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Erschienen in: Journal of Anesthesia 2/2013

01.04.2013 | Original Article

Anesthesia for children with mitochondrial disorders: a national survey and review

verfasst von: Muhammad B. Rafique, Staci D. Cameron, Qaiser Khan, Suur Biliciler, Salman Zubair

Erschienen in: Journal of Anesthesia | Ausgabe 2/2013

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Abstract

Purpose

Mitochondrial diseases are a heterogeneous group of disorders. Patients with such diseases often need general anesthesia for diagnostic procedures and surgery; guidelines are lacking for the anesthetic care of these patients.

Methods

We conducted a survey to investigate the current practices of pediatric anesthesiologists in the US in order to determine and document current practice. The survey consisted of twenty questions, including two demographic questions. A link to the survey was sent via email to members of the Society for Pediatric Anesthesia (2440), and was available online for 14 weeks.

Results

Only 503 completed the survey: a response rate of 20.61 %. Among the responders, 93.2 % had children with mitochondrial disorders among their patients, but only 11 % had institutional guidelines for such cases in place. Among the responders, 80.3 % used the standard nil per os (NPO) status guidelines, while the rest give intravenous dextrose solution once NPO was in effect. Only 18.3 % took precautions for malignant hyperthermia during treatment. The majority of the practitioners chose sevoflurane as the safest inhaled agent for induction and maintenance (89.7 and 78.5 %, respectively). Regional anesthesia was deemed safe by 97.3 % of the responders. Lactated Ringer’s solution was considered safe for these children by 49 %; only 47.8 % used dextrose-containing fluids for fluid replacement. The blood glucose was monitored by 72.7 %, and the majority (85 %) of this monitoring was done in a postanesthesia care unit.

