Brazilian Journal of Anesthesiology
https://app.periodikos.com.br/journal/rba/article/doi/10.1590/S0034-70942010000200011
Brazilian Journal of Anesthesiology
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Anestesia para colecistectomia videolaparoscópica em paciente portador de Doença de Steinert: relato de caso e revisão de literatura

Anesthesia for videolaparoscopic cholecystectomy in a patient with Steinert Disease: case report and review of the literature

Flora Margarida Barra Bisinotto; Daniel Capucci Fabri; Maida Silva Calçado; Paula Borela Perfeito; Lucas Vieira Tostes; Gabriela Denardi Sousa

http://dx.doi.org/10.1590/S0034-70942010000200011 Braz J Anesthesiol, vol.60, n2, p.181-186, 2010
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Resumo

JUSTIFICATIVA E OBJETIVOS: As distrofias miotônicas são doenças neuromusculares de transmissão autossômica dominante. Dentre elas, a distrofia miotônica tipo 1 (DM1), ou doença de Steinert, é a mais comum no adulto e, além do envolvimento muscular, apresenta manifestações sistêmicas importantes. A DM1 representa um desafio para o anestesiologista. Os pacientes apresentam maior sensibilidade às drogas anestésicas e complicações, principalmente cardíacas e pulmonares. Além disso, há a possibilidade de apresentarem hipertermia maligna e crise miotônica. Descreveu-se o caso de um paciente que teve complicação pulmonar importante após ser submetido à anestesia geral. RELATO DO CASO: Paciente de 39 anos, portador de DM1, foi submetido à anestesia geral para colecistectomia videolaparoscópica. A anestesia foi venosa total com propofol e remifentanil e rocurônio. O procedimento cirúrgico de 90 minutos não apresentou intercorrências, mas após a extubação o paciente apresentou insuficiência respiratória e crise miotônica, que tornou a intubação traqueal impossível. Utilizou-se a máscara laríngea, que possibilitou a oxigenação adequada, e a ventilação mecânica foi mantida até a recuperação total da atividade respiratória. Evolução ocorreu sem outras complicações. CONCLUSÕES: A DM1 é uma doença que apresenta várias peculiaridades para o anestesiologista. O conhecimento minucioso do seu envolvimento sistêmico, associado à ação diferenciada das drogas anestésicas nesses pacientes, proporcionará um ato anestésicocirúrgico mais seguro.

Palavras-chave

DOENÇAS, Muscular, CIRURGIA, Abdominal, COMPLICAÇÕES, Pós-Operatórias

Abstract

BACKGROUND AND OBJECTIVES: Myotonic dystrophies are autosomal dominant neuromuscular diseases. Among them, myotonic dystrophy type 1 (MD1), or Steinert disease, is the most common in adults, and besides muscular involvement it also has important systemic manifestations. Myotonic dystrophy type 1 poses a challenge to the anesthesiologist. Those patients are more sensitive to anesthetics and prone to cardiac and pulmonary complications. Besides, the possibility of developing malignant hyperthermia and myotonic episodes is also present. CASE REPORT: This is a 39-year old patient with DM1 who underwent general anesthesia for videolaparoscopic cholecystectomy. Total intravenous anesthesia with propofol, remifentanil, and rocuronium was the technique chosen. Intercurrences were not observed in the 90-minute surgical procedure, but after extubation, the patient developed respiratory failure and myotonia, which made tracheal intubation impossible. A laryngeal mask was used, allowing adequate oxygenation, and mechanical ventilation was maintained until full recovery of the respiratory function. The patient did not develop further complications. CONCLUSIONS: Myotonic dystrophy type 1 presents several particularities to the anesthesiologist. Detailed knowledge of its systemic involvement along with the differentiated action of anesthetic drugs in those patients will provide safer anesthetic-surgical procedure.

Keywords

COMPLICATIONS, Postoperative, DISEASES, Muscular, SURGERY, abdominal

Referências

Tramonte JJ, Burns TM. Myotonic dystrophy. Arch Neurol. 2005;62:1316-19.

Day JW, Ranum LPW. Genetics and molecular pathogenesis of the myotonic dystrophies. Curr Neurol Neurosci Rep. 2005;5:55-59.

Mahadevan M, Tsilfidis C, Sabourin L. Myotonic dystrophy mutation: an unstable CTG repeat in the 3' untranslated region of the gene. Science. 1992;255:1253-1255.

Kornblum C, Lutterbey G, Bogdanow M. Distinct neuromuscular phenothypes in myotonic dystrophy types 1 e 2: a whole body highfield MRI study. J Neurol. 2006;253:753-761.

Luek JD, Mankodi A, Swanson MS. Muscle chloride channel dysfunction in two mouse models of myotonic dystrophy. J Gen Physiol. 2007;129:79-94.

Panaite PA, Gantelet E, Kraftsik R. Myotonic dystrophy transgenic mice exhibit pathologic abnormalities in diaphragm neuromuscular junctions and phrenic nerves. J Neuropathol Exp Neurol. 2008;67:763-772.

Sovari AA, Bodine CK, Farokhi F. Cardiovascular manifestations of myotonic dystrophy-1. Cardiol Rev. 2007;15:191-194.

Bassez G, Lazarus A, Desguerre I. Severe cardiac arrhythmias in young patients with myotonic dystrophy type 1. Neurology. 2004;63:1939-1941.

Ugalde V, Walsh S, Abresch RT. Respiratory abdominal muscle recruitment and chest wall motion in myotonic muscular dystrophy. J Appl Physiol. 2001;91:395-407.

