raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:141179

Non-involuting congenital hemangioma

Synonyms: NICH · Non-involuting congenital haemangioma

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

A rare congenital hemangioma characterized by flat or slightly bossed, round or oval shape, usually solitary, that are fully formed in utero and differ from rapidly involuting congenital hemangiomas (RICH) mainly because they do not undergo a postnatal involuting phase.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enNon-involuting congenital hemangiomaNICH · Non-involuting congenital haemangioma
frHémangiome congénital non involutifNICH
deNicht-involutierendes kongenitales HämangiomNICH
esHemangioma congénito no involutivoNICH
itEmangioma congenito non involutivoNICH
nlNiet-involuerend congenitaal hemangioomNICH
ptHemangioma não evolutivo, congénito
plNaczyniak wrodzony niezanikającyNICH
csNeinvolující vrozený hemangiomNICH
ukНеінвазивна вроджена гемангіомаNICH
zh非消退性先天性血管瘤NICH
trGerilemeyen doğumsal hemanjiyomNICH

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10D18.0NTBTattributedvalidated
ICD-112E81.2YNTBTindexvalidated
ICD-11XH5427Especificvalidated
MONDO0015403Evalidated
UMLSC1275417Evalidated
GARD10890Evalidated

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 (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—WorldwideClass onlyNot yet validatedORPHANET

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

12 annotations · validated 2019-05-15
HPOTerm (as annotated by Orphanet)Diagnostic
Obligate (100%)
HP:0001028HemangiomaDiagnostic criterion
Frequent (79-30%)
HP:0007466Midfrontal capillary hemangioma
HP:0031449Perineal hemangioma
HP:0007618Subcutaneous calcification
HP:0100585Telangiectasia of the skin
Occasional (29-5%)
HP:0001635Congestive heart failure
HP:0031207Hepatic hemangioma
HP:0100784Peripheral arteriovenous fistula
HP:0001015Prominent superficial veins
HP:0001873Thrombocytopenia
HP:0410266Visceral hemangioma
Excluded (0%)
HP:0012329Tufted angioma

Run the differential on the 1 obligate / very frequent terms

Annotation source: 27058448[PMID]_18676554[PMID]_27540637[PMID]

7

Sources for this card