raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:291

Congenital varicella syndrome

Synonyms: Antenatal varicella virus infection · Mother-to-child transmission of varicella syndrome

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
Antenatal, Neonatal
Status
active

A rare acquired developmental anomaly syndrome characterized by skin, neurological, ocular, limbs and growth defects secondary to maternal Varicella-Zoster Virus (VZV) infection.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enCongenital varicella syndromeAntenatal varicella virus infection · Mother-to-child transmission of varicella syndrome
frSyndrome de varicelle congénitaleEmbryopathie au virus de la varicelle
deVarizellen-Syndrom, kongenitalesVaricella-Virus-Infektion, pränatale · Varizellen-Embryo-Fetopathie · Varizellen-Syndrom durch Mutter-Kind-Übertragung · Varizellen-Syndrom, fetales
esSíndrome de varicela congénitaInfección prenatal por el virus de la varicela · Transmisión materna del síndrome de varicela · Transmisión materna del síndrome de varicela congénita
itSindrome da varicella fetaleInfezione prenatale da virus della varicella
nlCongenitaal varicellasyndroomPrenatale infectie door varicellavirus · Transmissie van moeder op kind van varicellasyndroom
ptSíndrome varicela congénitaInfeção pré-natal pelo vírus da varicela · Síndrome varicela fetal
plZespół wrodzonej ospy wietrznejPrenatalne zakażenie wirusem ospy wietrznej · Prenatalne zakażenie wirusem ospy wietrznej
csVrozený varicellový syndromPrenatální nákaza virem vericelly
ukВроджений синдром вітряної віспиАнтенатальна інфекція вірусу вітряної віспи · Передача вітряної віспи від матері до дитини
zh先天性水痘综合征母婴传播型水痘综合征
trDoğumsal suçiçeği sendromuAntenatal suçiçeği virüsü enfeksiyonu · Su çiçeği sendromunun anneden çocuğa bulaşması

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10P35.8NTBTinclusionvalidated
ICD-11KA62.2NTBTindexvalidated
MONDO0017372Evalidated
UMLSC4275251Evalidated
GARD45Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (2):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

2 records
TypeClassValueAreaQualificationValidationSource
Cases/families130 casesWorldwideCase(s)Validated26965725[PMID]
Point prevalenceUnknown—WorldwideClass onlyValidatedORPHANET_26965725[PMID]

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

9 annotations · validated 2016-06-01
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0000987Atypical scarring of skin
HP:0001511Intrauterine growth retardation
Frequent (79-30%)
HP:0000518Cataract
HP:0002120Cerebral cortical atrophy
HP:0001263Global developmental delay
HP:0001249Intellectual disability
HP:0000252Microcephaly
HP:0002983Micromelia
HP:0000568Microphthalmia

Run the differential on the 2 obligate / very frequent terms

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Sources for this card