A 20-year-old woman with adult glycogen storage disease (GSD) type 0, a congenital glycogen synthase deficiency, underwent a velopharyngeal closure for velopharyngeal insufficiency following a palatoplasty. The patient had been diagnosed as having GSD type 0a because of hypoglycemia and a short stature at the age of one year. When she underwent a palatoplasty at the age of 3 years, however, she was suspected of having GSD type 0b because she also exhibited intraoperative metabolic acidosis, hypoglycemia, a high body temperature, and an elevation in postoperative creatine phosphokinase levels during surgery. When she underwent closure of a palate fistula at the age of 12 years, no such symptoms were observed during total intravenous anesthesia (TIVA) using propofol. For the presently reported procedure, therefore, we planned to use TIVA with propofol and remifentanil and without a muscle relaxant.
A solution containing 2.5% dextrose and 1% dextrose was infused during anesthesia. Her intraoperative body temperature ranged from 36.5°C to 37.2°C, and the velopharyngeal closure was completed without any adverse events.
GSD type 0 can potentiate the occurrence of several problems, such as hypoglycemia, muscle weakness, myoglobinuria, and myocardial damage. In the present case, therefore, we infused a solution containing glucose to avoid hypoglycemia and performed TIVA without a muscle relaxant to avoid serial complications caused by GSD type 0.
