heart or great vessel related acquired abnormality
    • LB01 Congenital anomaly of atrioventricular or ventriculo-arterial connections
      • LB01.1 Transposition of the great arteries
      • LB01.2 Double outlet right ventricle
      • LB01.3 Double outlet left ventricle
      • LB01.4 Common arterial trunk
      • LB01.Y Other specified congenital anomaly of atrioventricular or ventriculo-arterial connections
      • LB01.Z Congenital anomaly of atrioventricular or ventriculo-arterial connections, unspecified
    • LB02 Congenital anomaly of the mediastinal veins Congenital anomaly of atria or atrial septum
    • LB20 Congenital anomaly of atrioventricular valves or septum
    • LB21 Congenital anomaly of ventricles and ventricular septum
      • LB21.1 Congenital right ventricular outflow tract obstruction  
      • LB21.2 Double-chambered right ventricle  
      • LB21.3 Tetralogy of Fallot
      • LB21.4 Congenital left ventricular outflow tract obstruction  
      • LB21.5 Congenital ventricular septal defects 
      • LB21.Y Other specified congenital anomaly of ventricles and ventricular septum
      • LB21.Z Congenital anomaly of ventricles and ventricular septum, unspecified  
    • LB22 Functionally univentricular heart
    • LB23 Congenital anomaly of ventriculo-arterial valves and adjacent regions
    • LB24 Congenital anomaly of great arteries including arterial duct
      • LB.1 Congenital aorto-pulmonary window
      • LB.2 Congenital anomaly of pulmonary arterial tree
      • LB.3 Congenital anomaly of aorta and its branches
      • LB.4 Tracheo-oesophageal compressive syndrome
      • LB.5 Patent arterial duct
      • LB.Y Other specified congenital anomaly of great arteries including arterial duct
      • LB.Z Congenital anomaly of great arteries including arterial duct, unspecified
    • LB25 Anomalous position-orientation of heart
    • LB26 Total mirror imagery
    • LB27 Left isomerism
    • LB28 Congenital anomaly of coronary arteries
    • LB29 Structural developmental anomalies of the pericardium
    • LB2Y Other specified structural developmental anomaly of heart and great vessels
    • LB2Z Structural developmental anomaly of heart and great vessels, unspecified
  • LB30 Structural developmental anomalies of the peripheral vascular system
    • LB30.1 Capillary malformations
    • LB30.2 Lymphatic malformations
      • LB30.21 Macrocystic lymphatic malformation
      • LB30.22 Microcystic lymphatic malformation
      • LB30.23 Cystic hygroma in fetus
      • BD23.1 Primary lymphoedema
          • EK91 Yellow nail syndrome
          • LC5F.26 Noonan syndrome
      • LB30.2Y Other specified lymphatic malformations
      • LB30.2Z Lymphatic malformations, unspecified
    • LB30.3 Peripheral venous malformations
    • LB30.4 Peripheral arteriovenous malformations
    • LB30.5 Peripheral arterial malformations
    • LB30.6 Pulmonary arteriovenous fistula
    • LB30.Y Other specified structural developmental anomalies of the peripheral vascular system
    • LB30.Z Structural developmental anomalies of the peripheral vascular system, unspecified
  • LB3Y Other specified structural developmental anomalies of the circulatory system
  • LB3Z Structural developmental anomalies of the circulatory system, unspecified
[CD-11 Beta Draft - NOT FINAL, updated on a daily basis, It is not approved by WHO, NOT TO BE USED for CODING except for agreed FIELD TRIALS.


See also International Classification of Diseases | Abnormalities

Links: ICD10 Q20-Q28 Detailed | Cardiovascular System - Abnormalities

Congenital malformations of the respiratory system (Q30-Q34)

Links: Q30-Q34 Detailed | Respiratory Abnormalities
  • Q30 Congenital malformations of nose Excl.: congenital deviation of nasal septum (Q67.4)
  • Q31 Congenital malformations of larynx Excl.: congenital (laryngeal) stridor NOS (P28.8)
  • Q32 Congenital malformations of trachea and bronchus Excl.: congenital bronchiectasis (Q33.4)
  • Q33 Congenital malformations of lung
  • Q34 Other congenital malformations of respiratory system


Links: Q30-Q34 Detailed | Respiratory Abnormalities

Cleft lip and cleft palate (Q35-Q37)

Links: Q35-Q37 Detailed | Cleft lip and cleft palate

Use additional code (Q30.2), if desired, to identify associated malformations of the nose. Excl.: Robin's syndrome (Q87.0)

  • Q35 Cleft palate Incl.: fissure of palate palatoschisis Excl.: cleft palate with cleft lip (Q37.-)
  • Q36 Cleft lip Incl.: cheiloschisis congenital fissure of lip harelip labium leporinum Excl.: cleft lip with cleft palate (Q37.-)
  • Q37.0 Cleft hard palate with bilateral cleft lip


