raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:399307

Idiopathic avascular necrosis

Synonyms: Idiopathic AVN

Type
Disease
Group
Disorder
Inheritance
Not applicable
Status
active

A rare osteonecrosis characterized by bone necrosis due to disrupted blood supply in the absence of a known cause. Affected bones include the femoral head, talus, vertebral body, humerus, and scaphoid, among others. Patients may initially be asymptomatic but subsequently present with gradually developing refractory pain, swelling, and reduced range of motion. If left untreated, the condition may progress to bone collapse with secondary degeneration, fragmentation, and pathological fracture, as well as osteoarthritis.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enIdiopathic avascular necrosisIdiopathic AVN
frNécrose avasculaire idiopathiqueAVN idiopathique
deAvaskuläre Nekrose, idiopathischeIdiopathische AVN
esNecrosis avascular idiopáticaAVN idiopática
itNecrosi avascolare idiopaticaAVN idiopatica
nlIdiopathische avasculaire necroseIdiopathische AVN
ptNecrose avascular idiopática
plIdiopatyczna martwica awaskularnaIdiopatyczna AVN
csIdiopatická avaskulární nekrózaIdiopatická AVN
ukІдіопатичний аваскулярний некрозІдіопатичний АВН
zh特发性缺血性坏死特发性AVN
trİdiyopatik avasküler nekrozİdyopatik AVN

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10M87.0Especificvalidated
ICD-11FB82.1NTBTindexvalidated
MONDO0018380Evalidated
UMLSC1404767Evalidated

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

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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

0 records

No prevalence record in this release.

6

HPO phenotypes

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

7

Sources for this card