Anesthetic Considerations in Von Gierke Disease (GSD Type I)

Preoperative Glucose Management

Minimizing preoperative fasting based on the patient's individual dietary history is crucial; if patients cannot tolerate 4-6 hours of fasting without hypoglycemia, intravenous glucose should be initiated 1, 2.
Scheduling surgery early in the day to minimize fasting time is ideal, preferably as the first case of the day 1.
Capillary glucose monitoring before and every hour during surgery is necessary; any unexplained distress should be considered hypoglycemia until proven otherwise 3.

Initiating a 10% dextrose infusion at a sufficient rate and concentration to maintain blood glucose ≥70 mg/dL from the start of fasting is recommended 1, 4, 5.
The glucose target should be 70-180 mg/dL during the entire perioperative period; infusion should only be stopped if glucose exceeds 300 mg/dL 1, 3.
Continuing glucose infusion until the patient can resume oral feeding and maintain normoglycemia independently is necessary 4, 5.

Preoperative evaluation of liver enzymes and synthetic function is essential, as some patients develop progressive cirrhosis; hepatotoxic anesthetic agents should be avoided in cirrhotic patients 4, 5.
Considering the impact of hepatomegaly on diaphragmatic excursion, which can affect ventilation and anesthetic management, is crucial 4, 5.
Evaluating the presence of hepatic adenomas through imaging studies is necessary, as they are present in approximately 50% of patients and can complicate surgical management 1, 2.

Preoperative coagulation function evaluation is necessary due to platelet dysfunction and von Willebrand defect associated with GSD I 2.
Anticipating a bleeding tendency, manifested as recurrent epistaxis, easy bleeding after dental or surgical procedures, and menorrhagia in women, is essential 2.

Avoiding anesthetic agents with known negative hepatic effects in patients with cirrhosis is recommended 4, 5.
Both volatile and total intravenous anesthesia are reasonable, as there is no evidence that a specific anesthetic agent is associated with better outcomes in patients with metabolic disorders 6, 3.

Monitoring capillary glucose every hour during the procedure, especially in prolonged surgeries, is necessary 3.
Maintaining blood glucose ≥70 mg/dL at all times; levels <60 mg/dL require immediate treatment with rapid-acting glucose 1, 7.
Treating hypoglycemia with glucose polymers or tablets/gels for diabetics, followed by cornstarch or feeding to maintain normal levels, is recommended 7.

Continuing glucose infusion until the patient is conscious, alert, and can resume oral feeding with the ability to maintain normoglycemia is necessary 1, 4, 5.
Frequently monitoring glucose in the immediate postoperative period until a stable feeding pattern is established is essential 1, 7.
Resuming oral feeding as soon as possible with small, frequent meals; avoiding prolonged fasting >3-4 hours in children and >5-6 hours in adults is recommended 1.
Restarting raw cornstarch according to the patient's usual regimen once they can tolerate oral feeding is necessary 1, 7.

Considering the risk of acute pancreatitis in patients with severe persistent hypertriglyceridemia (>1,000 mg/dL) is essential 2.
All patients should wear a medical alert bracelet, as rapid and appropriate treatment is critical 7.
Coordinating with the metabolic team before elective surgeries to optimize metabolic control and plan perioperative management is recommended 1.