Open repair of abdominal aortic aneurysms in patients with vascular Ehlers-Danlos syndrome

被引：6
|
作者：
Dittman, James M. [1 ]
Saldana-Ruiz, Nallely [1 ]
Newhall, Karina [2 ]
Byers, Peter H. [3 ]
Starnes, Benjamin W. [1 ]
Shalhub, Sherene [1 ,4 ,5 ]
机构：
[1] Univ Washington, Dept Surg, Div Vasc Surg, Seattle, WA 98195 USA
[2] Univ Rochester, Dept Surg, Div Vasc Surg, Rochester, MN 14620 USA
[3] Univ Washington, Dept Lab Med & Pathol, Seattle, WA USA
[4] Oregon Hlth & Sci Univ, Dept Surg, Div Vasc & Endovasc Surg, Portland, OR 97239 USA
[5] Oregon Hlth & Sci Univ, Dept Mol & Med Genet, Dept Surg & Mol & Med Genet, Div Vasc & Endovasc Surg, 3181 SW Sam Jackson Pk Rd, Portland, OR 97239 USA
来源：
JOURNAL OF VASCULAR SURGERY CASES INNOVATIONS AND TECHNIQUES | 2023年 / 9卷 / 02期
关键词：
Abdominal aortic aneurysm; Connective tissue disorder; Genetic aortopathy; Vascular Ehlers-Danlos syndrome; SYNDROME TYPE-IV; MANAGEMENT; COMPLICATIONS; DIAGNOSIS; PHENOTYPE;
D O I：
10.1016/j.jvscit.2023.101194
中图分类号：
R61 [外科手术学];
学科分类号：
摘要：
Vascular Ehlers-Danlos syndrome (VEDS) is rare, affecting an estimated 1 per 50,000 individuals, and is associated with abdominal aortic aneurysms (AAAs), among other arteriopathies. We present three patients with genetically confirmed VEDS who underwent successful open AAA surgical repair and demonstrate that elective open AAA repair with careful tissue manipulation is safe and feasible for patients with VEDS. These cases also demonstrate that the VEDS genotype is associated with the aortic tissue quality (genotypeesurgical phenotype correlation), with the most friable tissue encountered in the patient with a large amino acid substitution and the least friable tissue in the patient with a null (haploinsufficiency) variant. (J Vasc Surg Cases Innov Tech 2023;9:101194.)
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页数：6
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