Hasan M Shahnaz, Leong Kok Weng, Chan Chris Yin Wei, Kwan Mun Keong
1 Department of Anesthesiology, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.
2 Department of Orthopaedic Surgery, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.
J Orthop Surg (Hong Kong). 2017 Jan;25(1):2309499016684743. doi: 10.1177/2309499016684743.
Segawa's syndrome or dopa-responsive dystonia is a rare hereditary disorder characterized by progressive dystonia of childhood onset, diurnal fluctuation of symptoms and complete or near complete alleviation of symptoms with administration of low-dose oral levodopa. From our literature search in PubMed, we found only three related publications: two on anesthesia for cesarean section and one on anesthesia for electroconvulsive therapy. We report our experience in providing anesthesia for corrective scoliosis surgery in two biological sisters with Segawa's syndrome. A review of the literature is also included.
泽川综合征或多巴反应性肌张力障碍是一种罕见的遗传性疾病,其特征为儿童期起病的进行性肌张力障碍、症状的日波动以及口服小剂量左旋多巴后症状完全或近乎完全缓解。通过在PubMed上检索文献,我们仅发现三篇相关出版物:两篇关于剖宫产麻醉,一篇关于电休克治疗麻醉。我们报告了为两名患有泽川综合征的亲姐妹进行脊柱侧弯矫正手术麻醉的经验。本文还包括文献综述。