Endovascular repair of a ruptured subclavian artery aneurysm in a patient with Ehlers–Danlos syndrome using a sandwich technique

Abstract

We present the case of a type IV Ehlers–Danlos syndrome patient with a ruptured right subclavian artery aneurysm and associated arteriovenous fistula who underwent successful endovascular repair requiring simultaneous stent graft repair of the innominate artery using a sandwich technique. A 17-year-old man with known type IV Ehlers–Danlos syndrome developed right neck and shoulder swelling. CTA study demonstrated a 17 × 13-cm ruptured subclavian artery aneurysm with an associated internal jugular vein arteriovenous fistula. In the hybrid suite, a 7 mm × 15-cm stent graft (Viabahn, WL Gore & Associates, Flagstaff, AZ) was advanced from the right brachial approach into the innominate artery. A separate wire was placed into the right carotid artery via the right femoral approach (7 Fr), and a 7 mm × 10-cm stent graft (Viabahn) was advanced into the innominate artery. An additional 8 mm × 10-cm stent graft (Viabahn) was placed from the right brachial approach to obtain a distal-landing zone in the axillary artery. Complex vascular anatomy, in which graft seal creation may be challenging, does not exclude endovascular approaches as the sandwich technique can be utilized as a suitable alternative to open repair.

Keywords

Ehlers–Danlos syndrome endovascular approaches sandwich technique endovascular repair

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References

Germain

. Ehlers-Danlos syndrome type IV. Orphanet J Rare Dis 2007; 2: 32 (Review)–32 (Review).

Germain

. The vascular Ehlers-Danlos syndrome. Curr Treat Options Cardiovasc Med 2006; 8: 121–127.

Germain

. Clinical and genetic features of vascular Ehlers-Danlos syndrome. Ann Vasc Surg 2002; 16: 391–397.

Pepin

Schwarze

Superti-Furga

. Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med 2000; 342: 673–680.

Pepin MG and Byers PH. Ehlers-Danlos syndrome type IV, 1999 (updated May 3, 2011). In: Pagon RA, Bird TD, Dolan CR, et al. (eds) GeneReviews™ [Internet]. Seattle, WA: University of Washington, Seattle, 1993. See http://www.ncbi.nlm.nih.gov/books/NBK1494/ (last checked 12 August 2013).

Karkos

Prasad

Mukhopadhyay

. Rupture of the abdominal aorta in patients with Ehlers-Danlos syndrome. Ann Vasc Surg 2000; 14: 274–277.

Bergqvist

. Ehlers-Danlos type IV syndrome. A review from a vascular surgical point of view. Eur J Surg 1996; 162: 163–170.

Oderich

Panneton

Bower

. The spectrum, management and clinical outcome of Ehlers-Danlos syndrome type IV: a 30-year experience. J Vasc Surg 2005; 42: 98–106.

Lobato AC and Camacho-Lobato L. A new technique to enhance endovascular thoracoabdominal aortic aneurysm therapy—the sandwich procedure. Semin Vasc Surg 2012; 25: 153–160.