Leigh Sendromlu Pediyatrik Olguda Anestezi Yönetimi
Özet
Leigh sendromu (LS), mitokondriyal enerji üretimindeki bozukluklar sonucu gelişen, subakut nekrotizan ensefalomyelopati ile seyreden ilerleyici ve nadir bir nörodejeneratif hastalıktır. Genellikle bebeklik döneminde gelişimsel gerilik, nöbetler ve laktik asidoz atakları ile kendini gösterir. Makale, laparoskopik cerrahi uygulanan 27 aylık bir LS olgusunun perioperatif anestezi yönetimini detaylandırmaktadır. LS hastaları, cerrahi stres ve anestezik ajanlara karşı aşırı duyarlılık nedeniyle metabolik dekompansasyona ve solunum yetmezliğine yatkındır. Vakada, mitokondriyal fonksiyonları baskılayabilen volatil anestezikler ve propofolden kaçınılarak midazolam ve remifentanil infüzyonu ile toplam intravenöz anestezi (TIVA) uygulanmıştır. Kas gevşetici olarak roküronyum tercih edilmiş, blokajın geri döndürülmesinde ise antikolinesterazların olumsuz etkilerinden kaçınmak amacıyla sugammadeks kullanılmıştır. İntraoperatif süreçte laktat içeren sıvılardan sakınılmış, dekstrozlu solüsyonlar ve bikarbonat desteği ile metabolik denge korunmuştur. Sonuç olarak, LS'li hastalarda preoperatif hazırlık, normoterminin sağlanması, uygun ajan seçimi ve yakın metabolik monitörizasyon başarılı bir anestezi yönetimi için kritiktir.
Leigh syndrome (LS) is a rare, progressive neurodegenerative disorder characterized by subacute necrotizing encephalomyelopathy caused by defects in mitochondrial energy production. It typically presents in infancy with developmental delay, seizures, and lactic acidosis attacks. This article details the perioperative anesthetic management of a 27-month-old pediatric patient with LS undergoing laparoscopic surgery. Patients with LS are predisposed to metabolic decompensation and respiratory failure due to surgical stress and hypersensitivity to anesthetic agents. In this case, total intravenous anesthesia (TIVA) with midazolam and remifentanil infusion was administered, avoiding volatile anesthetics and propofol, which can suppress mitochondrial functions. Rocuronium was preferred as the neuromuscular blocker, and sugammadex was used to reverse the blockade to avoid the adverse effects of anticholinesterases. Lactate-containing fluids were avoided intraoperatively, and metabolic balance was maintained with dextrose solutions and bicarbonate support. Consequently, preoperative preparation, maintaining normothermia, appropriate agent selection, and close metabolic monitoring are critical for successful anesthetic management in patients with LS.
Referanslar
Baertling F, Rodenburg RJ, Schaper J, et al. A guide to diagnosis and treatment of Leigh syndrome. Journal of Neurology, Neurosurgery & Psychiatry 2014;85: 257-265. doi: 10.1136/jnnp-2012-304426
Lee HF, Tsai CR, Chi CS, et al. Leigh syndrome: clinical and neuroimaging follow-up. Pediatric Neurology 2009;40(2):88-93. doi: 10.1016/j.pediatrneurol.2008.09.020.
Thorburn DR, Rahman J, Rahman S. Mitochondrial DNA-Associated Leigh Syndrome and NARP. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1173/ (Accessed 15th Feb 2022).
Finsterer J. Leigh and Leigh-like syndrome in children and adults. Pediatric Neurology 2008;39(4):223-235. doi: 10.1016/j.pediatrneurol.2008.07.013.
Ng YS, Martikainen MH, Gorman GS, et al. Pathogenic variants in MT-ATP6: A United Kingdom-based mitochondrial disease cohort study. Annals of Neurology 2019;86(2):310-315. doi: 10.1002/ana.25525.
Lake NJ, Compton AG, Rahman S, et al. Leigh syndrome: One disorder, more than 75 monogenic causes. Annals of Neurology 2016;9(2):190-203. doi: 10.1002/ana.24551.
Rahman S, Blok RB, Dahl HH, et al. Leigh syndrome: clinical features and biochemical and DNA abnormalities. Annals of Neurology 1996;39(3):343-51. doi: 10.1002/ana.410390311.
Staley KJ, Sims KB, Grant PE, et al. Case records of the Massachusetts General Hospital. Case 28-2008. An 8-day-old infant with congenital deafness, lethargy, and hypothermia. The New England Journal of Medicine 2008;359(11):1156-1167. doi: 10.1056/NEJMcpc0804642.
O’Ferral E. Mitochondrial myopathies: Clinical features and diagnosis. Available from: https://www.uptodate.com/contents/mitochondrial-myopathies-clinical-features-and-diagnosis (Accessed 01.02.2022)
Lisle C, Gregory, A. Effects of Dexmedetomidine Administration on Drosophila melanogaster with Leigh Syndrome. 2020. PhD Thesis. Webster University.
Driessen J, Willems S, Dercksen S, et al. Anesthesia-related morbidity and mortality after surgery for muscle biopsy in children with mitochondrial defects. Paediatric Anaesthesia 2007;17: 16–21. doi: 10.1111/j.1460-9592.2006.02043.x.
Footitt EJ, Sinha MD, Raiman JA, et al. Mitochondrial disorders and general anesthesia: a case series and review. British Journal of Anaesthesia. 2008;100: 436–441. doi: 10.1093/bja/aen014.
Casta A, Quackenbush EJ, Houck CS, et al. Perioperative white matter degeneration and death in a patient with a defect in mitochondrial oxidative phosphorylation. Anesthesiology 1997;87: 420–425. doi: 10.1097/00000542-199708000-00030.
