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 the full listing of the classifications, only the major sub-headings that relate to development (use the "tables" links to see additional sub-sub-headings). Please refer to the original official WHO ICD-11 Database for detailed information and any personal encoding uses.

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 | | Category:ICD-11

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 | coelom abnormalities | endocrine abnormalities | gastrointestinal abnormalities | genital abnormalities | head abnormalities | integumentary abnormalities | musculoskeletal abnormalities | limb abnormalities | neural abnormalities | neural crest abnormalities | placenta abnormalities | renal abnormalities | respiratory 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 | | Category:ICD-11
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 | | Category:ICD-11
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 | | Category:ICD-11
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 | | Category:ICD-11
  • LD9Y Other specified developmental anomalies
  • LD9Z Developmental anomalies, unspecified

Neural

 ICD-11 LA00-LA0Z Structural developmental anomalies of the nervous system
  • LA00.0 Anencephaly - a neural tube defect, characterized by the total or partial absence of the cranial vault and the covering skin, the brain being missing or reduced to a small mass. Most cases are stillborn, although some infants have been reported to survive for a few hours. In most cases autopsy findings reveal absence of adrenal glands. Anencephaly is likely to be multifactorial, the result of gene-environment interactions. Familial cases with a seemingly autosomal recessive mode of inheritance have been described but most cases are sporadic. Folic acid and zinc deficiencies, as well as maternal obesity, have been shown to be risk factors.
  • LA00.1 Iniencephaly - a rare form of neural tube defect in which a malformation of the cervico-occipital junction is associated with a malformation of the central nervous system. The cardinal features are occipital bone defect, partial or total absence of cervicothoracic vertebrae, fetal retroflexion of the head and characteristic absence of the neck. It is associated with malformations of the central nervous (spina bifida and/or anencephaly), gastrointestinal (omphalocele) and cardiovascular systems.
  • LA00.2 Acephaly
  • LA00.3 Amyelencephaly - Amyelencephaly is the absence of both the brain and spinal cord.
  • LA01 Cephalocele - failure of the skull to correctly close during the antenatal period. This condition is characterized by herniation of the meninges. This condition may present with herniation of brain, or developmental delay. Confirmation is through observation of herniated meninges by imaging.
  • LA02 Spina bifida -
    • LA02.0 Spina bifida cystica - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage and the presence of meningoceles on the back. This condition may present with physical or mental impairment.
    • LA02.00 Myelomeningocele with hydrocephalus - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage and hydrocephalus. This condition may also present with syringomyelia, hip dislocation, headache, nausea, vomiting, blurry vision, balance problems, bladder control problems, meningitis, or mental impairment.
    • LA02.01 Myelomeningocele without hydrocephalus - failure of the neural tube to close completely during fetal development. This condition is characterized by nerve damage. This condition may also present with syringomyelia, hip dislocation, headache, nausea, vomiting, blurry vision, balance problems, bladder control problems, meningitis, or mental impairment.
    • LA02.02 Myelocystocele - failure of the neural tube to close completely during fetal development. The condition is characterized by skin covered lumbosacral masses, an arachnoid lined meningocele that is directly continuous with the spinal subarachnoid space, and a low lying hydromyelic spinal cord that traverses the meningocele and expands into a large terminal cyst. This condition can present with neural damage and consequent impairment of function below the site of the myelocystocele.
    • LA02.1 Spina bifida aperta - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage originating from a known location in the spine, signified by the presence of a meningocele or myelomeningocele. This condition may present with physical or mental impairment.
  • LA03 Arnold-Chiari malformation type II - failure of the brain and spinal cord to correctly develop during the antenatal period. This condition is characterized by extension of both cerebellar and brain stem tissue into the foramen magnum. This condition may present with partial or complete absence of the cerebellar vermis, myelomeningocele, neck pain, balance problems, muscle weakness, limb numbness, dizziness, vision problems, difficulty swallowing, ringing in the ears, hearing loss, vomiting, insomnia, depression, or impairment of motor skills.
  • LA04 Congenital hydrocephalus - failure of the brain to correctly develop during the antenatal period. This condition is characterized by a rapid increase in head circumference or an unusually large head size due to excessive accumulation of cerebrospinal fluid in the brain. This condition may also present with vomiting, sleepiness, irritability, downward deviation of the eyes, or seizures. Confirmation is through observation of cerebrospinal fluid within cerebral ventricles by imaging.
