raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:284460

Acute annular outer retinopathy

Synonyms: AAOR

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

A rare, acquired retinal disorder characterized by unilateral, acute onset, rapidly progressive visual field loss. Sometimes patients have photopsia and complain of floaters. Typical ophthalmoscopic finding is a unilateral, yellowish-white annular intraretinal line, splitting the retinal field to affected outer retina with thinning, and normal retina. Gradual spontaneous visual recovery has been observed.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enAcute annular outer retinopathyAAOR
frRétinopathie externe annulaire aiguë= en
deRetinopathie, akute äußere ringförmige= en
esRetinopatía externa anular aguda= en
itRetinopatia esterna anulare acuta= en
nlAcute ringvormige perifere retinopathieAAOR · Acute annulaire perifere retinopathie
ptRetinopatia externa anular aguda
plOstra zewnętrzna retinopatia pierścieniowa= en
csAkutní anulární zevní retinopatie= en
ukГостра кільцева зовнішня ретинопатія= en
zh急性环状外视网膜病变= en
trAkut anüler dış retinopati= en

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10H35.8NTBTattributedvalidated
ICD-119B71.YNTBTattributedvalidated
MONDO0017299Evalidated
UMLSC5191002Evalidated

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/families12 casesWorldwideCase(s)Validated16181293[PMID]
Point prevalence<1 / 1 000 000—WorldwideClass onlyValidatedORPHANET

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