Cooper MA, Fox R. Anesthesia for corrective spinal surgery in a patient with Leigh’s disease. Anesthesia & Analgesia 2003;97: 1539–1541. doi: 10.1213/01.ANE.0000081787.94275.DE.
Grattan-Smith PJ, Shield LK, Hopkins IJ, et al. Acute respiratory failure precipitated by general anesthesia in Leigh’s syndrome. Journal of Child Neurology 1990;5: 137–141. doi: 10.1177/088307389000500214.
Cohen PJ. Effect of anesthetics on mitochondrial function. Anesthesiology 1973;39: 153–164. doi: 10.1097/00000542-197308000-00007.
Harris RA, Munroe J, Farmer B, et al. Action of halothane upon mitochondrial respiration. Archives of Biochemistry and Biophysics 1971;142: 435–444. doi: 10.1016/0003-9861(71)90507-8.
Nahrwold ML, Cohen PJ. Additive effect of nitrous oxide and halothane on mitochondrial function. Anesthesiology. 1973;39: 534–536. doi: 10.1097/00000542-197311000-00014.
Miró O, Barrientos A, Alonso JR, et al. Effects of general anesthetic procedures on mitochondrial function of human skeletal muscle. Eur J Clin Pharmacol 1999;55: 35–41. doi: 10.1007/s002280050589.
Nahrwold ML, Cohen PJ. The effects of forane and fluroxene on mitochondrial respiration: correlation with lipid solubility and in-vivo potency. Anesthesiology 1973;38: 437–444. doi: 10.1097/00000542-197305000-00005.
Devin A, Nogueira V, Avéret N, et al. Profound effects of the general anesthetic etomidate on oxidative phosphorylation without effects on their yield. The Journal of Bioenergetics and Biomembranes 2006;38: 137–142. doi: 10.1007/s10863-006-9013-6.
Markham A, Cameron I, White SJ. The effect of ketamine hydrochloride, a non-barbiturate parenteral anaesthetic on oxidative phosphorylation in rat liver mitochondria. Biochemical Pharmacology 1981;30: 2165–2168. doi: 10.1016/0006-2952(81)90238-0.
Driessen JJ. Neuromuscular and mitochondrial disorders: what is relevant to the anaestheslologist? Current Opinion in Anesthesiology 2008;21(3): 350-355. doi: 10.1097/ACO.0b013e3282f82bcc.
Finsterer J, Haberler G, Schmiedel J. Deterioration of Keams-Sayre syndrome following articaine administration for local anesthesia. Clinical Neuropharmacology 2005;28(3): 148-149. doi: 10.1097/01.wnf.0000165352.10841.21.
Russell SH, Hirsch NP. Anaesthesia and myotonia. British Journal of Anaesthesia. 1994;72(2): 210-216.
Cook DR. Can succinylcholine be abandoned? Anesthesia & Analgesia 2000;90(5): 24-28. doi: 10.1097/00000539-200005001-00006
Flick RP, Gleich SJ, Herr MMH, et al. The risk of malignant hyperthermia in children undergoing muscle biopsy for suspected neuromuscular disorder. Paediatric Anaesthesia 2007;17(l): 22-27. doi: 10.1111/j.1460-9592.2006.02105.x
Mattman A, Sirrs S, Mezei MM, et al. Mitochondrial disease clinical manifestations: an over- view. British Columbia Medical Journal 2011;53(4): 183-187.
Gegenava GP, Chistiakov VV. Effect of morphine in vitro on the oxidative phosphorylation in rat liver mitochondria. Bulletin of Experimental Biology and Medicine 1975;80:77–79.
Moussa TA, el-Beih ZM. Effects of morphine on the Golgi apparatus and mitochondria of mammalian neurones. Cellule 1972;69: 191–203.
Nouette-Gaulain K, Dadure G, Morau D, et al. Age-dependent bupivacaine-induced muscle toxicity during continuous peripheral nerve block m rats. Anesthesiology 2009;111(5): 1120-1127. doi: 10.1097/ALN.0b013e3181bbc949.
Nouette-Gaulain K, Bringuier S, Ganal-Raffin M, et al. Time course of mitochondrial metabolism alterations to repeated injections of bupivacaine in rat muscle. The Canadian Journal of Anesthesia 2010;57(9): 835-842. doi: 10.1007/s12630-010-9347-8.
Driessen J, Smeitink J. Author's reply to anesthesia care for muscle biopsy in children with myopathies by Dr Ferrari Fabio. Paediatric Anaesthesia 2009;19(l): 54-72.
Schnabel RM, Marcus MAE, Theunissen HMS, et al. Anesthetic management for a child with mitochondrial complex II deficiency, Paediatric Anaesthesia 2008;18(8): 802-803. doi: 10.1111/j.1460-9592.2008.02552.x.
Nomura F, Hatano H, Ohnishi K, et al. Effects of anticonvulsant agents on halothane‐induced liver injury in human subjects and experimental animals. Hepatology 1986;6(5):952-956. doi: 10.1002/hep.1840060523.
Parikh S, Goldstein A, Koenig MK, et al. Diagnosis and management of mitochondrial disease: a consensus statement from the Mitochondrial Medicine Society. Genetics in Medicine 2015;17(9): 689-701. doi: 10.1038/gim.2014.177.
Nouette-Gaulain K, Jose G, Gapdevila X, et al. From analgesia to myopathy: when local anesthetics impair the mitochondrion, Biochemistry and Cell Biology 2011;43(l): 14-19. doi: 10.1016/j.biocel.2010.10.005.
