Perioperative management of maxillary cyst and genetic analysis in a patient with glycogen storage disease type 1a: A rare case report

Glycogen storage disease type 1a (GSD1a) is an autosomal recessive genetic disease with symptoms such as hypoglycemia, lactic acidosis, and bleeding tendency.A 15‐year‐old male patient underwent cystectomy under general anesthesia. He had GSD1a. Despite perioperative blood glucose control, lactic acidosis worsened. No abnormal bleeding or infections were observed. A homozygous mutation of c.648G>T was detected by genetic testing for glucose‐6‐phosphatase (G6PC).Perioperative complications specific to this disease should be managed.