  • LA05 Cerebral structural developmental anomalies
    • LA05.0 Microcephaly
    • LA05.1 Megalencephaly
    • LA05.2 Holoprosencephaly
    • LA05.3 Corpus callosum agenesis
    • LA05.4 Arhinencephaly
    • LA05.5 Abnormal neuronal migration
    • LA05.6 Encephaloclastic disorders
    • LA05.7 Brain cystic malformations
  • LA06 Cerebellar structural developmental anomalies
    • LA06.0 Dandy-Walker malformation
    • LA06.1 Hypoplasia or agenesis of cerebellar hemispheres
    • LA06.2 Focal cerebellar dysplasia
  • LA07 Structural developmental anomalies of the neurenteric canal, spinal cord or vertebral column
International Classification of Diseases  ICD-11 Neural developmental anomalies 
 ICD-11 LA00-LA0Z Structural developmental anomalies of the nervous system
  • LA00.0 Anencephaly - a neural tube defect, characterized by the total or partial absence of the cranial vault and the covering skin, the brain being missing or reduced to a small mass. Most cases are stillborn, although some infants have been reported to survive for a few hours. In most cases autopsy findings reveal absence of adrenal glands. Anencephaly is likely to be multifactorial, the result of gene-environment interactions. Familial cases with a seemingly autosomal recessive mode of inheritance have been described but most cases are sporadic. Folic acid and zinc deficiencies, as well as maternal obesity, have been shown to be risk factors.
  • LA00.1 Iniencephaly - a rare form of neural tube defect in which a malformation of the cervico-occipital junction is associated with a malformation of the central nervous system. The cardinal features are occipital bone defect, partial or total absence of cervicothoracic vertebrae, fetal retroflexion of the head and characteristic absence of the neck. It is associated with malformations of the central nervous (spina bifida and/or anencephaly), gastrointestinal (omphalocele) and cardiovascular systems.
  • LA00.2 Acephaly
  • LA00.3 Amyelencephaly - Amyelencephaly is the absence of both the brain and spinal cord.
  • LA01 Cephalocele - failure of the skull to correctly close during the antenatal period. This condition is characterized by herniation of the meninges. This condition may present with herniation of brain, or developmental delay. Confirmation is through observation of herniated meninges by imaging.
  • LA02 Spina bifida -
    • LA02.0 Spina bifida cystica - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage and the presence of meningoceles on the back. This condition may present with physical or mental impairment.
    • LA02.00 Myelomeningocele with hydrocephalus - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage and hydrocephalus. This condition may also present with syringomyelia, hip dislocation, headache, nausea, vomiting, blurry vision, balance problems, bladder control problems, meningitis, or mental impairment.
    • LA02.01 Myelomeningocele without hydrocephalus - failure of the neural tube to close completely during fetal development. This condition is characterized by nerve damage. This condition may also present with syringomyelia, hip dislocation, headache, nausea, vomiting, blurry vision, balance problems, bladder control problems, meningitis, or mental impairment.
    • LA02.02 Myelocystocele - failure of the neural tube to close completely during fetal development. The condition is characterized by skin covered lumbosacral masses, an arachnoid lined meningocele that is directly continuous with the spinal subarachnoid space, and a low lying hydromyelic spinal cord that traverses the meningocele and expands into a large terminal cyst. This condition can present with neural damage and consequent impairment of function below the site of the myelocystocele.
    • LA02.1 Spina bifida aperta - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage originating from a known location in the spine, signified by the presence of a meningocele or myelomeningocele. This condition may present with physical or mental impairment.
  • LA03 Arnold-Chiari malformation type II - failure of the brain and spinal cord to correctly develop during the antenatal period. This condition is characterized by extension of both cerebellar and brain stem tissue into the foramen magnum. This condition may present with partial or complete absence of the cerebellar vermis, myelomeningocele, neck pain, balance problems, muscle weakness, limb numbness, dizziness, vision problems, difficulty swallowing, ringing in the ears, hearing loss, vomiting, insomnia, depression, or impairment of motor skills.
  • LA04 Congenital hydrocephalus - failure of the brain to correctly develop during the antenatal period. This condition is characterized by a rapid increase in head circumference or an unusually large head size due to excessive accumulation of cerebrospinal fluid in the brain. This condition may also present with vomiting, sleepiness, irritability, downward deviation of the eyes, or seizures. Confirmation is through observation of cerebrospinal fluid within cerebral ventricles by imaging.
  • LA05 Cerebral structural developmental anomalies
    • LA05.0 Microcephaly
    • LA05.1 Megalencephaly
    • LA05.2 Holoprosencephaly
    • LA05.3 Corpus callosum agenesis
    • LA05.4 Arhinencephaly
    • LA05.5 Abnormal neuronal migration
    • LA05.6 Encephaloclastic disorders
    • LA05.7 Brain cystic malformations
  • LA06 Cerebellar structural developmental anomalies
