Novel Approach to Perioperative Management of a Child with Nonketotic Hyperglycinemia

Abstract

Nonketotic hyperglycinemia is a rare condition that requires a specialized approach. Perioperative management remains uncertain due to both the rarity of the disease and the limited literature available. A 7-year-old girl was admitted for a surgical dental procedure. She remained fasting for 6 hours without receiving any intravenous dextrose-containing fluids. Oral dexmedetomidine at a dose of 4 mcg/kg was selected for premedication. Sevoflurane was used as the sole agent for both induction and maintenance of anesthesia. The patient awakened 20 minutes after the discontinuation of sevoflurane. In this case, we emphasize that the use of dexmedetomidine for premedication may reduce preoperative anxiety and the potential for postoperative delirium. Furthermore, preoperative glycine concentrations are likely proportional to the duration of awakening. To avoid prolonged emergence, the use of Bispectral Index monitoring is recommended. Additionally, it has been shown that preoperative fasting without intravenous fluid supplementation can be safe.