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患有弗里曼-谢尔顿综合征的成年患者行心脏直视手术的麻醉注意事项。

Anesthetic Considerations for an Adult Patient with Freeman-Sheldon Syndrome Undergoing Open Heart Surgery.

作者信息

Viehmeyer S, Gabriel P, Bauer K, Bauer S, Sodian R, Hilberath J N

机构信息

Department of Anesthesiology and Critical Care Medicine, MediClin Heart Institute Lahr/Baden, Lahr, Germany.

Department of Cardiac Surgery, MediClin Heart Institute Lahr/Baden, Lahr, Germany.

出版信息

Case Rep Anesthesiol. 2018 Feb 13;2018:7862327. doi: 10.1155/2018/7862327. eCollection 2018.

Abstract

Freeman-Sheldon syndrome (FSS) or "whistling face" syndrome is a rare congenital disorder complicated by characteristic facial deformities and muscular contractures. We report on a 64-year-old male patient presenting for surgical replacement of his aortic valve and review the available literature on anesthetic considerations and perioperative management principles. FSS frequently poses a significant challenge to airway management and gaining vascular access. Moreover, these patients are reportedly at risk for developing malignant hyperthermia (MH) or neuroleptic malignant syndrome.

摘要

弗里曼-谢尔顿综合征(FSS)或“吹口哨脸”综合征是一种罕见的先天性疾病,伴有特征性面部畸形和肌肉挛缩。我们报告一例64岁男性患者,因主动脉瓣置换术前来就诊,并回顾了有关麻醉注意事项和围手术期管理原则的现有文献。FSS常常给气道管理和建立血管通路带来重大挑战。此外,据报道这些患者有发生恶性高热(MH)或抗精神病药恶性综合征的风险。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6709/5831973/cb1ea8026d03/CRIA2018-7862327.001.jpg

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