Kaur Manbir, Sharma Umadatt, Solanki Rajendra K
Department of Anaesthesia, Dr. Sampurnanand Medical College (Mahatma Gandhi Hospital), Jodhpur, Rajasthan, India.
Saudi J Anaesth. 2020 Oct-Dec;14(4):524-527. doi: 10.4103/sja.SJA_809_19. Epub 2020 Sep 24.
Segawa's syndrome, dopamine-responsive dystonia, is a rare genetic disorder that typically begins in childhood by around 4-6 years of age. It is characterized by abnormal gait and dystonia. A 33-year-old man presented for autologous skin grafting of a nonhealing wound under general anesthesia. Successful anesthetic management of patients with this rare disease, though analogous in many ways to that of patients with Parkinson's disease, still pose significant challenges. We present anesthetic nuances to be considered in the management of a patient with Segawa's disease along with a pertinent review of the literature.
泽川综合征,即多巴胺反应性肌张力障碍,是一种罕见的遗传性疾病,通常在儿童期4至6岁左右发病。其特征为异常步态和肌张力障碍。一名33岁男性在全身麻醉下接受自体皮肤移植以治疗不愈合伤口。对患有这种罕见疾病的患者进行成功的麻醉管理,尽管在许多方面与帕金森病患者的麻醉管理类似,但仍面临重大挑战。我们介绍了在泽川病患者管理中需考虑的麻醉细微差别,并对相关文献进行了综述。