Port J Card Thorac Vasc Surg. 2026 May 10;33(1):45-49. doi: 10.48729/pjctvs.567.
ABSTRACT
Vascular Ehlers-Danlos Syndrome (VEDS) is a rare genetic disorder characterized by arterial fragility and a high risk of spontaneous vascular rupture. Splenic artery rupture is an uncommon but life-threatening manifestation of this condition. We report the case of a 34-year-old male with VEDS who presented with sudden abdominal pain and rapid progression to hemorrhagic shock. Imaging confirmed active bleeding from the distal splenic artery. After multidisciplinary evaluation, endovascular embolization via a left humeral approach was performed using multiple coils. The procedure achieved hemostasis, and the patient showed immediate clinical improvement. He was discharged after 27 days following immunization against encapsulated organisms due to presumed hyposplenism. This case highlights the critical role of endovascular techniques in managing vascular emergencies in VEDS patients, offering a minimally invasive and effective alternative to open surgery, minimizing vascular manipulation in fragile tissues. Splenic artery embolization may be life-saving, but long-term surveillance and preventive strategies are crucial, given the risk of recurrence and complications.
PMID:42155133 | DOI:10.48729/pjctvs.567