raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:693815

Uterine arteriovenous malformation

Synonyms: Arteriovenous malformation of the uterus · UAVM · UVM · Uterine vascular malformation

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

A rare visceral arteriovenous malformation characterized by abnormal connections between uterine arteries and veins. Vaginal bleeding is the most common clinical feature, may be very severe, and is refractory to conventional treatments. Variable clinical features may include abdominal pulsatile masses, frequent urination or urinary incontinence, dyspareunia, and hematuria. Some patients remain asymptomatic.

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Names in Orphadata languages

9 of 12 languages
LangNameSynonyms
enUterine arteriovenous malformationArteriovenous malformation of the uterus · UAVM · UVM · Uterine vascular malformation
frMalformation artérioveineuse utérineMAV utérine · MAVU · Malformation artérioveineuse de l'uterus
deArteriovenöse Fehlbildung des UterusUAVM · UVM · Vaskuläre Malformation des Uterus
esMalformación arteriovenosa uterinaMalformación arteriovenosa del útero · Malformación vascular uterina · UAVM · UVM
itMalformazione arterovenosa uterinaMalformazione arterovenosa dell'utero · UAVM · UVM · Malformazione vascolare uterina
nlArterioveneuze malformatie van uterusArterioveneuze malformatie van baarmoeder · UAVM · UVM · Uteriene arterioveneuze malformatie · Uteriene vasculaire malformatie
ptMalformação arteriovenosa uterinaMalformação arteriovenosa do útero · Malformação vascular uterina · UAVM · UVM
plMalformacja tętniczo-żylna macicy= en
csUterine arteriovenous malformation= en

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10Q27.3NTBTattributedvalidated
ICD-11LA90.32Especificvalidated

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 (2):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

2 records
TypeClassValueAreaQualificationValidationSource
Cases/families150 casesWorldwideCase(s)ValidatedORPHANET
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.

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Sources for this card