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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.

DOI:10.1155/2018/7862327
PMID:29666709
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5831973/
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/f173565aa761/CRIA2018-7862327.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6709/5831973/cb1ea8026d03/CRIA2018-7862327.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6709/5831973/f173565aa761/CRIA2018-7862327.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6709/5831973/cb1ea8026d03/CRIA2018-7862327.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6709/5831973/f173565aa761/CRIA2018-7862327.002.jpg

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本文引用的文献

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Anesthetic consideration for neuromuscular diseases.神经肌肉疾病的麻醉考量
Curr Opin Anaesthesiol. 2017 Jun;30(3):435-440. doi: 10.1097/ACO.0000000000000466.
2
The embryonic myosin R672C mutation that underlies Freeman-Sheldon syndrome impairs cross-bridge detachment and cycling in adult skeletal muscle.导致弗里曼-谢尔登综合征的胚胎肌球蛋白R672C突变会损害成年骨骼肌中的横桥分离和循环。
Hum Mol Genet. 2015 Jun 15;24(12):3348-58. doi: 10.1093/hmg/ddv084. Epub 2015 Mar 3.
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Anaesthesia challenges in Freeman-Sheldon syndrome.弗里曼-谢尔顿综合征的麻醉挑战
Indian J Anaesth. 2013 Nov;57(6):632-3. doi: 10.4103/0019-5049.123352.
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Anesthetic management of a patient with Freeman-Sheldon syndrome: case report and literature review.《一例 Freeman-Sheldon 综合征患者的麻醉管理:病例报告及文献复习》。
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