Mitochondrial Disease and Anesthesia

Abstract

It is increasingly common for children with mitochondrial disease to undergo surgery and anesthesia. Although many different anesthetics have been used successfully for these patients, serious, unexpected complications have occurred during and following anesthetic exposure. This has led to the widespread opinion among anesthesiologists that mitochondrial patients are at increased risk from the stress of surgery and anesthesia. Defects in function of the mitochondrial electron transport chain can lead to striking hypersensitivity to volatile anesthetics in children. Despite this striking finding, the connection between mitochondrial function and response to anesthetics is unknown. We review here the anesthetic considerations for patients with mitochondrial defects. In addition, we present an approach to anesthetic care of these patients at our institutions.

The Role of Inflammation after Surgery for Elders (RISE) study: Examination of [11C]PBR28 binding and exploration of its link to post-operative delirium

“…In neonatal rats, exposures to sevoflurane (an inhalational anesthetic commonly used in humans) have been shown to decrease mitochondrial density in hippocampal axon terminals by 20% ( Amrock et al, 2015 ). In vitro studies have also confirmed that a number of common general and ancillary anesthetic agents inhibit mitochondrial function ( Hsieh et al, 2017 ). Given that TSPO is a transmembrane protein located in the outer mitochondrial membrane ( Papadopoulos et al, 2006 ), decreased [ 11 C]PBR28 binding following surgery might reflect reduced mitochondrial activity.…”

Multidisciplinary Perioperative Care for Children with Neuromuscular Disorders

“…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.…”

“…The utilization and avoidance of certain anesthetic agents for specific conditions is described below, but there are several intraoperative considerations common to all patients with NMDs. Succinylcholine should be avoided in patients with hypotonia or neuromuscular disease due to concerns for risk of MH, rhabdomyolysis, and hyperkalemia from upregulation of acetylcholine receptors [ 28 ]. Reports on the response of patients with mitochondrial disease to nondepolarizing muscle relaxants vary [ 33 ].…”

ADVERSE REACTION TO ANESTHESIA IN A M.8993T>C CARRIER WITH LEIGH SYNDROME

“…The effects of using anesthesia in patients with mitochondrial disease have been well reviewed in the literature since almost all anesthetics impair the mitochondrial function 1 , 2 , 3 . The difficulty in this clinical case was that the differential diagnosis did not consider mitochondrial disease as a hypothesis.…”

“…Os efeitos da utilização de anestesia em pacientes com doença mitocondrial estão bem revistos na literatura, praticamente todos os anestésicos enfraquecem a função mitocondrial 1 , 2 , 3 . A dificuldade neste caso clínico foi que no diagnóstico diferencial não foi considerada a hipótese de doença mitocondrial.…”