Links: Q35-Q37 Detailed | Cleft lip and cleft palate

Other congenital malformations of the digestive system (Q38-Q45)

  • Q38 Other congenital malformations of tongue, mouth and pharynx Excl.: macrostomia (Q18.4) microstomia (Q18.5)
  • Q39 Congenital malformations of oesophagus
  • Q40 Other congenital malformations of upper alimentary tract
  • Q41 Congenital absence, atresia and stenosis of small intestine Incl.: congenital obstruction, occlusion and stricture of small intestine or intestine NOS Excl.: meconium ileus (E84.1)
  • Q42 Congenital absence, atresia and stenosis of large intestine Incl.: congenital obstruction, occlusion and stricture of large intestine
  • Q43 Other congenital malformations of intestine
  • Q44 Congenital malformations of gallbladder, bile ducts and liver
  • Q45 Other congenital malformations of digestive system Excl.: congenital: diaphragmatic hernia (Q79.0) hiatus hernia (Q40.1)


Links: Q38-Q45 Detailed | Gastrointestinal Abnormalities

Congenital malformations of genital organs (Q50-Q56)

Links: Q50-Q56 Detailed | Genital Abnormalities

Excl.: androgen resistance syndrome (E34.5) syndromes associated with anomalies in the number and form of chromosomes (90-99) testicular feminization syndrome (E34.5)

  • Q50 Congenital malformations of ovaries, fallopian tubes and broad ligaments
  • Q51 Congenital malformations of uterus and cervix
  • Q52 Other congenital malformations of female genitalia
  • Q53 Undescended testicle
  • Q54 Hypospadias Excl.: epispadias (64.0)
  • Q55 Other congenital malformations of male genital organs Excl.: congenital hydrocele (P83.5) hypospadias (54.-)
  • Q56 Indeterminate sex and pseudohermaphroditism Excl.: pseudohermaphroditism: female, with adrenocortical disorder (E25.-) male, with androgen resistance (E34.5) with specified chromosomal anomaly (96-99)


Links: Q50-Q56 Detailed | Genital Abnormalities

Congenital malformations of the urinary system (Q60-Q64)

Links: Q60-Q64 Detailed | Renal Abnormalities | Renal Development
  • Q60 Renal agenesis and other reduction defects of kidney Incl.: atrophy of kidney: congenital infantile congenital absence of kidney
  • Q61 Cystic kidney disease Excl.: acquired cyst of kidney (N28.1) Potter's syndrome (60.6)
  • Q62 Congenital obstructive defects of renal pelvis and congenital malformations of ureter
  • Q63 Other congenital malformations of kidney Excl.: congenital nephrotic syndrome (N04.-)
  • Q64 Other congenital malformations of urinary system


Links: Q60-Q64 Detailed | Renal Abnormalities | Renal Development

Congenital malformations and deformations of the musculoskeletal system

Links: Q65-Q79 Detailed | Musculoskeletal Abnormalities | Limb Abnormalities
  • Q65 Congenital deformities of hip Excl.: clicking hip (R29.4)
  • Q66 Congenital deformities of feet Excl.: reduction defects of feet (Q72.-) valgus deformities (acquired) (M21.0) varus deformities (acquired) (M21.1)
  • Q67 Congenital musculoskeletal deformities of head, face, spine and chest Excl.: congenital malformation syndromes classified to Q87.- Potter's syndrome (Q60.6)
  • Q68 Other congenital musculoskeletal deformities Excl.: reduction defects of limb(s) (Q71-Q73)
  • Q69 Polydactyly
  • Q70 Syndactyly
  • Q71 Reduction defects of upper limb
  • Q72 Reduction defects of lower limb
  • Q73 Reduction defects of unspecified limb
  • Q74 Other congenital malformations of limb(s) Excl.: polydactyly (Q69.-) reduction defect of limb (Q71-Q73) syndactyly (Q70.-)
  • Q75 Other congenital malformations of skull and face bones Excl.: congenital malformation of face NOS (Q18.-) congenital malformation syndromes classified to Q87.- dentofacial anomalies [including malocclusion] (K07.-) musculoskeletal deformities of head and face (Q67.0-Q67.4) skull defects associated with congenital anomalies of brain such as: anencephaly (Q00.0) encephalocele (Q01.-) hydrocephalus (Q03.-) microcephaly (Q02)
  • Q76 Congenital malformations of spine and bony thorax Excl.: congenital musculoskeletal deformities of spine and chest (Q67.5-Q67.8)
  • Q77 Osteochondrodysplasia with defects of growth of tubular bones and spine Excl.: mucopolysaccharidosis (E76.0-E76.3)
  • Q78 Other osteochondrodysplasias
  • Q79 Congenital malformations of the musculoskeletal system, not elsewhere classified Excl.: congenital (sternomastoid) torticollis (Q68.0)


