Pancreatic arteriovenous malformation
Synonyms: Arteriovenous malformation of the pancreas
- Type
- Malformation syndrome
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare visceral arteriovenous malformation of the pancreas characterized by abnormal blood flow from the arterial system directly into the venous system without passing through the capillaries. Patients may present with episodic gastrointestinal bleeding or hemoperitoneum, epigastric or abdominal pain, pancreatitis, portal hypertension, and pseudocysts. Some patients remain asymptomatic.
Names in Orphadata languages
9 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pancreatic arteriovenous malformation | Arteriovenous malformation of the pancreas |
| fr | Malformation artérioveineuse pancréatique | MAVP · Malformation artérioveineuse du pancréas |
| de | Pankreatische arteriovenöse Fehlbildung | Arteriovenöse Fehlbildung der Bauchspeicheldrüse |
| es | Malformación arteriovenosa pancreática | Malformación arteriovenosa del páncreas |
| it | Malformazione arterovenosa pancreatica | Malformazione arterovenosa del pancreas |
| nl | Arterioveneuze malformatie van pancreas | Pancreatische arterioveneuze malformatie |
| pt | Malformação arteriovenosa pancreática | Malformação arteriovenosa do pâncreas |
| pl | Malformacja tętniczo-żylna trzustki | = en |
| cs | Pancreatic 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 | 100 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/693826
- This record as JSON: /data/d/693826.json