Splenic arteriovenous malformation
Synonyms: Arteriovenous malformation of the spleen
- Type
- Malformation syndrome
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Neonatal
- Status
- active
A rare visceral arteriovenous malformation characterized by abnormal communication between splenic artery and the splenic vein at the level of the splenic hilum. These malformations may lead to the formation of splenic artery aneurysms that can rupture. Clinical features include abdominal pain, hematemesis, melena, diarrhea, and portal hypertension due to venous congestion. The spleen is typically enlarged with extensive areas of infarction and dilated vessels.
Names in Orphadata languages
9 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Splenic arteriovenous malformation | Arteriovenous malformation of the spleen |
| fr | Malformation artérioveineuse splénique | Malformation artérioveineuse de la rate · Malformation artérioveineuse des vaisseaux spléniques |
| de | Splenische arteriovenöse Fehlbildung | Arteriovenöse Fehlbildung der Milz |
| es | Malformación arteriovenosa esplénica | Malformación arteriovenosa del bazo |
| it | Malformazione arterovenosa splenica | Malformazione arterovenosa della milza |
| nl | Arterioveneuze malformatie van milt | Splenische vasculaire malformatie |
| pt | Malformação arteriovenosa esplénica | Malformação arteriovenosa do baço |
| pl | Malformacja tętniczo-żylna śledziony | = en |
| cs | Splenic arteriovenous malformation | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q27.3 | NTBT | attributed | validated |
| ICD-11 | LA90.3Y | NTBT | attributed | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:108971Non-syndromic visceral malformation of the liver, biliary tract, pancreas or spleen
rare developmental anomalies during embryogenesis; rare abdominal surgical diseases; rare transplant-related disorders - ORPHA:693855Visceral fast-flow vascular malformation
rare developmental anomalies during embryogenesis; rare circulatory system diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 40 cases | Worldwide | Case(s) | Validated | ORPHANET | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | 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.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/693863
- This record as JSON: /data/d/693863.json