Endovascular Iliac Artery Aneurysm Repair With Iliac Branch Endoprosthesis in Type III Loeys-Dietz Syndrome

Abstract

Loeys-Dietz syndrome (LDS) type III is a rare connective tissue disorder caused by SMAD3 gene mutations that manifests with arterial tortuosity, systemic aneurysms, aortic root dilatation, and osteoarthritis. The endovascular treatment of iliac artery aneurysms with an iliac branch endoprosthesis (IBE) in LDS patients has not been described. We have presented the safety and technical feasibility of branched endovascular iliac artery aneurysm repair in a patient with a rare variant SMAD3 mutation resulting in LDS type III. A 52-year-old man with an ascending aortic root aneurysm, intercostal artery aneurysm, infrarenal abdominal aortic aneurysm, and bilateral common iliac artery (CIA) aneurysms underwent extended gene analysis. He was found to have a rare variant heterozygous autosomal dominant SMAD3 gene mutation (p.I396N) consistent with LDS type III. Because the patient was in extremis from infrarenal abdominal aortic aneurysm rupture, he underwent open replacement with a bifurcated Dacron graft and ligation of the right hypogastric artery at an unaffiliated hospital. Computed tomography angiography revealed a residual 4.7-cm left CIA aneurysm and an anatomic constraint for the IBE (proximal CIA diameter, <17 mm; Fig 1). The previously described alternative endograft aortoiliac reconstruction technique facilitated IBE repair by creation of an adequate proximal CIA landing zone with an Ovation limb (Endologix, Irving, Calif). The contralateral gate was cannulated through a left axillary cutdown. Because of a tortuous hypogastric artery, Viabahn endoprostheses (W.L. Gore & Associates, Flagstaff, Ariz) were used for hypogastric extensions, with a bridging Gore VBX stent graft. No perioperative complications occurred. At the 5-month follow-up visit, he was alive without cardiovascular morbidity. Computed tomography angiography showed branch vessel patency and sac regression, with no evidence of an endoleak or stent graft migration (Fig 2). Although branched endovascular iliac artery aneurysm repair is not the historical treatment of choice for patients with LDS type III, IBE repair can be a safe and effective minimally invasive treatment option for anatomically suitable patients.Fig 2Postoperative three-dimensional computed tomography angiogram showing patent left common iliac, external iliac, and hypogastric arteries with no evidence of endoleak or stent graft migration.View Large Image Figure ViewerDownload Hi-res image Download (PPT)