[Anesthetic management in a patient with trifunctional protein deficiency]

A 4-year-old girl with trifunctional protein deficiency was scheduled for gastrostomy. She had recurrent episodes of rhabdomyolysis triggered by fasting, infection, stress and uncertain causes. In the management of anesthesia, we avoided both propofol and inhalational anesthetics and anesthetized her with benzodiazepine and opioid combined with regional anesthesia to minimize the stress response. Her metabolism was kept stable throughout the intraoperative period. However, on the second postoperative day, she developed rhabdomyolysis and was treated with glucose containing infusion. This case provides instructive information that strict management is needed for patients with trifunctional protein deficiency in the perioperative period.