Hashizume Kenichi, Shimizu Hideyuki, Honda Masanori, Inoue Shinya, Takaki Hidenobu, Hayashi Kanako, Kaneyama Hiroaki
Department of Cardiovascular Surgery, Saiseikai Utsunomiya Hospital, Utsunomiya, Tochigi, Japan.
Department of Cardiovascular Surgery, Keio University School of Medicine, Shinjuku, Tokyo, Japan.
Ann Thorac Surg. 2017 Jul;104(1):e39-e42. doi: 10.1016/j.athoracsur.2017.02.017.
Loeys-Dietz syndrome (LDS) is a rare connective tissue disorder (CTD) caused by mutations in the gene encoding transforming growth factor-β receptors Ⅰ and Ⅱ. Patients with LDS manifest spontaneous aneurysms and dissections of the aorta and peripheral artery. We report a successful treatment with a hybrid endovascular repair for a rapidly expanding thoracoabdominal aneurysm in a 41-year-old woman affected by LDS. To overcome the difficulties of anatomical and surgical repair, we applied an original strategy using surgeon-modified fenestrated endografts.
洛伊氏综合征（LDS）是一种罕见的结缔组织疾病（CTD），由编码转化生长因子-β受体Ⅰ和Ⅱ的基因突变引起。LDS患者表现为主动脉和外周动脉的自发性动脉瘤和夹层。我们报告了一例对一名41岁患LDS的女性患者迅速扩张的胸腹主动脉瘤进行杂交血管腔内修复的成功治疗案例。为克服解剖和手术修复的困难，我们采用了一种使用外科医生改良开窗型血管内移植物的原创策略。