    • LA06.0 Dandy-Walker malformation
    • LA06.1 Hypoplasia or agenesis of cerebellar hemispheres
    • LA06.2 Focal cerebellar dysplasia
  • LA07 Structural developmental anomalies of the neurenteric canal, spinal cord or vertebral column
 ICD-11 LA02 Spina bifida
  • LA02.0 Spina bifida cystica - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage and the presence of meningoceles on the back. This condition may present with physical or mental impairment.
  • LA02.00 Myelomeningocele with hydrocephalus - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage and hydrocephalus. This condition may also present with syringomyelia, hip dislocation, headache, nausea, vomiting, blurry vision, balance problems, bladder control problems, meningitis, or mental impairment.
  • LA02.01 Myelomeningocele without hydrocephalus - failure of the neural tube to close completely during fetal development. This condition is characterized by nerve damage. This condition may also present with syringomyelia, hip dislocation, headache, nausea, vomiting, blurry vision, balance problems, bladder control problems, meningitis, or mental impairment.
  • LA02.02 Myelocystocele - failure of the neural tube to close completely during fetal development. The condition is characterized by skin covered lumbosacral masses, an arachnoid lined meningocele that is directly continuous with the spinal subarachnoid space, and a low lying hydromyelic spinal cord that traverses the meningocele and expands into a large terminal cyst. This condition can present with neural damage and consequent impairment of function below the site of the myelocystocele.
  • LA02.1 Spina bifida aperta - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage originating from a known location in the spine, signified by the presence of a meningocele or myelomeningocele. This condition may present with physical or mental impairment.
spina bifida | neural abnormalities |  ICD-11
International Classification of Diseases - Spina bifida 
 ICD-11 LA02 Spina bifida
  • LA02.0 Spina bifida cystica - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage and the presence of meningoceles on the back. This condition may present with physical or mental impairment.
  • LA02.00 Myelomeningocele with hydrocephalus - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage and hydrocephalus. This condition may also present with syringomyelia, hip dislocation, headache, nausea, vomiting, blurry vision, balance problems, bladder control problems, meningitis, or mental impairment.
  • LA02.01 Myelomeningocele without hydrocephalus - failure of the neural tube to close completely during fetal development. This condition is characterized by nerve damage. This condition may also present with syringomyelia, hip dislocation, headache, nausea, vomiting, blurry vision, balance problems, bladder control problems, meningitis, or mental impairment.
  • LA02.02 Myelocystocele - failure of the neural tube to close completely during fetal development. The condition is characterized by skin covered lumbosacral masses, an arachnoid lined meningocele that is directly continuous with the spinal subarachnoid space, and a low lying hydromyelic spinal cord that traverses the meningocele and expands into a large terminal cyst. This condition can present with neural damage and consequent impairment of function below the site of the myelocystocele.
  • LA02.1 Spina bifida aperta - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage originating from a known location in the spine, signified by the presence of a meningocele or myelomeningocele. This condition may present with physical or mental impairment.
spina bifida | neural abnormalities |  ICD-11
 ICD-11 LA05 Cerebral structural developmental anomalies
  • LA05.0 Microcephaly - failure of the head to correctly develop during the antenatal period. This condition is characterized by a head size that is significantly smaller than normal for their age and sex. This condition may also present with developmental delays, difficulties with balance and coordination, short stature, hyperactivity, mental retardation, seizures, or other neurological abnormalities.
  • LA05.1 Megalencephaly - failure of the brain to correctly develop during the antenatal period. This condition is characterized by increased size or weight of an otherwise correctly formed brain. This condition may also present with seizures, motor deficits, mental retardation and mild cognitive impairment.
  • LA05.2 Holoprosencephaly - brain malformation resulting from incomplete cleavage of the prosencephalon, occurring between the 18th and the 28th day of gestation and affecting both the forebrain and the face. In most of the cases, facial anomalies are observed: cyclopia, proboscis and median or bilateral cleft lip/palate in severe forms, and ocular hypotelorism or solitary median maxillary central incisor in minor forms. These latter midline defects can occur without the cerebral malformations (microforms). Children with HPE have many medical problems: developmental delay and feeding difficulties, epilepsy, and instability of temperature, heart rate and respiration. Endocrine disorders like diabetes insipidus, adrenal hypoplasia, hypogonadism, thyroid hypoplasia and growth hormone deficiency are frequent.
  • LA05.3 Corpus callosum agenesis - the most common brain malformation and is characterized by total or partial absence of the main interhemispheric commissure, the corpus callosum.
