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Start free with EleplanPancreatic arteriovenous malformation
ORPHA:693826Malformation syndrome
Also called Arteriovenous malformation of the pancreas
What it is
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.
Key facts
- Prevalence
- <1 / 1 000 000
- Age of onset
- All ages
- 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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