A review of anaesthetic outcomes in patients with genetically confirmed mitochondrial disorders

Abstract

No relationship between choice of anaesthetic agent and subsequent perioperative complication was observed. It is likely that individual optimisation on a case-by-case basis is more important overall than choice of any one particular technique. What is Known: • General anaesthesia (GA) poses a potential risk of decompensation in children with mitochondrial disorders. • There is a great diversity in the anaesthetic approaches undertaken in this cohort, and little guidance exists for anaesthetists and other clinicians regarding the optimal anaesthetic agents and perioperative management to provide to patients with mitochondrial disease. What is New: • In this study of 26 patients with genetically confirmed mitochondrial disease who underwent 65 GAs, no relationship between choice of anaesthetic agent and subsequent perioperative complication was observed • It is likely that individual optimisation on a case-by-case basis is more important overall than choice of any one particular technique.

Endotracheal Intubation Using Alfentanil in a Pediatric Patient with a Mitochondrial Myopathy and Gastroparesis

“…Even short-term use of propofol in children with mitochondrial disorders has been associated with delayed recovery and the need for intensive care unit admission. [3][4][5]7 Patients with mitochondrial disorders are also at an increased risk of having coexisting undiagnosed myopathies that may have a higher incidence of hyperkalemia with depolarizing neuromuscular blockers such as succinylcholine. Our patient presented with muscle weakness and dysmetria, raising concerns for a myopathy.…”

“…2 Children with mitochondrial disorders are at an increased risk of metabolic decompensation that may be exacerbated by illness or decreased oral intake, leading to severe lactic acidosis resulting in metabolic encephalopathy. 1,[3][4][5] The use of glucose-containing solutions while avoiding lactate-containing solutions is necessary to prevent lactic acidosis in these patients by promoting aerobic metabolism. [3][4][5] Here, we describe the challenges and success in selecting an anesthetic induction drug for our patient given her underlying mitochondrial disorder.…”

Mitochondrial Disease: Advances in Clinical Diagnosis, Management, Therapeutic Development, and Preventative Strategies

“…Anesthesia is generally tolerated well in patients with mitochondrial disease. However, some patients, particularly those having complex I dysfunction, have been found to have volatile anesthetic sensitivity and subsequent adverse events such as respiratory depression, regression, and white matter changes (49). Current clinical guidelines support a cautious approach to using anesthesia in patients with mitochondrial disease.…”

Multidisciplinary Perioperative Care for Children with Neuromuscular Disorders

“…While a variety of anesthetic approaches have been used safely for patients with mitochondrial myopathies [ 17 , 18 ] and past concerns about an association with MH have been dismissed [ 19 ], every anesthetic agent studied has been shown to depress mitochondrial function [ 20 , 21 ]. Desflurane, isoflurane, and sevoflurane suppress cellular respiration at complex I, V, and coenzyme Q.…”

“…A large retrospective study of patients with NMDs undergoing muscle biopsies revealed no serious complications, and its authors concluded that “increased awareness of anesthesia-related concerns in an NMD patient directs management along specific safe pathways” [ 25 ]. While increased awareness is evident from multiple existing reviews of perioperative concerns for patients with NMDs [ 5 , 17 , 18 , 19 , 20 , 24 , 26 , 27 , 28 , 29 ], most have been directed toward anesthesia providers and very few define in precise and actionable ways the “specific safe pathways” that should be followed throughout the perioperative continuum of care. Indeed, utilization of standardized guidelines to assist in management of these patients remains low.…”