  • LA05.4 Arhinencephaly - failure of the olfactory organs to correctly develop during the antenatal period. This condition is characterized by absence of the olfactory bulbs and tracts.
  • LA05.5 Abnormal neuronal migration - any condition caused by abnormal migration of neuronal cells during the antenatal period. These conditions may present with poor muscle tone and motor function, seizures, developmental delays, mental retardation, failure to grow and thrive, difficulties with feeding, swelling in the extremities or microcephaly.
  • LA05.6 Encephaloclastic disorders
  • LA05.7 Brain cystic malformations - A disease caused by expansion of the roof plate of the brain vesicle, or by extraaxial structures such as an arachnoid membrane or migrating ependymal cells. This disease is characterized by the presence of fluid filled cysts in the brain. This disease may present with asymmetry of the skull, brain compression, raised intracranial pressure, hydrocephalus, bleeding or seizures. This disease may also be asymptomatic. Confirmation is through observation of intracerebral cysts by imaging.
cerebral anomalies | neural abnormalities |  ICD-11
International Classification of Diseases - Cerebral structural developmental anomalies 
 ICD-11 LA05 Cerebral structural developmental anomalies
  • LA05.0 Microcephaly - failure of the head to correctly develop during the antenatal period. This condition is characterized by a head size that is significantly smaller than normal for their age and sex. This condition may also present with developmental delays, difficulties with balance and coordination, short stature, hyperactivity, mental retardation, seizures, or other neurological abnormalities.
  • LA05.1 Megalencephaly - failure of the brain to correctly develop during the antenatal period. This condition is characterized by increased size or weight of an otherwise correctly formed brain. This condition may also present with seizures, motor deficits, mental retardation and mild cognitive impairment.
  • LA05.2 Holoprosencephaly - brain malformation resulting from incomplete cleavage of the prosencephalon, occurring between the 18th and the 28th day of gestation and affecting both the forebrain and the face. In most of the cases, facial anomalies are observed: cyclopia, proboscis and median or bilateral cleft lip/palate in severe forms, and ocular hypotelorism or solitary median maxillary central incisor in minor forms. These latter midline defects can occur without the cerebral malformations (microforms). Children with HPE have many medical problems: developmental delay and feeding difficulties, epilepsy, and instability of temperature, heart rate and respiration. Endocrine disorders like diabetes insipidus, adrenal hypoplasia, hypogonadism, thyroid hypoplasia and growth hormone deficiency are frequent.
  • LA05.3 Corpus callosum agenesis - the most common brain malformation and is characterized by total or partial absence of the main interhemispheric commissure, the corpus callosum.
  • LA05.4 Arhinencephaly - failure of the olfactory organs to correctly develop during the antenatal period. This condition is characterized by absence of the olfactory bulbs and tracts.
  • LA05.5 Abnormal neuronal migration - any condition caused by abnormal migration of neuronal cells during the antenatal period. These conditions may present with poor muscle tone and motor function, seizures, developmental delays, mental retardation, failure to grow and thrive, difficulties with feeding, swelling in the extremities or microcephaly.
  • LA05.6 Encephaloclastic disorders
  • LA05.7 Brain cystic malformations - A disease caused by expansion of the roof plate of the brain vesicle, or by extraaxial structures such as an arachnoid membrane or migrating ependymal cells. This disease is characterized by the presence of fluid filled cysts in the brain. This disease may present with asymmetry of the skull, brain compression, raised intracranial pressure, hydrocephalus, bleeding or seizures. This disease may also be asymptomatic. Confirmation is through observation of intracerebral cysts by imaging.
cerebral anomalies | neural abnormalities |  ICD-11
 ICD-11 LA00.0 Anencephaly 
a neural tube defect, characterized by the total or partial absence of the cranial vault and the covering skin, the brain being missing or reduced to a small mass. Most cases are stillborn, although some infants have been reported to survive for a few hours. In most cases autopsy findings reveal absence of adrenal glands. Anencephaly is likely to be multifactorial, the result of gene-environment interactions. Familial cases with a seemingly autosomal recessive mode of inheritance have been described but most cases are sporadic. Folic acid and zinc deficiencies, as well as maternal obesity, have been shown to be risk factors.
  • LA00.1 Iniencephaly - a rare form of neural tube defect in which a malformation of the cervico-occipital junction is associated with a malformation of the central nervous system. The cardinal features are occipital bone defect, partial or total absence of cervicothoracic vertebrae, fetal retroflexion of the head and characteristic absence of the neck. It is associated with malformations of the central nervous (spina bifida and/or anencephaly), gastrointestinal (omphalocele) and cardiovascular systems.
  • LA00.2 Acephaly
  • LA00.3 Amyelencephaly - Amyelencephaly is the absence of both the brain and spinal cord.

