Ponde Vrushali, Desai Ankit, Ekambaram Karthic, Thakur Selabh
Children's Anesthesia Services and Department of Anesthesiology, Surya Children Hospital, Mumbai, Maharashtra, India.
Department of Pediatric and Preventive Dentistry, Surya Children Hospital, Mumbai, Maharashtra, India.
Indian J Anaesth. 2020 Apr;64(4):325-327. doi: 10.4103/ija.IJA_812_19. Epub 2020 Mar 28.
Rett syndrome is a genetic neurodevelopmental disorder which occurs in females and includes autism, spasticity, hypotonia, microcephaly, scoliosis, stereotyping, abnormal respiratory control and seizures. They are at an increased predisposition for QT interval prolongation and cardiac arrhythmias. An 8-year-old severely intellectually impaired girl with Rett syndrome was referred to us for anaesthesia for multiple dental abscess drainage and rehabilitation. Her frequency of convulsions had increased and she convulsed every time an attempt was made to feed her. The pain of chewing exacerbated the convulsions. The cornerstone of our management was to provide adequate pain relief, anaesthesia without muscle relaxant and opioids, and judicious use of local anaesthetics. We discuss the anaesthetic management and its advantages and limitations in this case report.
雷特综合征是一种发生于女性的遗传性神经发育障碍,其症状包括自闭症、痉挛、肌张力减退、小头畸形、脊柱侧弯、刻板动作、呼吸控制异常和癫痫发作。她们发生QT间期延长和心律失常的易感性增加。一名患有雷特综合征的8岁重度智力障碍女孩因多处牙脓肿引流及康复治疗被转诊至我院接受麻醉。她的抽搐频率增加,每次试图喂她时都会抽搐。咀嚼疼痛加剧了抽搐。我们治疗的关键是提供充分的疼痛缓解、不使用肌肉松弛剂和阿片类药物的麻醉以及谨慎使用局部麻醉剂。我们在本病例报告中讨论麻醉管理及其优缺点。