Department of Anesthesiology and ICM, Faculty of Medicine, Ankara University, Mesa Koza sitesi, 2.blok no:15, GOP, 06700 Ankara, Turkey.
J Anesth. 2010 Dec;24(6):949-50. doi: 10.1007/s00540-010-1005-3. Epub 2010 Aug 14.
Prader-Willi syndrome (PWS) is a rare disorder of chromosome abnormalities in which the paternal genes in chromosome 15 are lacking. The clinical course is characterized by hypotonia, hyperphagia, and morbid obesity. Both general and regional anesthesia in these patients is difficult due to morbid obesity and hypotonia. We report our anesthetic management in a patient with PWS with a body mass index (BMI) of 29.43 kg/m² who underwent orchiopexy and hypospadias repair. The clinical course of the patient was uneventful during the procedure and postoperative period. However, arrangements with a pediatric intensive care setting for the postoperative period are recommended for patients with PWS undergoing surgery.
普拉德-威利综合征（PWS）是一种罕见的染色体异常疾病，其 15 号染色体上的父系基因缺失。临床病程的特征是肌张力低下、多食和病态肥胖。由于病态肥胖和肌张力低下，这些患者的全身和局部麻醉都很困难。我们报告了一例体重指数（BMI）为 29.43kg/m²的 PWS 患者的麻醉管理，该患者接受了睾丸固定术和尿道下裂修复术。手术过程中和术后患者的临床过程都很顺利。然而，对于接受手术的 PWS 患者，建议在术后期间与儿科重症监护病房进行安排。
