Splenic arteriovenous fistula (SAVF) without a splenic arterial aneurysm is a rare entity and may either be congenital or acquired. Acquired SAVFs are commonly due to adjacent splenic arterial aneurysm rupture, trauma or infection. We report a large idiopathic SAVF in a 50-year-old woman who had no symptoms. CT showed that the fistula was around the splenic hilum and no other specific findings. SAVF has a risk of sudden onset of portal hypertension and it should be treated as soon as diagnosed. Endovascular treatment for SAVF is now being performed successfully with increasing frequency, however, there is still no definitive evidence of treatment for SAVF. Treatment options should be determined appropriately depending on individual case characteristics and vascular anatomical variations. Our case was treated successfully with splenectomy, and long-term results are good without need of reintervention.
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