Brazilian Journal of Anesthesiology
https://bjan-sba.org/article/doi/10.1016/j.bjane.2019.02.003
Brazilian Journal of Anesthesiology
Scientific Article

Anesthesia for muscle biopsy to test susceptibility to malignant hyperthermia

Anestesia durante biópsia muscular para teste de suscetibilidade à hipertermia maligna

Helga Cristina Almeida da Silva, Elton Shinji Onari, Isac de Castro, Marcelo Vaz Perez, Alexandre Hortensi, José Luiz Gomes do Amaral

Downloads: 1
Views: 761

Abstract

Introduction: Malignant hyperthermia is an autosomal dominant pharmacogenetic disorder, characterized by hypermetabolic crisis triggered by halogenated anesthetics and/or succinylcholine. The standard method for diagnosing malignant hyperthermia susceptibility is the in vitro muscle contracture test in response to halothane-caffeine, which requires muscle biopsy under anesthesia. We describe a series of anesthetic procedures without triggering agents in malignant hyperthermia, comparing peripheral nerve block and subarachnoid anesthesia. Method: We assessed the anesthetic record charts of 69 patients suspected of malignant hyperthermia susceptibility who underwent muscle biopsy forin vitro muscle contracture in the period of 7 years. Demographic data, indication for malignant hyperthermia investigation, in vitro muscle contracture test results, and surgery/anesthesia/recovery data were analyzed. Results: Sample with 34 ± 13.7 years, 60.9% women, 65.2% of in vitro muscle contracture test positive. Techniques used: peripheral nerve blocks ---- lateral femoral and femoral cutaneous, latency 65 ± 41 min ---- (47.8%); subarachnoid anesthesia (49.3%), and total venous anesthesia (1.4%). There was 39.4% failure of peripheral nerve block and 11.8% of subarachnoid anesthesia. Adverse events (8.7%) occurred only with subarachnoid blockade (bradycardia, nausea, and transient neurological syndrome). All patients remained in the post-anesthesia care unit until discharge. Age and weight were significantly higher in patients with blockade failure (ROC cutoff point of 23.5 years and 59.5 kg) and blockade failure was more frequent in the presence of increased idiopathic creatine kinase. Conclusion: Anesthesia with non-triggering agents has been shown to be safe in patients with malignant hyperthermia susceptibility. Variables such as age, weight, and history of increased idiopathic creatine kinase may be useful in selecting the anesthetic technique for this group of patients.

Keywords

Malignant hyperthermia; Anesthesia; Nerve block

Resumo

Introdução: Hipertermia maligna é uma doença farmacogenética autossômica dominante, caracterizada por crise hipermetabólica desencadeada por anestésicos halogenados e/ou succinilcolina. O padrão para diagnóstico da suscetibilidade à hipertermia maligna é o teste de contratura muscular in vitro em resposta ao halotano-cafeína, para o qual é necessária biopsia muscular sob anestesia. Descrevemos uma série de anestesias sem agentes desencadeantes na hipertermia maligna e comparamos bloqueios de nervo periférico e anestesias subaracnóideas. Método: Foram analisados os prontuários/fichas anestésicas de 69 pacientes suspeitos de susceptibilidade à hipertermia maligna, submetidos à biópsia muscular para teste de contratura muscular in vitro durante sete anos. Analisamos dados demográficos, indicação para investigação de hipertermia maligna, resultado do teste de contratura muscular in vitro e dados da cirurgia/anestesia/recuperação. Resultados: Amostra com 34 ± 13,7 anos, 60,9% mulheres, 65,2% de teste de contratura muscular in vitro positivos. Técnicas empregadas: 47,8% bloqueios de nervo periférico (femoral e cutâneo femoral lateral, latência 65 ± 41 minutos), 49,3% anestesias subaracnóideas e 1,4% anestesia venosa total. Falha em 39,4% dos bloqueios de nervo periférico e 11,8% das anestesias subaracnóideas. Eventos adversos (8,7%) como bradicardia, náuseas e síndrome neurológica transitória só ocorreram com bloqueio subaracnóideo. Todos os pacientes permaneceram na sala de recuperação pós-anestésica até liberação. Idade e peso foram significativamente maiores nos pacientes com falha no bloqueio (ponto de corte da curva ROC de 23,5 anos e 59,5 Kg) e esta foi mais frequente na presença de aumento idiopático de creatinoquinase. Conclusão: Anestesia com agentes não desencadeantes mostrou-se segura em pacientes suscetíveis à hipertermia maligna. Variáveis como idade, peso e antecedente de aumento idiopático de creatinoquinase podem ser úteis para selecionar a técnica anestésica nesse grupo.

Palavras-chave

Hipertermia maligna; Anestesia; Bloqueio nervoso

References

1. Denborough MA, Forster JF, Lovell RR, et al. Anaesthetic deaths in a family. Br J Anaesth. 1962;34:395---6.

2. Rosenberg H, Sambuughin N, Riazi S, et al. Malignant hyperthermia susceptibility. In: Pagon RA, Adam MP, Ardinger HH, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993---2015, 1993-2015. 12.19.2003 [accessed 01.31.2013]. [Online]. Available: http://www.ncbi.nlm.nih.gov/books/NBK1146/

3. da Silva HC, Almeida CS, Brandao JC, et al. Malignant hyperthermia in Brazil: analysis of hotline activity in 2009. Braz J Anesthesiol. 2013;63:13---9.

