raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:693872

Urinary tract arteriovenous malformation

Synonyms: Arteriovenous malformation of the urinary tract

Type
Malformation syndrome
Group
Disorder
Inheritance
Not applicable
Age of onset
Antenatal, Infancy, Neonatal
Status
active

A rare visceral arteriovenous malformation characterized by abnormal communication between veins and arteries of the ureter, urethra, or urinary bladder. Major clinical signs include hematuria and suprapubic pain. Additional clinical features may include acute urinary retention and increased urinary frequency.

1

Names in Orphadata languages

9 of 12 languages
LangNameSynonyms
enUrinary tract arteriovenous malformationArteriovenous malformation of the urinary tract
frMalformation artérioveineuse des voies urinairesMalformation artérioveineuse de l'appareil urinaire
deArteriovenöse Fehlbildung der Harnwege
esMalformación arteriovenosa del tracto urinarioMalformación arteriovenosa de las vías urinarias
itMalformazione arterovenosa delle vie urinarie
nlArterioveneuze malformatie van urinewegen
ptMalformação arteriovenosa do tracto urinárioTracto urinário com malformação arteriovenosa
plMalformacja tętniczo-żylna układu moczowego= en
csUrinary tract arteriovenous malformation= en

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10Q27.3NTBTattributedvalidated
ICD-11LA90.3YNTBTattributedvalidated

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

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—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