Ureterolithotripsy under Spinal Anesthesia in Pompe Disease (Glycogen Storage Disease Type 2): A Case Report

作     者：Arzu Acar Mustafa Nuri Deniz Elvan Erhan Gulden Ugur 

作者机构：Department of Anaesthesiology and ReanimationSchool of MedicineEge UniversityIzmirTurkey. 

出 版 物：《Open Journal of Anesthesiology》 (麻醉学期刊（英文）)

年 卷 期：2013年第3卷第2期

页      面：61-63页

学科分类：1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学] 

主　　题：Pompe Disease Spinal Anesthesia Ureterorenoscopie 

摘      要：Pompe disease is an autosomal recessive inherited glycogen storage disease, usually respiratory muscles, cardiac muscle and skeletal muscles are affected in childhood and mortality is inevitable but has a slower progression in the adult. The patient was a 35-year-old male patient who was scheduled to undergo ureterorenoscopy due to right ureteral stone. He was diagnosed with pompe disease after detection of myopathy on EMG due to muscle weakness in the legs and hips. He was started on myozyme vials once every 15 days. Spinal anesthesia with 3 ml 0.5% hyperbaric bupivacaine was performed and adequate sensorial and motor block was achieves at T10 level. The surgery was completed with no complications and he was discharged two days later. The selection of appropriate anesthetic technique and a careful perioperative and postoperative monitoring are of utmost importance due to progressive muscle weakness, hypotonia, respiratory failure and cardiomyopathy in pompe disease.