Olabarrieta-Zarain Unai, Martínez-Santos Leire, Alberdi-Enríquez Alex, Guereca-Gala Ane, Bravo-Sevilla Blanca, Martínez-Ruiz Alberto
Servicio de Anestesiología, Reanimación y Terapia de Dolor, Hospital Universitario de Cruces, Osakidetza.
Facultad de Medicina, Universidad País Vasco, EHU/UPV. Vizcaya, España.
Cir Cir. 2023;91(4):576-580. doi: 10.24875/CIRU.21000786.
Loeys-Dietz syndrome (LDS) is a connective tissue disease related to β-transforming growth factor mutations, which causes aneurysms formation, vascular tortuosity and skeletal manifestations. The prognosis is very poor, and mortality occurs at the age of 27 in patients without surgical treatment. Despite being diagnosed in childhood, is not usual surgical aortic replacement in children. We report a case of 12 years old child with LDS and multiple aneurysms in thoracic aorta, undergoing complete aortic arch replacement and our proposal for the anesthetic management, due to surgical complexity and implications in pediatric population.
洛伊斯-迪茨综合征（LDS）是一种与β转化生长因子突变相关的结缔组织疾病，可导致动脉瘤形成、血管迂曲和骨骼表现。预后非常差，未经手术治疗的患者在27岁时会死亡。尽管在儿童期被诊断出，但儿童进行主动脉置换手术并不常见。我们报告一例12岁患有LDS且胸主动脉有多个动脉瘤的儿童病例，因其手术复杂性及对儿科人群的影响，该患儿接受了全主动脉弓置换术以及我们提出的麻醉管理方案。