LA01 Cephalocele - failure of the skull to correctly close during the antenatal period. This condition is characterized by herniation of the meninges. This condition may present with herniation of brain, or developmental delay. Confirmation is through observation of herniated meninges by imaging.

anencephaly | neural abnormalities |  ICD-11
International Classification of Diseases - Anencephaly 
 ICD-11 LA00.0 Anencephaly 
a neural tube defect, characterized by the total or partial absence of the cranial vault and the covering skin, the brain being missing or reduced to a small mass. Most cases are stillborn, although some infants have been reported to survive for a few hours. In most cases autopsy findings reveal absence of adrenal glands. Anencephaly is likely to be multifactorial, the result of gene-environment interactions. Familial cases with a seemingly autosomal recessive mode of inheritance have been described but most cases are sporadic. Folic acid and zinc deficiencies, as well as maternal obesity, have been shown to be risk factors.
  • LA00.1 Iniencephaly - a rare form of neural tube defect in which a malformation of the cervico-occipital junction is associated with a malformation of the central nervous system. The cardinal features are occipital bone defect, partial or total absence of cervicothoracic vertebrae, fetal retroflexion of the head and characteristic absence of the neck. It is associated with malformations of the central nervous (spina bifida and/or anencephaly), gastrointestinal (omphalocele) and cardiovascular systems.
  • LA00.2 Acephaly
  • LA00.3 Amyelencephaly - Amyelencephaly is the absence of both the brain and spinal cord.

LA01 Cephalocele - failure of the skull to correctly close during the antenatal period. This condition is characterized by herniation of the meninges. This condition may present with herniation of brain, or developmental delay. Confirmation is through observation of herniated meninges by imaging.

