Abstract / Summary
Abstract Background Glucose transporter type 1 deficiency syndrome (GLUT1DS) is a rare metabolic encephalopathy in which ketone bodies serve as an essential cerebral energy substrate. In the perioperative setting, conventional management strategies may inadvertently suppress ketogenesis and compromise cerebral energy metabolism. Case presentation We report the perioperative management of a woman in her 40s with adult-onset GLUT1DS undergoing hip osteotomy under total intravenous anesthesia (TIVA). The patient had been maintained on a well-controlled ketogenic diet prior to surgery. Anesthesia was induced and maintained using propofol, remifentanil, and rocuronium, while glucose-containing solutions were avoided. Lipid emulsion was administered during fasting as an alternative energy substrate to support ketogenesis. Depth of anesthesia was continuously monitored using the bispectral index (BIS), which remained within the normal range throughout the procedure. Perioperative laboratory findings demonstrated stable blood glucose levels without hypoglycemia or hyperglycemia, suppressed insulin levels, and elevated ketone bodies with preserved acid–base balance. The patient recovered uneventfully and resumed a ketogenic diet 4 hours after emergence without neurological complications. Conclusions This case highlights that perioperative management focused on preserving therapeutic ketosismay be feasible in adult-onset GLUT1DS. Careful metabolic and neurological monitoring, avoidance of unnecessary glucose administration, and predefined contingency plans are essential for safe anesthetic management.