Begin P, Mathieu J, Almirrall J. Relationship between chronic hypercapnia and inspiratory: muscle weakness in myotonic dystrophy. Am J Respir Crit Care Med. 1997;156:133-139.

Ronnblom A, Andersson S, Hellstrom PM. Gastric emptying in myotonic dystrophy. Eur J Clin Invest. 2002;32:570-574.

Ertekin C, Yüceyar N, Aydogdu Ï. Electrophysiological evaluation of oropharyngeal swallowing in myotonic dystrophy. J Neurol Neurosurg Psychiatry. 2001;70:363-371.

Heatwole CR, Miller J, Martens B. Laboratory abnormalities in ambulatory patients with myotonic dystrophy type 1. Arch Neurol. 2006;63:1149-1153.

Modoni A, Silvestri G, Pomponi MG. Characterization of the pattern of cognitive impairment in myotonic dystrophy type 1. Arch Neurol. 2004;61:1943-1947.

Antonini G, Soscia F, Giubilei F. Health-related quality of life in myotonic dystrophy type 1 and its relationship with cognitive and emotional functioning. J Rehabil Med. 2006;38:181-185.

Schulz PE, McIntosh AD, Kasten MR. A role for myotonic dystrophy protein kinase in synaptic plasticity. J Neurophysiol. 2003;89:1177-1186.

Mahr A, Attof Y, Flamens C. Prise en charge anesthésique des patients porteurs de myotonie de Steinert: à propos de deux cas cliniques. Ann Fr Anesth Reanim. 2009;28:161-164.

Takhar AS, Thaper A, Byrne A. Laparoscopic cholecystectomy in a patient with myotonic dystrophy. J R Soc Med. 2004;97:284-5.

Araújo FS, Bessa Jr RC, Castro CH. Anestesia no paciente com doença de Steinert: Relato de caso. Rev Bras Anestesiol. 2006;56:649-653.

Speedy H. Exaggerated physiological response to propofol in myotonic dystrophy. Br J Anaesth. 1990;64:110-112.

Morimoto Y, Mii M, Hirata T. Target-controlled infusion of propofol for a patient with myotonic dystrophy. J Anesth. 2005;19:336-338.

Parness J, Bandschapp O, Girard T. The myotonias and susceptibility to malignant hyperthermia. Anesth Analg. 2009;109:1054-1064.

Larach MG, Rosenberg H, Gronert GA. Hyperkalemic cardiac arrest during anesthesia in infants and children with occult myopathies. Clin Pediatr (Phila). 1997;36:9-16.

Tomlison S, Macartney I, Lam S. Dystrophica myotonia and suxamethonium. Anaesthesia. 1999;54.

Nightingale P, Healy TEJ, McGuinness K. Dystrophia myotonica and atracurium: A case report. Br J Anaesth. 1985;57:1131-5.

Nishi M, Itoh H, Tsubokawa T. Effective doses of vecuronium in a patient with myotonic dystrophy. Anaesthesia. 2004;59:1216-1218.

Diefenbach C, Lynch J, Abel M. Vecuronium for muscle relaxation in patients with dystrophia myotonica. Anesth Analg. 1993;76:872-874.

Buzello W, Krieg N, Schlickewei A. Hazards of neostgmine in patients with neuromuscular disorders: Report of two cases. Br J Anaesth. 1982;54:529-534.

Grimsehl K, Wilson E. Remifentanil in myotonic dystrophy: avoiding the use of muscle relaxants and long acting opioids. Internet J Anesthesiol. 2000;4(1).

Sei Y, Sambuughin N, Muldoon S. Malignant hyperthermia genetic testing in North America, Working Group Meeting: Bethesda, Maryland, September 4-5, 2002. Anesthesiology. 2004;100:464-465.

Payen JF, Bosson JL, Brambilla E. Histological support for the difference between malignant hyperthermia susceptible (MHS), equivocal (MHE) and negative (MHN) muscle biopsies. Br J Anaesth. 1997;79:327-331.

Ioscovich A, Barth D, Briskin A. Biphasic intermittent positive airway pressure (BIPAP) ventilation support in the postoperative period for patients with myotonic dystrophy. Internet J Anesthesiol. 2006;10(2).

Wappler F. Malignant hyperthermia. Eur J Anaesthesiol. 2001;18:632-652.

Gelsomino S, Lorusso R, Billè G. Cardiac surgery in type-1-myotonic muscular dystrophy (Steinert syndrome) associated to Barlow disease. Interactive Cardiovasc Thorac Surg. 2008;7:222-6.

Klompe L, Lancé M, van der Woerd D. Anaesthesiological and ventilatory precautions during cardiac surgery in Steinert's disease. J Card Surg. 2007;22:74-75.

Mathieu J, Allard P, Gobeil G. Anesthetic and surgical complications in 219 cases of myotonic dystrophy. Neurology. 1997;49:1649-1650.

McCrory CR, McShane AJ. Gastroesophageal reflux during spontaneous respiration with the laryngeal mask airway. Can J Anaesth. 1999;46:268-270.

White RJ, Bass S. Anaesthetic management of a patient with myotonic dystrophy. Paediatr Anaesth. 2001;11:494-497.

Aguilo R, Togores B, Pons S. Noninvasive ventilatory support after lung resectional surgery. Chest. 1997;112:117-121.

Groudine SB, Lumb PD, Sandison MR. Pressure support ventilation with the laryngeal mask airway: a method to manage severe reactive airway disease postoperatively. Can J Anaesth. 1995;42:341-343.

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