Anesthesia management in a patient with very long-chain acyl-Coenzyme A dehydrogenase deficiency

Abstract

Background In a patient with very long-chain acyl-Coenzyme A dehydrogenase (VLCAD) deficiency, metabolism of fatty acids is impaired and a supply of alternative energy is limited when glucose level is insufficient on starvation. Case presentation A 37-year-old woman with VLCAD deficiency was diagnosed with an ovarian cyst and was scheduled for laparoscopic ovarian cystectomy. Glucose was administered intravenously with the start of fasting. Anesthesia was induced with remifentanil, midazolam, and thiamylal, maintained with desflurane and remifentanil. Body temperature was maintained at 36.2–36.7 °C. During anesthesia, hypoglycemia did not occur, creatine kinase levels were in the normal range, and myoglobinuria was not detected. No shivering was observed after extubation. Conclusions Glucose was administered to avoid perioperative hypoglycemia. Body temperature was controlled to avoid shivering, which would otherwise increase skeletal muscle energy needs. Blood creatine kinase did not increase, and myoglobinuria was not detected; thus, rhabdomyolysis was unlikely to develop.

Anesthetic Management and Neuromonitoring in a Patient with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency Undergoing Scoliosis Surgery: A Case Report and Review of Literature

“…Volatile anesthetics could not be used for the majority of the case due to its inhibitory effect on the anterior horn cells of the spinal cord that preclude neuromonitoring. Despite previous conflicting recommendations due to concerns for rhabdomyolysis with volatile anesthetic use in VLCADD patients [ 12 ], recent literature reports and the review by Redshaw and Stewart have demonstrated that inhaled anesthetics are safe to use in VLCADD patients and were safely used at the end of our case as well [ 4 , 5 , 13 , 14 ]. The few existing reports on managing patients with VLCADD recommend strict intraoperative monitoring of glucose and serum CK levels along with continuous glucose infusions to prevent hypoglycemia [ 12 , 14 ].…”

“…VLCADD is a rare disorder with an estimated incidence of 1 : 30,000–400,000 live births [ 3 ]. Due to deficient or defective VLCAD enzyme, these patients are unable to break down fatty acids to utilize as a secondary energy source when glucose levels are low, and thus are prone to hypoglycemia [ 4 , 5 ]. The clinical severity of symptoms varies greatly among patients: some present with multisystemic disease and organ failure, while others only exhibit mild clinical symptoms with vigorous exercise or illness [ 2 ].…”

“…Because pain activates a sympathetic response in the body, it was necessary to provide adequate analgesia with a multimodal approach without obtunding the patient's sensory responses needed for neuromonitoring [ 4 ]. Hypothermia was avoided since involuntary muscle movements generate heat and increase skeletal muscle energy demand [ 5 ]. Furthermore, the patient's prone positioning during the case and the nature of higher intraoperative blood loss in scoliosis cases required consideration of the risks and benefits of permissive hypotension to decrease blood loss.…”