Conclusion

Although the response rate was low, the majority of the responders provide care to these children routinely, so it can be inferred that the results of this survey are the closest published results to the true trend.
Literatur
1.
Zurück zum Zitat Blm M. Current management in child neurology. 4th ed. Shelton: People’s Medical Publishing House; 2008. Blm M. Current management in child neurology. 4th ed. Shelton: People’s Medical Publishing House; 2008.
2.
Zurück zum Zitat Thorburn DR, Smeitink J. Diagnosis of mitochondrial disorders: clinical and biochemical approach. J Inherit Metab Dis. 2001;24:312–6.PubMedCrossRef Thorburn DR, Smeitink J. Diagnosis of mitochondrial disorders: clinical and biochemical approach. J Inherit Metab Dis. 2001;24:312–6.PubMedCrossRef
3.
Zurück zum Zitat Bernier FP, Boneh A, Dennett X, Chow CW, Cleary MA, Thorburn DR. Diagnostic criteria for respiratory chain disorders in adults and children. Neurology. 2002;59:1406–11.PubMedCrossRef Bernier FP, Boneh A, Dennett X, Chow CW, Cleary MA, Thorburn DR. Diagnostic criteria for respiratory chain disorders in adults and children. Neurology. 2002;59:1406–11.PubMedCrossRef
4.
Zurück zum Zitat Footitt EJ, Sinha MD, Raiman JA, Dhawan A, Moganasundram S, Champion MP. Mitochondrial disorders and general anaesthesia: a case series and review. Br J Anaesth. 2008;100:436–41.PubMedCrossRef Footitt EJ, Sinha MD, Raiman JA, Dhawan A, Moganasundram S, Champion MP. Mitochondrial disorders and general anaesthesia: a case series and review. Br J Anaesth. 2008;100:436–41.PubMedCrossRef
5.
Zurück zum Zitat Driessen J, Willems S, Dercksen S, Giele J, van der Staak F, Smeitink J. Anesthesia-related morbidity and mortality after surgery for muscle biopsy in children with mitochondrial defects. Paediatr Anaesth. 2007;17:16–21.PubMedCrossRef Driessen J, Willems S, Dercksen S, Giele J, van der Staak F, Smeitink J. Anesthesia-related morbidity and mortality after surgery for muscle biopsy in children with mitochondrial defects. Paediatr Anaesth. 2007;17:16–21.PubMedCrossRef
6.
Zurück zum Zitat Rosaeg OP, Morrison S, MacLeod JP. Anaesthetic management of labour and delivery in the parturient with mitochondrial myopathy. Can J Anaesth. 1996;43:403–7.PubMedCrossRef Rosaeg OP, Morrison S, MacLeod JP. Anaesthetic management of labour and delivery in the parturient with mitochondrial myopathy. Can J Anaesth. 1996;43:403–7.PubMedCrossRef
7.
Zurück zum Zitat Hsiao PN, Cheng YJ, Tseng HC, Chuang YH, Kao PF, Tsai SK. Spinal anesthesia in MELAS syndrome: a case with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes. Acta Anaesthesiol Sin. 2000;38:107–10.PubMed Hsiao PN, Cheng YJ, Tseng HC, Chuang YH, Kao PF, Tsai SK. Spinal anesthesia in MELAS syndrome: a case with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes. Acta Anaesthesiol Sin. 2000;38:107–10.PubMed
8.
Zurück zum Zitat Lauwers MH, Van Lersberghe C, Camu F. Inhalation anaesthesia and the Kearns–Sayre syndrome. Anaesthesia. 1994;49:876–8.PubMedCrossRef Lauwers MH, Van Lersberghe C, Camu F. Inhalation anaesthesia and the Kearns–Sayre syndrome. Anaesthesia. 1994;49:876–8.PubMedCrossRef
9.
Zurück zum Zitat Miller RD. Miller’s anesthesia. 7th ed. Philadelphia: Churchill Livingstone; 2009. Miller RD. Miller’s anesthesia. 7th ed. Philadelphia: Churchill Livingstone; 2009.
10.
Zurück zum Zitat Fleisher LA. Anesthesia and uncommon diseases. 5th ed. Philadelphia: Elsevier; 2005. Fleisher LA. Anesthesia and uncommon diseases. 5th ed. Philadelphia: Elsevier; 2005.
11.
Zurück zum Zitat Hanley PJ, Ray J, Brandt U, Daut J. Halothane, isoflurane and sevoflurane inhibit NADH:ubiquinone oxidoreductase (complex I) of cardiac mitochondria. J Physiol. 2002;544:687–93.PubMedCrossRef Hanley PJ, Ray J, Brandt U, Daut J. Halothane, isoflurane and sevoflurane inhibit NADH:ubiquinone oxidoreductase (complex I) of cardiac mitochondria. J Physiol. 2002;544:687–93.PubMedCrossRef
12.
