Combination of general anaesthesia and postoperative epidural analgesia in mitochondrial myopathy

Perioperative management of patients with genetic multisystem diseases associated with pre‑excitation

“…Regarding their cardiovascular responses, patients with MELAS syndrome generally exhibit good tolerance to common anaesthetics, neuromuscular blockers and their standard reversals (i.e. anticholinergic/neostigmine) [54,[58][59][60]. Due to possible susceptibility to malignant hyperthermia, caution is needed with potentially triggering agents, such as succinylcholine and volatiles [54,[61][62][63], although no complications have been reported in published cases where these agents were used [54,64,65].…”

“…Drug interactions, especially between anticonvulsants and general anaesthetics/ adjuvants, should also be taken into account [66]. Monitoring of the anaesthetic depth and neuromuscular transmission should be considered, since the patients may exhibit excessive sensitivity to sedatives and hypnotics [58,67], and also an unpredictable response to NMBs [67]. The latter ones should better be omitted, if possible [55,62].…”

“…Propofol, etomidate and thiopental were given for in-duction, while desflurane, isoflurane or halothane were used in the majority of cases for maintenance of anaesthesia, without complications [54]. Total intravenous anaesthesia with propofol -with or without remifentanil -seems advantageous in terms of minimizing the risk of MH [61], and is preferred by many authors [58][59][60][61][67][68][69][70]. Notably, although propofol infusion has been used uneventfully for a few hours [54,[58][59][60]71], caution is needed in case of prolonged administration -i.e.…”

“…Total intravenous anaesthesia with propofol -with or without remifentanil -seems advantageous in terms of minimizing the risk of MH [61], and is preferred by many authors [58][59][60][61][67][68][69][70]. Notably, although propofol infusion has been used uneventfully for a few hours [54,[58][59][60]71], caution is needed in case of prolonged administration -i.e. for sedation in intensive care units -because it may further decrease the mitochondrial ATP production and cause severe acidosis [72].…”

Dental Treatment of a Child with Mitochondrial Myopathy Under General Anesthesia

“…There are several reports in the literature of this type of disease tolerant to a wide range of anesthetics including volatile anesthetics, propofol and local anesthetics. 6,7 In 2013, Gentili et al published a letter to the editor in which they described a severe case of mitochondrial myoptahy (MELAS syndrome: mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) that was operated for combined colectomy and ovariectomy under laparotomy with an intra-operative time of 5 hours. 7 The operation was performed under general anesthesia in combination with postoperative epidural anesthesia.…”