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Start free with EleplanSplenic arteriovenous malformation
ORPHA:693863Malformation syndrome
Also called Arteriovenous malformation of the spleen
What it is
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.
Key facts
- Prevalence
- <1 / 1 000 000
- Age of onset
- Neonatal
- Inheritance
- Not applicable
- Classified as
- Malformation syndrome
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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