Zurück zum Zitat Bains R, Moe MC, Vinje ML, Berg-Johnsen J. Sevoflurane and propofol depolarize mitochondria in rat and human cerebrocortical synaptosomes by different mechanisms. Acta Anaesthesiol Scand. 2009;53:1354–60.PubMedCrossRef Bains R, Moe MC, Vinje ML, Berg-Johnsen J. Sevoflurane and propofol depolarize mitochondria in rat and human cerebrocortical synaptosomes by different mechanisms. Acta Anaesthesiol Scand. 2009;53:1354–60.PubMedCrossRef
13.
Zurück zum Zitat Morgan PG, Hoppel CL, Sedensky MM. Mitochondrial defects and anesthetic sensitivity. Anesthesiology. 2002;96:1268–70.PubMedCrossRef Morgan PG, Hoppel CL, Sedensky MM. Mitochondrial defects and anesthetic sensitivity. Anesthesiology. 2002;96:1268–70.PubMedCrossRef
14.
Zurück zum Zitat Gurrieri C, Kivela JE, Bojanic K, Gavrilova RH, Flick RP, Sprung J, Weingarten TN. Anesthetic considerations in mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome: a case series. Can J Anaesth. 2011;58:751–63. Gurrieri C, Kivela JE, Bojanic K, Gavrilova RH, Flick RP, Sprung J, Weingarten TN. Anesthetic considerations in mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome: a case series. Can J Anaesth. 2011;58:751–63.
15.
16.
Zurück zum Zitat Koruk S, Mizrak A, Kaya Ugur B, Ilhan O, Baspinar O, Oner U. Propofol/dexmedetomidine and propofol/ketamine combinations for anesthesia in pediatric patients undergoing transcatheter atrial septal defect closure: a prospective randomized study. Clin Ther. 2010;32:701–9. Koruk S, Mizrak A, Kaya Ugur B, Ilhan O, Baspinar O, Oner U. Propofol/dexmedetomidine and propofol/ketamine combinations for anesthesia in pediatric patients undergoing transcatheter atrial septal defect closure: a prospective randomized study. Clin Ther. 2010;32:701–9.
17.
Zurück zum Zitat Segal IS, Vickery RG, Walton JK, Doze VA, Maze M. Dexmedetomidine diminishes halothane anesthetic requirements in rats through a postsynaptic alpha 2 adrenergic receptor. Anesthesiology. 1988;69:818–23.PubMedCrossRef Segal IS, Vickery RG, Walton JK, Doze VA, Maze M. Dexmedetomidine diminishes halothane anesthetic requirements in rats through a postsynaptic alpha 2 adrenergic receptor. Anesthesiology. 1988;69:818–23.PubMedCrossRef
18.
Zurück zum Zitat Shukry M, Kennedy K. Dexmedetomidine as a total intravenous anesthetic in infants. Paediatr Anaesth. 2007;17:581–3.PubMedCrossRef Shukry M, Kennedy K. Dexmedetomidine as a total intravenous anesthetic in infants. Paediatr Anaesth. 2007;17:581–3.PubMedCrossRef
19.
Zurück zum Zitat Engelhard K, Werner C, Kaspar S, Mollenberg O, Blobner M, Bachl M, Kochs E. Effect of the alpha2-agonist dexmedetomidine on cerebral neurotransmitter concentrations during cerebral ischemia in rats. Anesthesiology. 2002;96:450–7.PubMedCrossRef Engelhard K, Werner C, Kaspar S, Mollenberg O, Blobner M, Bachl M, Kochs E. Effect of the alpha2-agonist dexmedetomidine on cerebral neurotransmitter concentrations during cerebral ischemia in rats. Anesthesiology. 2002;96:450–7.PubMedCrossRef
20.
Zurück zum Zitat Wong J, Steil GM, Curtis M, Papas A, Zurakowski D, Mason KP. Cardiovascular effects of dexmedetomidine sedation in children. Anesth Analg. 2012;114:193–9. Wong J, Steil GM, Curtis M, Papas A, Zurakowski D, Mason KP. Cardiovascular effects of dexmedetomidine sedation in children. Anesth Analg. 2012;114:193–9.
21.
Zurück zum Zitat Mastronicola D, Arcuri E, Arese M, Bacchi A, Mercadante S, Cardelli P, Citro G, Sarti P. Morphine but not fentanyl and methadone affects mitochondrial membrane potential by inducing nitric oxide release in glioma cells. Cell Mol Life Sci. 2004;61:2991–7.PubMedCrossRef Mastronicola D, Arcuri E, Arese M, Bacchi A, Mercadante S, Cardelli P, Citro G, Sarti P. Morphine but not fentanyl and methadone affects mitochondrial membrane potential by inducing nitric oxide release in glioma cells. Cell Mol Life Sci. 2004;61:2991–7.PubMedCrossRef
22.
