A 37-year-old man presented to the emergency department with abdominal pain and shock. He underwent endovascular embolization for splenic arterial aneurysm rupture caused by Ehlers-Danlos syndrome, which had been confirmed by genetic testing three years prior. Computed tomography showed an intra-abdominal hemorrhage, celiac arterial aneurysm, and embolic coils in the splenic artery (Picture 1, white and black arrows and arrowhead, respectively). Emergency angiography also showed a celiac arterial aneurysm without contrast extravasation and an embolic coil in the splenic artery (Picture 2, black and white arrows, respectively). The successful occlusion of the celiac artery aneurysm and its branches was achieved using Interlock Fibered IDC coils (Boston Scientific, Natick, MA, USA) (Picture 3). The patient was discharged on postoperative day 17 without abdominal organ ischemia. Arterial aneurysm rupture is a life-threatening complication of vascular Ehlers-Danlos syndrome (1). Arterial aneurysm rupture can occur repeatedly; therefore, long-term follow-up is necessary with β1-antagonist administration and regular non-invasive imaging examinations (1,2).
Picture 1.
Picture 2.
Picture 3.
The authors state that they have no Conflict of Interest (COI).
References
- 1. Bergqvist D, Björck M, Wanhainen A. Treatment of vascular Ehlers-Danlos syndrome: a systematic review. Ann Surg 258: 257-261, 2013. [DOI] [PubMed] [Google Scholar]
- 2. Ong KT, Perdu J, De Backer J, et al. Effect of celiprolol on prevention of cardiovascular events in vascular Ehlers-Danlos syndrome: a prospective randomised, open, blinded-endpoints trial. Lancet 376: 1476-1484, 2010. [DOI] [PubMed] [Google Scholar]