anencephaly | neural abnormalities |  ICD-11
 ICD-11 Cerebral palsy
8D20 Spastic cerebral palsy - characterized by increased muscle tone associated with hyperactive muscle stretch reflexes (deep tendon reflexes) and an increase in resistance to rapid muscle stretch. Extensor plantar responses are commonly present.
  • 8D20.0 Spastic unilateral cerebral palsy - a form of cerebral palsy in which the spasticity is confined to one side; it is often accompanied by cortical sensory impairment and varying degrees of hemineglect, demonstrable by testing stereognosis and graphesthesia. Early hand preference is often the first sign of this disorder, and may be apparent in the first months of life.
  • 8D20.1 Spastic bilateral cerebral palsy
  • 8D21 Dyskinetic cerebral palsy - (extrapyramidal cerebral palsy) is characterized by impairment of voluntary movement because of the presence of interfering involuntary movements, and inappropriate co-contraction of agonist and antagonist muscles (dystonia). This group of disorders includes choreoathetotic cerebral palsy and dystonic cerebral palsy. The former is characterized by large amplitude, involuntary movements of mainly distal limbs(athetosis) with or without small amplitude, fleeting, asymmetric contractions of individual muscle groups (chorea). Dystonic cerebral palsy predominantly affects proximal trunk and limb muscles, which may show slow, persistent movements, leading to the adoption of unusual postures, such as torticollis.
  • 8D22 Ataxic cerebral palsy - dominated by signs of cerebellar dysfunction, including hypotonia, ataxia, dysdiadochokinesis, dysmetria, dysarthria and nystagmus. Reflexes may be pendular, although there are often also signs of spasticity.
  • 8D23 Worster-Drought syndrome - a form of cerebral palsy characterized by congenital pseudobulbar (suprabulbar) paresis manifesting as selective weakness of the lips, tongue and soft palate, dysphagia, dysphonia, drooling and jaw jerking.
International Classification of Diseases - Cerebral palsy 
 ICD-11 Cerebral palsy
8D20 Spastic cerebral palsy - characterized by increased muscle tone associated with hyperactive muscle stretch reflexes (deep tendon reflexes) and an increase in resistance to rapid muscle stretch. Extensor plantar responses are commonly present.
  • 8D20.0 Spastic unilateral cerebral palsy - a form of cerebral palsy in which the spasticity is confined to one side; it is often accompanied by cortical sensory impairment and varying degrees of hemineglect, demonstrable by testing stereognosis and graphesthesia. Early hand preference is often the first sign of this disorder, and may be apparent in the first months of life.
  • 8D20.1 Spastic bilateral cerebral palsy
  • 8D21 Dyskinetic cerebral palsy - (extrapyramidal cerebral palsy) is characterized by impairment of voluntary movement because of the presence of interfering involuntary movements, and inappropriate co-contraction of agonist and antagonist muscles (dystonia). This group of disorders includes choreoathetotic cerebral palsy and dystonic cerebral palsy. The former is characterized by large amplitude, involuntary movements of mainly distal limbs(athetosis) with or without small amplitude, fleeting, asymmetric contractions of individual muscle groups (chorea). Dystonic cerebral palsy predominantly affects proximal trunk and limb muscles, which may show slow, persistent movements, leading to the adoption of unusual postures, such as torticollis.
  • 8D22 Ataxic cerebral palsy - dominated by signs of cerebellar dysfunction, including hypotonia, ataxia, dysdiadochokinesis, dysmetria, dysarthria and nystagmus. Reflexes may be pendular, although there are often also signs of spasticity.
  • 8D23 Worster-Drought syndrome - a form of cerebral palsy characterized by congenital pseudobulbar (suprabulbar) paresis manifesting as selective weakness of the lips, tongue and soft palate, dysphagia, dysphonia, drooling and jaw jerking.

Gastrointestinal

 ICD-11 - Structural developmental anomalies of the digestive tract (LB10 - LB18)
  • LB10 Structural developmental anomalies of salivary glands or ducts
  • LB11 Congenital diverticulum of pharynx
  • LB12 Structural developmental anomalies of oesophagus  
    • LB12.1 Congenital oesophageal web or ring  
    • LB12.2 Atresia of oesophagus  
    • LB12.3 Oesophageal fistula without atresia  
    • LB12.4 Congenital stenosis or stricture of oesophagus  
    • LB12.5 Congenital diverticulum of oesophagus  
    • LB12.6 Congenital dilatation of oesophagus  
    • LB12.Y Other specified structural developmental anomalies of oesophagus  
    • LB12.Z Structural developmental anomalies of oesophagus, unspecified  
  • LB13 Structural developmental anomalies of stomach  
  • LB14 Structural developmental anomalies of duodenum  
  • LB15 Structural developmental anomalies of small intestine  
  • LB16 Structural developmental anomalies of large intestine  
  • LB17 Structural developmental anomalies of anal canal  
  • LB18 Congenital anomalies of intestinal fixation  
  • DA02.1 Genetic or developmental disorders involving lips or oral mucosa  
  • LB1Y Other specified structural developmental anomalies of the digestive tract  
  • LB1Z Structural developmental anomalies of the digestive tract, unspecified  
  • Inborn Errors of Metabolism
  • 13 Diseases of the digestive system
gastrointestinal abnormalities |  ICD-11
International Classification of Diseases - Structural developmental anomalies of the digestive tract 
 ICD-11 - Structural developmental anomalies of the digestive tract (LB10 - LB18)
  • LB10 Structural developmental anomalies of salivary glands or ducts
  • LB11 Congenital diverticulum of pharynx
  • LB12 Structural developmental anomalies of oesophagus  
    • LB12.1 Congenital oesophageal web or ring  
    • LB12.2 Atresia of oesophagus  
    • LB12.3 Oesophageal fistula without atresia  
    • LB12.4 Congenital stenosis or stricture of oesophagus  
    • LB12.5 Congenital diverticulum of oesophagus  
    • LB12.6 Congenital dilatation of oesophagus  
    • LB12.Y Other specified structural developmental anomalies of oesophagus  
    • LB12.Z Structural developmental anomalies of oesophagus, unspecified  
  • LB13 Structural developmental anomalies of stomach  
  • LB14 Structural developmental anomalies of duodenum  
  • LB15 Structural developmental anomalies of small intestine  
  • LB16 Structural developmental anomalies of large intestine  
  • LB17 Structural developmental anomalies of anal canal  
  • LB18 Congenital anomalies of intestinal fixation  
  • DA02.1 Genetic or developmental disorders involving lips or oral mucosa  
  • LB1Y Other specified structural developmental anomalies of the digestive tract  
  • LB1Z Structural developmental anomalies of the digestive tract, unspecified  
  • Inborn Errors of Metabolism
  • 13 Diseases of the digestive system
gastrointestinal abnormalities |  ICD-11


