Open AccessAnesthetic Management for an Adult With Glycogen Storage Disease Type 0
Author(s) -
Airi Sakamizu,
Erika Yaguchi,
Shinsuke Hamaguchi
Publication year - 2020
Publication title -
anesthesia progress
Language(s) - English
Resource type - Journals
eISSN - 1878-7177
pISSN - 0003-3006
DOI - 10.2344/anpr-67-02-06
Subject(s) - medicine , remifentanil , anesthesia , palatoplasty , velopharyngeal insufficiency , propofol , glycogen storage disease , anesthetic , surgery , glycogen
A 20-year-old woman with glycogen storage disease type 0 (GSD-0) underwent velopharyngeal closure for velopharyngeal insufficiency following palatoplasty. To reduce the risk of complications attributed to GSD-0, general anesthesia was administered using a total intravenous anesthesia (TIVA) technique with propofol and remifentanil, along with supplemental glucose-containing intravenous fluids. Her blood glucose remained stable, intraoperative body temperature ranged from 36.5 to 37.2°C, and the velopharyngeal closure was completed without any adverse events.