University of Health Sciences, Antalya Training and Research Hospital, Department of Anaesthesiology and Reanimation, Antalya, Turkey.
University of Health Sciences, Antalya Training and Research Hospital, Department of Anaesthesiology and Reanimation, Antalya, Turkey.
Braz J Anesthesiol. 2021 Jan-Feb;71(1):84-86. doi: 10.1016/j.bjane.2020.09.010. Epub 2020 Dec 28.
Mitochondrial Neurogastrointestinal Encephalomyopathy (MNGIE) is a rare autosomal-recessive disorder which is due to mutations in TYMP. The case reported here is of an 18-year-old male with MNGIE syndrome who presented for two different operations on two different occasions under regional anesthesia. The patient presented with urinary incontinence and abdominal pain. A cystoscopy under spinal anesthesia was scheduled. At 3 months after discharge, gastric perforation was diagnosed and combined spinal-epidural anesthesia, surgical repair was planned. Surgical and perioperative periods were uneventful. Based on this experience, we believe that regional anesthesia can be considered safe for use in patients with MNGIE disease.
线粒体神经胃肠型脑肌病(MNGIE)是一种罕见的常染色体隐性遗传病,由 TYMP 基因突变引起。本文报道了 1 例 18 岁男性 MNGIE 综合征患者,因不同疾病分别在不同场合接受两次区域麻醉下的手术治疗。患者以尿失禁和腹痛为主要表现。予椎管内麻醉下行膀胱镜检查。出院后 3 个月,诊断为胃穿孔,拟行椎管内麻醉下手术修补。手术及围术期均顺利。基于此经验,我们认为区域麻醉可安全用于 MNGIE 疾病患者。