Loeys-Dietz综合征患儿完全性主动脉弓置换术的麻醉处理。

IF 0.5 4区医学 Q4 SURGERY

Cirugia Y Cirujanos Pub Date : 2023-01-01 DOI:10.24875/CIRU.21000786

Unai Olabarrieta-Zarain, Leire Martínez-Santos, Alex Alberdi-Enríquez, Ane Guereca-Gala, Blanca Bravo-Sevilla, Alberto Martínez-Ruiz

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引用次数: 0

摘要

Loeys-Dietz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、血管扭曲和骨骼表现。预后很差，不进行手术治疗的患者在27岁时死亡。尽管在儿童时期被诊断出来，但在儿童中并不常见手术主动脉置换术。我们报告一例患有LDS和胸主动脉多发动脉瘤的12岁儿童，由于手术复杂性和对儿科人群的影响，我们建议进行完全主动脉弓置换术和麻醉处理。

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Anesthetic management of a child with Loeys-Dietz syndrome undergoing complete aortic arch replacement.

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.

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来源期刊

Cirugia Y Cirujanos 医学-外科

CiteScore

0.90

自引率

20.00%

发文量

207

审稿时长

6-12 weeks

期刊介绍： Cirugía y Cirujanoses exponente del desarrollo académico, científico, médico, quirúrgico y tecnológico en materia de salud en México y en el ámbito internacional. Es una revista bimestral, open access, revisada por pares, que publica en español y en inglés (traducido sin coste para los autores) artículos científicos originales, casos clínicos, artículos de revisión de interés general y cartas al editor. Los artículos se seleccionan y publican siguiendo un riguroso análisis, de acuerdo con los estándares internacionalmente aceptados. Sus espacios están abiertos a los académicos, así como a todo miembro de la comunidad médica que manifieste interés por utilizar este foro para publicar sus trabajos.