Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke (MELAS) syndrome is a progressive multisystemic neurodegenerative disorder. MELAS syndrome impairs oxidative phosphorylation and predisposes patients to lactic acidosis, particularly under metabolic stress. We report 2 siblings with MELAS-associated idiopathic scoliosis who underwent posterior spinal instrumented fusion with measures taken to minimise anaesthetic and surgical stress, blood loss, and operating time.
PavlakisSG, PhillipsPC, DiMauroS, De VivoDC, RowlandLP. Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes: A Distinctive clinical syndrome. Ann Neurol1984;16:481–8.
2.
Morgan-HughesJA, SweeneyMG, CooperJM, HammansSR, BrockingtonM, SchapiraAH. Mitochondrial DNA (mtDNA) diseases: Correlation of genotype to phenotype. Biochim Biophys Acta1995;1271:135–40.
3.
CohenBH, GoldDR. Mitochondrial cytopathy in adults: What we know so far. Cleve Clin J Med2001;68:625–42.
4.
DobbsMB, LenkeLG, KimYJ, KamathG, PeelleMW, BridwellKH. Selective posterior thoracic fusions for adolescent idiopathic scoliosis: Comparison of hooks versus pedicle screws. Spine (Phila Pa 1976)2006;31:2400–4.
5.
KaratoprakO, UnayK, TezerM, OzturkC, AydoganM, MirzanliC. Comparative analysis of pedicle screw versus hybrid instrumentation in adolescent idiopathic scoliosis surgery. Int Orthop2008;32:523–9.
6.
KimYJ, LenkeLG, ChoSK, BridwellKH, SidesB, BlankeK. Comparative analysis of pedicle screw versus hook instrumentation in posterior spinal fusion of adolescent idiopathic scoliosis. Spine (Phila Pa 1976) 2004;29:2040–8.
7.
YilmazG, BorkhuuB, DhawaleAA, OtoM, LittletonAG, MasonDE. Comparative analysis of hook, hybrid, and pedicle screw instrumentation in the posterior treatment of adolescent idiopathic scoliosis. J Pediatr Orthop2012;32:490–9.
8.
CahillPJ, PahysJM, AsgharJ, YaszayB, MarksMC, BastromTP. The effect of surgeon experience on outcomes of surgery for adolescent idiopathic scoliosis. J Bone Joint Surg Am2014;96:1333–9.
9.
HeffernanMJ, SeehausenDA, AndrasLM, SkaggsDL. Comparison of outcomes after posterior spinal fusion for adolescent idiopathic and neuromuscular scoliosis: Does the surgical first assistant's level of training matter?Spine (Phila Pa 1976)2014;39:648–55.
10.
IalentiMN, LonnerBS, VermaK, DeanL, ValdevitA, ErricoT. Predicting operative blood loss during spinal fusion for adolescent idiopathic scoliosis. J Pediatr Orthop2013;33:372–6.
11.
AmesCP, BarryJJ, KeshavarziS, DedeO, WeberMH, DevirenV. Perioperative outcomes and complications of pedicle subtraction osteotomy in cases with single versus two attending surgeons. Spine Deform2013;1:51–8.
12.
HalanskiMA, ElfmanCM, CassidyJA, HassanNE, SundSA, NoonanKJ. Comparing results of posterior spine fusion in patients with AIS: Are two surgeons better than one?J Orthop2013;10:54–8.
13.
StevanatoR, MomoF, MarianM, RigobelloMP, BindoliA, BragadinM. Effects of nitrosopropofol on mitochondrial energy-converting system. Biochem Pharmacol2002;64:1133–8.
14.
GurrieriC, KivelaJE, BojanicK, GavrilovaRH, FlickRP, SprungJ. Anesthetic considerations in mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome: A Case series. Can J Anaesth2011;58:751–63.
ChongCT, ManninenP, SivanaserV, SubramanyamR, LuN, VenkatraghavanL. Direct comparison of the effect of desflurane and sevoflurane on intraoperative motor-evoked potentials monitoring. J Neurosurg Anesthesiol2014;26:306–12.
17.
SekimotoK, NishikawaK, IshizekiJ, KuboK, SaitoS, GotoF. The effects of volatile anesthetics on intraoperative monitoring of myogenic motor-evoked potentials to transcranial electrical stimulation and on partial neuromuscular blockade during propofol/fentanyl/nitrous oxide anesthesia in humans. J Neurosurg Anesthesiol2006;18:106–11.
18.
VaughaDJ, ThorntonC, WrightDR, FernandesJR, RobbinsP, DoreC. Effects of different concentrations of sevoflurane and desflurane on subcortical somatosensory evoked responses in anaesthetized, non-stimulated patients. Br J Anaesth2001;86:59–62.
19.
DindyalS, MistryK, AngamuthuN, SmithG, HiltonD, ArumugamP. MELAS syndrome presenting as an acute surgical abdomen. Ann R Coll Surg Engl2014;96:101E–103E.
20.
GentiliME, RaudC, EnelD, HenotM, BothereauH. Combination of general anaesthesia and postoperative epidural analgesia in mitochondrial myopathy. Ann Fr Anesth Reanim2013;32:e149.
21.
SeessleJ, StremmelW, EbingerF, MerleU. An unusual case of paralytic ileus. Z Gastroenterol2012;50:1161–5.
22.
FukuyamaK, IshikawaY, OginoT, InoueH, YamaokaR, HiroseT. Mucosal necrosis of the small intestine in myopathy, encephalopathy, lactic acidosis, and stroke-like episodes syndrome. World J Gastroenterol2012;18:5986–9.
23.
ImaiY, KitamuraT, KawamuraG, NakamuraM, YamadaY. Anesthetic management of emergency total gastrectomy in a patient with mitochondrial encephalomyopathy: A Case of gastric perforation accompanied by systemic inflammatory response syndrome [in Japanese]. Masui2010;59:765–9.
24.
ParkJS, BaekCW, KangH, ChaSM, ParkJW, JungYH. Total intravenous anesthesia with propofol and remifentanil in a patient with MELAS syndrome: A Case report. Korean J Anesthesiol2010;58:409–12.
25.
SasanoN, TamuraT, AzamiT, SasanoH. Severe hyponatremia occurring after surgical stress in a patient with mitochondrial disease. J Anesth2009;23:587–90.
26.
SinghB, LowPS, YeoJF. MELAS: A Case report. Ann Acad Med Singapore2004;33(4 Suppl):69–71.