Links: Q65-Q79 Detailed | Musculoskeletal Abnormalities | Limb Abnormalities

Other congenital malformations (Q80-Q89)

Links: Q80-Q89 Detailed | Integumentary Abnormalities
  • Q80 Congenital ichthyosis
  • Q81 Epidermolysis bullosa
  • Q83 Congenital malformations of breast
  • Q84 Other congenital malformations of integument
  • Q85 Phakomatoses, not elsewhere classified
  • Q86 Congenital malformation syndromes due to known exogenous causes, not elsewhere classified
  • Q87 Other specified congenital malformation syndromes affecting multiple systems
  • Q89 Other congenital malformations, not elsewhere classified


Links: Q80-Q89 Detailed | Integumentary Abnormalities

Chromosomal abnormalities, not elsewhere classified (Q90-Q99)

Links: Q90-Q99 Detailed | Genetic Abnormalities | Trisomy 21 | Trisomy 18 | Trisomy 13 | Trisomy X | Philadelphia chromosome | Disorders of Sex Development
  • Q90 Down's syndrome
  • Q91 Edwards' syndrome and Patau's syndrome
  • Q92 Other trisomies and partial trisomies of the autosomes, not elsewhere classified Incl.: unbalanced translocations and insertions Excl.: trisomies of chromosomes 13, 18, 21 (Q90-Q91)
  • Q93 Monosomies and deletions from the autosomes, not elsewhere classified
  • Q95 Balanced rearrangements and structural markers, not elsewhere classified Incl.: Robertsonian and balanced reciprocal translocations and insertions
  • Q96 Turner's syndrome Excl.: Noonan's syndrome (Q87.1)
  • Q97 Other sex chromosome abnormalities, female phenotype, not elsewhere classified Excl.: Turner's syndrome (Q96.-)
  • Q98 Other sex chromosome abnormalities, male phenotype, not elsewhere classified
  • Q99 Other chromosome abnormalities, not elsewhere classified


Links: Q90-Q99 Detailed | Genetic Abnormalities | Trisomy 21 | Trisomy 18 | Trisomy 13 | Trisomy X | Philadelphia chromosome | Disorders of Sex Development

ICD-10 Chapter XVI Certain conditions originating in the perinatal period (P00-P96)

Includes conditions that have their origin in the perinatal period even though death or morbidity occurs later.

Excludes congenital malformations, deformations and chromosomal abnormalities (Q00-Q99); endocrine, nutritional and metabolic diseases (E00-E90); injury, poisoning and certain other consequences of external causes (S00-T98); neoplasms (C00-D48); tetanus neonatorum (A33)



Links: Chapter XVI Perinatal Period | Neonatal Development | Neonatal Diagnosis

ICD-10 Chapter XV Pregnancy, childbirth and the puerperium (O00-O99)

The codes included in this chapter are to be used for conditions related to or aggravated by the pregnancy, childbirth or by the puerperium (maternal causes or obstetric causes)

Excludes Certain diseases or injuries complicating pregnancy, childbirth and the puerperium classified elsewhere: external causes (for mortality) (V, W, X, Y); injury, poisoning and certain other consequences of external cause (S00-T88.1, , T88.6-T98); mental and behavioural disorders associated with the puerperium (F53.-); obstetrical tetanus (A34); postpartum necrosis of pituitary gland (E23.0); puerperal osteomalacia (M83.0); supervision of high-risk pregnancy (Z35.-); supervision of normal pregnancy (Z34.-).



Links: ICD-10 Chapter XV Pregnancy Childbirth | birth


International Classification of Diseases - 10 - Australian Modification (ICD-10-AM)

ICD-10-AM is the International Statistical Classification of Diseases and Related Health Problems, Tenth Revision, Australian Modification. It consists of a tabular list of diseases and accompanying index.

ICD-10-AM was developed by the National Centre for Classification in Health and has been in use since 1998. It was developed with assistance from clinicians and clinical coders to ensure that the classification is current and appropriate for Australian clinical practice. ICD-10-AM is a derived version of the World Health Organization (WHO) ICD-10. It uses an alphanumeric coding scheme for diseases and external causes of injury. It is structured by body system and aetiology, and comprises three, four and five character categories. ICD-10-AM is updated on a regular basis, with the regular updates of ICD-10 being included as part of the updating process.

(text from - Australian Consortium for Classification Development)


Links: ICD-10-AM/ACHI/ACS

References

World Health Organisation. International Statistical Classification of Diseases and Related Health Problems. (1992) 10th Revision (ICD-10). Geneva: WHO ICD-10 - 2016 Online (English)

  1. World Health Organization – Classifications, Terminologies, and Standards ICD 11 Update Newsletter (Nov 2015).

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Cite this page: Hill, M.A. (2018, August 21) Embryology International Classification of Diseases. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/International_Classification_of_Diseases

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