Print this pageEndovascular Repair Of Iliac Artery Aneurysm In A Patient With Type IV Ehlers-Danlos Syndrome
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Britt H. Tonnessen, MD, W. Charles Sternbergh, III, MD, Krishna Mannava, MD, Samuel R. Money, MD
Ochsner Clinic Foundation, New Orleans, LA
BACKGROUND:
Ehlers-Danlos syndrome type IV (EDS-IV), a rare inherited disorder of procollagen III synthesis, is linked with aneurysmal disease and spontaneous vessel rupture. Due to the extreme fragility of these patients’ vessels, open vascular procedures and even diagnostic arteriography carries high morbidity and mortality. We report a case of endovascular repair for a symptomatic iliac artery aneurysm as an alternative to open repair in an EDS-IV patient.
CASE:
A 57-year old male with EDS-IV genotype presented with an incidental right iliac artery aneurysm. Past surgical history was significant for spontaneous hemothorax and emergent ligation of an unknown ruptured vessel in his neck. Physical exam revealed a non-tender, pulsatile right lower quadrant mass (RLQ) and normal extremity pulses bilaterally. Computed tomography (CT) imaging showed a 2.5-cm right common iliac aneurysm and 1.6-cm celiac aneurysm. 3 months later, repeat CT demonstrated 0.5-cm increase in the iliac aneurysm diameter; additionally, the patient had noted vague RLQ pain for one month.
Via right femoral artery cutdown, a Zenith (Cook®) stent-graft limb was deployed extending from the right common to external iliac artery. The right hypogastric artery was deliberately covered. A proximal type I endoleak was sealed with a balloon-expandable stent. A late-filling type II endoleak was untreated. The femoral arteriotomy was closed with pledgeted suture. His post-operative course was uncomplicated.
CONCLUSIONS:
To our knowledge, this case reports the first endovascular aneurysm repair in an EDS-IV patient. Endovascular intervention for EDS-IV vascular complications should be considered and is potentially safer than open surgery in these challenging patients.