Kocamanoglu Ismail Serhat, Sarihasan Esra
MD; Associate Professor, Department of Anesthesia, Ondokuz Mayis School of Medicine, Samsun, Turkey.
MR, Department of Anesthesia, Ondokuz Mayis School of Medicine, Samsun, Turkey.
Braz J Anesthesiol. 2013 Mar-Apr;63(2):220-2. doi: 10.1016/j.bjane.2012.06.005. Epub 2013 Aug 13.
Leigh syndrome (LS) is a rare disease caused by abnormalities of mitochondrial energy generation. The central nervous system is most frequently affected, with psychomotor underdevelopment, seizures, nystagmus, ophthalmoparesis, optic atrophy, ataxia, dystonia, or respiratory failure. Surgical and anesthetic procedures stimulate the tracheal irritability, and could exacerbate risks of aspiration, wheezing, breathing difficulties, gasping, hypoventilation, and apnea.
We present the anesthetic management for a six-year-old boy with severe form of LS, involving repair of a femur fracture. Propofol and remifentanil were infused for general anesthesia. The patient was closely monitored during anesthesia and in the intensive care unit in the early postoperative period.
Close intraoperative monitoring of patients, including invasive arterial blood pressure monitoring and frequently measuring the levels of blood gases, glucose, and lactate, made this procedure run smoothly. Intensive care and breathing support for the patient with LS, under sedation with an analgesic combination during the early postoperative period, minimized the stress response due to pain after surgery.
Leigh综合征（LS）是一种由线粒体能量生成异常引起的罕见疾病。中枢神经系统最常受累，表现为精神运动发育迟缓、癫痫发作、眼球震颤、眼肌麻痹、视神经萎缩、共济失调、肌张力障碍或呼吸衰竭。外科手术和麻醉操作会刺激气管易激惹，可能会加重误吸、喘息、呼吸困难、喘气、通气不足和呼吸暂停的风险。
我们介绍了一名患有严重LS的六岁男孩股骨骨折修复手术的麻醉管理。采用丙泊酚和瑞芬太尼静脉输注进行全身麻醉。在麻醉期间及术后早期重症监护病房对患者进行密切监测。
术中对患者进行密切监测，包括有创动脉血压监测以及频繁测量血气、血糖和乳酸水平，使手术顺利进行。术后早期对LS患者进行重症监护和呼吸支持，并使用镇痛合剂进行镇静，最大限度地减少了术后疼痛引起的应激反应。
