Splenic venous malformation
Synonyms: Splenic cavernous malformation · Splenic slow-flow venous malformation · Venous malformation of the spleen
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A rare venous malformation characterized by splenic non-encapsulated, non-neoplastic vascular channels of varying sizes (ranging from capillaries to cavernous vessels) containing slow-flowing blood. The majority of patients are asymptomatic and the condition is usually discovered incidentally. When patients are symptomatic, they may present with dyspnea, abdominal pain, splenomegaly, diarrhea, or constipation. Lesions are usually benign with no malignant potential. However, very large lesions may require splenectomy due to their increased risk of rupture, which may lead to intra-abdominal hemorrhage.
Names in Orphadata languages
9 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Splenic venous malformation | Splenic cavernous malformation · Splenic slow-flow venous malformation · Venous malformation of the spleen |
| fr | Malformation veineuse splénique | Malformation de la veine splénique · Malformation veineuse de la rate |
| de | Venöse Fehlbildung der Milz | Kavernöse Fehlbildung der Milz · Slow-flow venöse Malformation der Milz |
| es | Malformación venosa esplénica | Malformación cavernosa esplénica · Malformación venosa del bazo · Malformación venosa esplénica de flujo lento |
| it | Malformazione venosa splenica | Malformazione cavernosa splenica · Malformazione venosa della milza · Malformazione venosa splenica a flusso lento |
| nl | Veneuze malformatie van milt | Veneuze malformatie van milt met trage bloedstroom · Caverneuze malformatie van milt · Slow-flow veneuze malformatie van milt |
| pt | Malformação venosa esplâncnica | Malformação venosa do baço · Malformação venosa esplâncnica de fluxo reduzido · Malformação cavernosa esplâncnica |
| pl | Malformacja żyły śledzionowej | = en |
| cs | Venózní malformace sleziny | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D73.8 | NTBT | attributed | not yet validated |
| MONDO | 975910 | E | 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:715334Unifocal peripheral venous 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
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/688523
- This record as JSON: /data/d/688523.json