Zurück zum Zitat Finsterer J, Stratil U, Bittner R, Sporn P. Increased sensitivity to rocuronium and atracurium in mitochondrial myopathy. Can J Anaesth. 1998;45:781–4.PubMedCrossRef Finsterer J, Stratil U, Bittner R, Sporn P. Increased sensitivity to rocuronium and atracurium in mitochondrial myopathy. Can J Anaesth. 1998;45:781–4.PubMedCrossRef
23.
Zurück zum Zitat D’Ambra MN, Dedrick D, Savarese JJ. Kearns–Sayer syndrome and pancuronium–succinylcholine-induced neuromuscular blockade. Anesthesiology. 1979;51:343–5.PubMedCrossRef D’Ambra MN, Dedrick D, Savarese JJ. Kearns–Sayer syndrome and pancuronium–succinylcholine-induced neuromuscular blockade. Anesthesiology. 1979;51:343–5.PubMedCrossRef
24.
Zurück zum Zitat Wiesel S, Bevan JC, Samuel J, Donati F. Vecuronium neuromuscular blockade in a child with mitochondrial myopathy. Anesth Analg. 1991;72:696–9.PubMedCrossRef Wiesel S, Bevan JC, Samuel J, Donati F. Vecuronium neuromuscular blockade in a child with mitochondrial myopathy. Anesth Analg. 1991;72:696–9.PubMedCrossRef
25.
Zurück zum Zitat Rowe RW, Helander E. Anesthetic management of a patient with systemic carnitine deficiency. Anesth Analg. 1990;71:295–7.PubMedCrossRef Rowe RW, Helander E. Anesthetic management of a patient with systemic carnitine deficiency. Anesth Analg. 1990;71:295–7.PubMedCrossRef
26.
Zurück zum Zitat Stickler DE, Valenstein E, Neiberger RE, Perkins LA, Carney PR, Shuster JJ, Theriaque DW, Stacpoole PW. Peripheral neuropathy in genetic mitochondrial diseases. Pediatr Neurol. 2006;34:127–31.PubMedCrossRef Stickler DE, Valenstein E, Neiberger RE, Perkins LA, Carney PR, Shuster JJ, Theriaque DW, Stacpoole PW. Peripheral neuropathy in genetic mitochondrial diseases. Pediatr Neurol. 2006;34:127–31.PubMedCrossRef
27.
Zurück zum Zitat Muravchick S. Clinical implications of mitochondrial disease. Adv Drug Deliv Rev. 2008;60:1553–60.PubMedCrossRef Muravchick S. Clinical implications of mitochondrial disease. Adv Drug Deliv Rev. 2008;60:1553–60.PubMedCrossRef
28.
Zurück zum Zitat Fernandes J. Inborn metabolic diseases: diagnosis and treatment. 4th ed. Heidelberg: Springer; 2006.CrossRef Fernandes J. Inborn metabolic diseases: diagnosis and treatment. 4th ed. Heidelberg: Springer; 2006.CrossRef
29.
Zurück zum Zitat Paut O, Lacroix F. Recent developments in the perioperative fluid management for the paediatric patient. Curr Opin Anaesthesiol. 2006;19:268–77.PubMedCrossRef Paut O, Lacroix F. Recent developments in the perioperative fluid management for the paediatric patient. Curr Opin Anaesthesiol. 2006;19:268–77.PubMedCrossRef
30.
Zurück zum Zitat Hirshberg E, Larsen G, Van Duker H. Alterations in glucose homeostasis in the pediatric intensive care unit: hyperglycemia and glucose variability are associated with increased mortality and morbidity. Pediatr Crit Care Med. 2008;9:361–6.PubMedCrossRef Hirshberg E, Larsen G, Van Duker H. Alterations in glucose homeostasis in the pediatric intensive care unit: hyperglycemia and glucose variability are associated with increased mortality and morbidity. Pediatr Crit Care Med. 2008;9:361–6.PubMedCrossRef
31.
Zurück zum Zitat Delogu G, Antonucci A, Moretti S, Marandola M, Tellan G, Signore M, Famularo G. Oxidative stress and mitochondrial glutathione in human lymphocytes exposed to clinically relevant anesthetic drug concentrations. J Clin Anesth. 2004;16:189–94.PubMedCrossRef Delogu G, Antonucci A, Moretti S, Marandola M, Tellan G, Signore M, Famularo G. Oxidative stress and mitochondrial glutathione in human lymphocytes exposed to clinically relevant anesthetic drug concentrations. J Clin Anesth. 2004;16:189–94.PubMedCrossRef
Metadaten
Titel
Anesthesia for children with mitochondrial disorders: a national survey and review
verfasst von
Muhammad B. Rafique
Staci D. Cameron
Qaiser Khan
Suur Biliciler
Salman Zubair
Publikationsdatum
01.04.2013
Verlag
Springer Japan
Erschienen in
Journal of Anesthesia / Ausgabe 2/2013
Print ISSN: 0913-8668
Elektronische ISSN: 1438-8359
DOI
https://doi.org/10.1007/s00540-012-1488-1

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