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

Abstract

AbstractBackgroundGlycogen storage disease type 1a (GSD1a) is an autosomal recessive genetic disease with symptoms such as hypoglycemia, lactic acidosis, and bleeding tendency.Case PresentationA 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).ConclusionPerioperative complications specific to this disease should be managed.