4. Carr AS, Lerman J, Cunliffe M, et al. Incidence of malignant hyperthermia reactions in 2,214 patients undergoing muscle biopsy. Can J Anaesth. 1995;42:281---6.

5. Rosero EB, Adesanya AO, Timaran CH, et al. Trends and outcomes of malignant hyperthermia in the United States, 2000 to 2005. Anesthesiology. 2009;110:89---94.

6. Ellis FR, Halsall PJ, Ording H, et al. A protocol for the investigation of malignant hyperpyrexia (MH) susceptibility. Br J Anaesth. 1984;56:1267---9.

7. Larach MG, for the North American Malignant Hyperthermia Group. Standardization of the caffeine halothane muscle contracture test. Anesth Analg. 1989;69:511---5.

8. Hopkins PM, Rüffert H, Snoeck MM, et al. European Malignant Hyperthermia Group guidelines for investigation of malignant hyperthermia susceptibility. Br J Anaesth. 2015;115:531---9.

9. Wappler F. Anesthesia for patients with a history of malignant hyperthermia. Curr Opin Anaesthesiol. 2010;23:417---22.

10. Scala D, Di Martino A, Cozzolino S, et al. Follow-up of patients tested for malignant hyperthermia susceptibility. Eur J Anaesthesiol. 2006;23:801---5.

11. Grinberg R, Edelist G, Gordon A. Postoperative malignant hyperthermia episodes in patients who received ‘‘safe’’ anaesthetics. Can Anaesth Soc J. 1983;30:273---6.

12. Adnet PJ, Krivosic-Horber RM, Haudecoeur G, et al. Diltiazem and nifedipine reduce the in vitro contracture response to halothane in malignant hyperthermia-susceptible muscle. Can J Anaesth. 1990;37:556---9.

13. Ording H. Influence of propranolol on the in vitro response to caffeine and halothane in malignant hyperthermia-susceptible muscle. Acta Anaesthesiol Scand. 1989;33:405---8.

14. Wappler F, Fiege M, Schulte am Esch J. Pathophysiological role of the serotonin system in malignant hyperthermia. Br J Anaest. 2001;87:794---8.

15. Fiege M, Wappler F, Scholz J, et al. Effects of the phosphodiesterase-III inhibitor enoximone on skeletal muscle specimens from malignant hyperthermia susceptible patients. J Clin Anesth. 2000;12:123---8.

16. Gunter JB, Ball J, Than-Win S. Preparation of the Drager Fabius anesthesia machine for the malignant-hyperthermia susceptible patient. Anesth Analg. 2008;107:1936---45.

17. Kim TW, Nemergut ME. Preparation of modern anesthesia workstations for malignant hyperthermia-susceptible patients: a review of past and present practice. Anesthesiology. 2011;114:205---12.

18. Maccani RM, Wedel DJ, Melton A, et al. Femoral and lateral femoral cutaneous nerve block for muscle biopsies in children. Paediatr Anaesth. 1995;5:223---7.

19. Veering BT. Regional anesthesia and the patient with preexisting neurological disease. Curr Opin Anaesthesiol. 2009;22:634---6.

20. Vercauteren M, Heytens L. Anaesthetic considerations for patients with a pre-existing neurological deficit: are neuraxial techniques safe? Acta Anaesthesiol Scand. 2007; 51:831---8.

21. Brandom BW. Ambulatory surgery and malignant hyperthermia. Curr Opin Anaesthesiol. 2009;22:744---7.

22. Pollock N, Langton E, McDonnell N, et al. Malignant hyperthermia and day stay surgery. Anaesth Intensive Care. 2006;34:40---5.

23. Barnes C, Stowell KM, Bulger T, et al. Safe duration of postoperative monitoring for malignant hyperthermia patients administered non-triggering anaesthesia: an update. Anaesth Intensive Care. 2015;43:98---104.

24. Fettes PD, Jansson JR, Wildsmith JA. Failed spinal anaesthesia: mechanisms, management, and prevention. Br J Anaesth. 2009;102:739---48.

25. Glahn KP, Ellis FR, Halsall PJ, et al. Recognizing and managing a malignant hyperthermia crisis: guidelines from the European Malignant Hyperthermia Group. Br J Anaesth. 2010; 105:417---20.

26. Riazi S, Kraeva N, Hopkins PM. Updated guide for the management of malignant hyperthermia. Can J Anaesth. 2018;65:709---21.

27. Bilmen JG, Hopkins PM. The use of charcoal filters in malignant hyperthermia: have they found their place? Anaesthesia. 2019;74:13---6.

28. Frei D, Stowell KM, Langton EE, et al. Administration of anaesthetic triggering agents to patients tested malignant hyperthermia normal and their relatives in New Zealand: an update. Anaesth Intensive Care. 2017;45:611---8.

5da9f9290e8825706565eae2 rba Articles
Links & Downloads

Braz J Anesthesiol

Share this page
Page Sections