raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:353351

Idiopathic macular telangiectasia type 3

Synonyms: Occlusive idiopathic juxtafoveolar retinal telangiectasis

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

Idiopathic macular telangiectasia type 3 is a rare, acquired, eye disease characterized by progressive visual loss, due to bilateral juxtafoveolar capillary occlusions, capillary telangiectasia, and minimal exudation. It is associated with systemic or cerebral vascular occlusive disease.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enIdiopathic macular telangiectasia type 3Occlusive idiopathic juxtafoveolar retinal telangiectasis
frTélangiectasie maculaire idiopathique type 3Télangiectasie rétinienne juxtafovéolaire idiopathique occlusive
deIdiopathische makuläre Teleangiektasie Typ 3= en
esTelangiectasia macular idiopática tipo 3Telangiectasia retiniana oclusiva yuxtafoveolar idiopática
itTelangectasia maculare idiopatica, tipo 3Telangectasia retinica iuxtafoveale idiopatica occlusiva
nlIdiopathische maculaire teleangiëctasie type 3Occlusieve idiopathische juxtafoveolaire retinale teleangiëctasie
ptTelangiectasia idiopática macular, tipo 3
plIdiopatyczna teleangiektazja plamki typu 3Zamykająca idiopatyczna teleangiektazja okołodołeczkowa siatkówki
csIdiopatická makulární teleangiektázie, typ 3Okluzivní idiopatické juxtafoveolární releangiektázie sítnice
ukІдіопатична макулярна телеангіектазія, тип 3Оклюзійний ідіопатичний юкстафовеолярний телеангіоектаз сітківки
zh特发性黄斑毛细血管扩张症3型阻塞性特发性黄斑旁视网膜毛细血管扩张症
trİdiyopatik maküler telenjiektazi tip 3Oklüzif idiyopatik jukstafoveolar retinal telenjiektazi

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10H35.0NTBTattributedvalidated
ICD-119B75.3NTBTattributedvalidated
MONDO0018147Evalidated
UMLSC4751436Evalidated

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

2 records
TypeClassValueAreaQualificationValidationSource
Cases/families9 casesWorldwideCase(s)Validated22219035[PMID]_ORPHANET
Point prevalence<1 / 1 000 000—WorldwideClass onlyValidated22219035[PMID]_ORPHANET

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

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card