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


Viral infection in the foetus or newborn

 ICD-11 KA62 Viral infection in the foetus or newborn
Any condition affecting foetuses or newborns, caused by an infection with a virus.
  • KA62.0 Congenital Zika virus infection
  • KA62.1 Congenital Epstein-Barr virus infection - There are several forms of Epstein–Barr virus infection. Infectious mononucleosis, nasopharyngeal carcinoma, and Burkitt's lymphoma can all be caused by the Epstein–Barr virus.
  • KA62.2 Congenital Varicella Zoster virus infection - Transplacentally acquired Varicella zoster virus infection. Both the gestational age at the time of maternal infection and the time interval between maternal infection and birth have major influences on the clinical course.
  • KA62.3 Congenital cytomegalovirus infection - A condition affecting neonates, caused by an infection with cytomegalovirus in utero. This condition is characterized by jaundice, low birth weight, splenomegaly, hepatomegaly, or pneumonia if symptoms develop shortly after birth, or may be asymptomatic. This condition commonly present later in life with loss of hearing, loss of vision, or developmental disabilities. Transmission is by vertical transmission. Confirmation is by detection of cytomegalovirus in neonatal urine, saliva, blood, or other body tissues within 2-3 weeks of birth.
  • KA62.4 Congenital echovirus infection - A disease affecting neonates, caused by an infection with enteric cytopathic human orphan (ECHO) virus in utero. This disease presents with various symptoms depending on the site of the infection, or may be asymptomatic. Transmission is by vertical transmission. Confirmation is by identification of ECHO virus in the neonate.
  • KA62.5 Congenital enterovirus infection - Congenital viral infections with enteroviruses (including coxsackie viruses and ECHO viruses) is an infectious embriofetopathy that have been reported to cause fetal malformations, acute systemic illness in the newborn and long-term neurodevelopmental abnormalities.
  • KA62.6 Congenital human immunodeficiency virus infection - A disease affecting neonates, caused by an infection with human immunodeficiency virus in utero. Transmission is by vertical transmission. Confirmation is by identification of human immunodeficiency virus in the neonate.
  • KA62.7 Congenital parvovirus syndrome - Fetal parvovirus syndrome is a fetopathy likely to occur when a pregnant woman is infected by parvovirus B19. Fetal parvovirus infection results in aplastic crisis. Anaemia induces a risk of hydrops and fetal death by cardiac failure in 10 to 20% of cases.
  • KA62.8 Congenital rubella syndrome - A disease caused by an infection with the rubella virus in utero. This disease presents with symptoms depending on the timing of infection of the fetus and may present with birth defects (such as hearing loss), or intrauterine growth retardation. Transmission is by vertical transmission. Confirmation is by identification of rubella virus or detection of anti-rubella virus IgM antibodies in the neonate or infant.
  • KA62.9 Congenital viral hepatitis - A disease of the liver affecting the neonate, caused by an infection with either hepatitis A, B, C, D, or E virus in utero. This disease is characterized by lethargy, jaundice, abdominal distention, failure to thrive, or clay coloured stools. Transmission is by vertical transmission. Confirmation is by identification of the hepatitis A, B, C, D, or E virus in a blood sample from the neonate.
  • KA62.A Perinatal Herpes simplex infection - Herpes simplex infection acquired during the perinatal period, normally from active herpes infection of the mother's genital tract, but may also be transmitted in utero.
viral infection | environmental abnormalities |  ICD-11 | Infections of the foetus or newborn
International Classification of Diseases - Viral infection 
 ICD-11 KA62 Viral infection in the foetus or newborn
Any condition affecting foetuses or newborns, caused by an infection with a virus.
