TITLE:
Ureterolithotripsy under Spinal Anesthesia in Pompe Disease (Glycogen Storage Disease Type 2): A Case Report
AUTHORS:
Arzu Acar, Mustafa Nuri Denız, Elvan Erhan, Gulden Ugur
KEYWORDS:
Pompe Disease; Spinal Anesthesia; Ureterorenoscopie
JOURNAL NAME:
Open Journal of Anesthesiology,
Vol.3 No.2,
March
6,
2013
ABSTRACT: 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.