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线粒体耗竭综合征合并肥厚型心肌病小儿患者行脊柱侧弯矫正术的麻醉管理:1例报告

Anesthetic Management of a Pediatric Patient With Mitochondrial Depletion Syndrome and Hypertrophic Cardiomyopathy Undergoing Scoliosis Correction: A Case Report.

作者信息

Alkuwaiti Ali, Albuthi Muneera, Mahmoud Ahmed Haroun M, Gosty Ghidaa, Alselaiti Abdulrahman A

机构信息

Pediatric Anesthesia, King Abdulaziz Medical City Riyadh, Riyadh, SAU.

Medical Education, College of Medicine, King Abdulaziz Medical City Riyadh, Riyadh, SAU.

出版信息

Cureus. 2025 Jul 24;17(7):e88669. doi: 10.7759/cureus.88669. eCollection 2025 Jul.

Abstract

This report describes a case of a 15-year-old pediatric patient with mitochondrial depletion syndrome, associated with hypertrophic cardiomyopathy, who underwent scoliosis correction surgery under neurophysiological monitoring. As propofol is mitochondrion-toxic, the anesthetic approach was modified to involve dexmedetomidine infusion combined with boluses of midazolam and ketamine. The patient was hemodynamically stable during surgery with effective neuromonitoring readings, and the surgery was concluded uneventfully. This case highlights the safety and efficacy of this anesthetic regimen in managing patients with complex metabolic and cardiac conditions.

摘要

本报告描述了一名15岁患有线粒体耗竭综合征并伴有肥厚型心肌病的儿科患者,在神经生理监测下接受脊柱侧弯矫正手术的病例。由于丙泊酚具有线粒体毒性,麻醉方法改为输注右美托咪定并联合推注咪达唑仑和氯胺酮。患者在手术期间血流动力学稳定,神经监测读数有效,手术顺利结束。该病例突出了这种麻醉方案在处理患有复杂代谢和心脏疾病患者时的安全性和有效性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e53/12374603/8b541682efd7/cureus-0017-00000088669-i01.jpg

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