NM-342
Managing Conflicting Anesthetic Goals in a Child with Mitochondrial Disease Undergoing Scoliosis Surgery
Goldman M, Hsieh V, Grigg E
Seattle Children's Hospital, Seattle, Washington, USA
Spinal fusion in a patient with mitochondrial disease presents difficult anesthetic considerations. Competing interests with respect to the patient’s underlying metabolic disease and the need for an anesthetic tailored for neuromonitoring must be balanced. Here we present our approach to management of a 12 year-old girl with scoliosis and mitochondrial disease. The patient had a mitochondrial complex IV deficiency and presented for a T2-pelvis posterior spinal fusion with the aid of motor and somatosensory evoked potentials. This case highlights the competing anesthetic concerns for mitochondrial patients undergoing prolonged spine surgery.
Mitochondrial disease is not one discrete entity, but instead a grouping of diseases caused by defects in different mitochondrial enzymes. However, there are some generalizations regarding anesthesia in patients with mitochondrial disease. Organ systems with high metabolic demands are most affected by mitochondrial disease [1]. Nearly all anesthetic agents affect mitochondrial metabolism, though to varying degrees. Propofol infusions are generally avoided due to susceptibility in mitochondrial patients for development of propofol infusion syndrome [1,2]. Volatile agents inhibit complex I, and mitochondrial patients tend to have increased sensitivity to these agents [3].
Our anesthetic of choice for this surgery in an otherwise healthy patient is a total intravenous anesthetic with propofol and remifentanil infusions, occasionally supplemented with a low-dose ketamine infusion. However, in children with mitochondrial disease, continuous propofol infusions are avoided for the reasons stated previously. Volatile anesthetics and dexmedetomidine [4], on the other hand, may interfere with neuromonitoring, which is essential for reducing the incidence of postoperative neurologic complications.
For management of this patient, we chose an inhalational induction, followed by transition to a balanced anesthetic with 0.5 MAC desflurane supplemented with remifentanil and ketamine infusions. A maintenance D5NS infusion was also used. Monitors included standard ASA monitors, an arterial line, and a BIS monitor. Initial dissection proceeded smoothly. BIS remained static at approximately 60 regardless of end-tidal desflurane concentration, and EEG monitoring by the neurophysiology team was used as an adjunct to assess anesthetic depth given questionable reliability of BIS in this patient. However, during the correction phase, MEPs were noted to be decreased. A phenylephrine infusion was started to augment spinal cord perfusion. Point-of-care blood gasses were done hourly to closely monitor metabolism. No significant changes from baseline were noted on ABGs aside from acute anemia to a hematocrit of 23 and mild hypocalcemia, both of which were corrected. The patient had an uneventful emergence from anesthesia and was extubated on arrival to the pediatric intensive care unit immediately following surgery. She was transferred out of the PICU on post-operative day 1, and discharged to home on post-operative day 5.
References:
[1] J Inborn Errors Metab Screen. 2017;5:1-5
[2] Lancet. 2001; 357(9256):606-607
[3] J Biol Chem. 2001; 276(23):20551-20558
[4] Paediatr Anaesth. 2008;18(11):1082-8
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