Abstract
Vascular Ehlers-Danlos syndrome is associated with severe arterial fragility and life-threatening visceral complications. We report a 30-year-old woman with genetically confirmed vascular Ehlers-Danlos syndrome who presented with abdominal pain, nausea, and emesis. Persistent symptoms prompted serial computed tomography angiography (CTA), which revealed progressive celiac trunk and splenic artery dissections with multiple pseudoaneurysms. Given interval pseudoaneurysm growth and ongoing pain, she underwent joint endovascular repair by interventional radiology and vascular surgery via open left femoral exposure, selective catheterization, and combined Onyx and detachable coil embolization. Postprocedure CTA showed stable pseudoaneurysm exclusion with preserved distal perfusion. At 30-day follow-up, she remained asymptomatic with durable repair on CTA, demonstrating successful repair using combined Onyx-coil embolization. However, at 5 years, she had rupture of a more distal segment of the splenic artery, highlighting the need for continued surveillance even in the setting of repair.