  • KA62.0 Congenital Zika virus infection
  • KA62.1 Congenital Epstein-Barr virus infection - There are several forms of Epstein–Barr virus infection. Infectious mononucleosis, nasopharyngeal carcinoma, and Burkitt's lymphoma can all be caused by the Epstein–Barr virus.
  • KA62.2 Congenital Varicella Zoster virus infection - Transplacentally acquired Varicella zoster virus infection. Both the gestational age at the time of maternal infection and the time interval between maternal infection and birth have major influences on the clinical course.
  • KA62.3 Congenital cytomegalovirus infection - A condition affecting neonates, caused by an infection with cytomegalovirus in utero. This condition is characterized by jaundice, low birth weight, splenomegaly, hepatomegaly, or pneumonia if symptoms develop shortly after birth, or may be asymptomatic. This condition commonly present later in life with loss of hearing, loss of vision, or developmental disabilities. Transmission is by vertical transmission. Confirmation is by detection of cytomegalovirus in neonatal urine, saliva, blood, or other body tissues within 2-3 weeks of birth.
  • KA62.4 Congenital echovirus infection - A disease affecting neonates, caused by an infection with enteric cytopathic human orphan (ECHO) virus in utero. This disease presents with various symptoms depending on the site of the infection, or may be asymptomatic. Transmission is by vertical transmission. Confirmation is by identification of ECHO virus in the neonate.
  • KA62.5 Congenital enterovirus infection - Congenital viral infections with enteroviruses (including coxsackie viruses and ECHO viruses) is an infectious embriofetopathy that have been reported to cause fetal malformations, acute systemic illness in the newborn and long-term neurodevelopmental abnormalities.
  • KA62.6 Congenital human immunodeficiency virus infection - A disease affecting neonates, caused by an infection with human immunodeficiency virus in utero. Transmission is by vertical transmission. Confirmation is by identification of human immunodeficiency virus in the neonate.
  • KA62.7 Congenital parvovirus syndrome - Fetal parvovirus syndrome is a fetopathy likely to occur when a pregnant woman is infected by parvovirus B19. Fetal parvovirus infection results in aplastic crisis. Anaemia induces a risk of hydrops and fetal death by cardiac failure in 10 to 20% of cases.
  • KA62.8 Congenital rubella syndrome - A disease caused by an infection with the rubella virus in utero. This disease presents with symptoms depending on the timing of infection of the fetus and may present with birth defects (such as hearing loss), or intrauterine growth retardation. Transmission is by vertical transmission. Confirmation is by identification of rubella virus or detection of anti-rubella virus IgM antibodies in the neonate or infant.
  • KA62.9 Congenital viral hepatitis - A disease of the liver affecting the neonate, caused by an infection with either hepatitis A, B, C, D, or E virus in utero. This disease is characterized by lethargy, jaundice, abdominal distention, failure to thrive, or clay coloured stools. Transmission is by vertical transmission. Confirmation is by identification of the hepatitis A, B, C, D, or E virus in a blood sample from the neonate.
  • KA62.A Perinatal Herpes simplex infection - Herpes simplex infection acquired during the perinatal period, normally from active herpes infection of the mother's genital tract, but may also be transmitted in utero.
viral infection | environmental abnormalities |  ICD-11 | Infections of the foetus or newborn

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”.


Links: Original ICD-10 page content

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. (2019, October 19) Embryology International Classification of Diseases. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/International_Classification_of_Diseases

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