Brazilian Journal of Anesthesiology
Brazilian Journal of Anesthesiology
Case Report

Opioid-free general anesthesia and induced recovery from anesthesia in a patient with myotonic dystrophy type-1: a case report

Anestesia geral sem opioide e recuperação induzida da anestesia em paciente com distrofia miotônica tipo-1: relato de caso

Hande Gurbuz Aytuluk, Kemal Tolga Saracoglu

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Myotonic dystrophy type-1 (Steinert disease) is an autosomal dominant, progressive multisystem disease in which myotonic crisis can be triggered by several factors including pain, emotional stress, hypothermia, shivering, and mechanical or electrical stimulation. In this report, dexmedetomidine-based general anesthesia, in combination with a thoracic epidural for laparoscopic cholecystectomy in a patient with Steinert disease, is presented. An Aintree intubation catheter with the guidance of a fiberoptic bronchoscope was used for intubation to avoid laryngoscopy. Prolonged anesthetic effects of propofol were reversed, and recovery from anesthesia was accelerated using an intravenous infusion of theophylline.


Anesthesia recovery period;  Delayed emergence from anesthesia;  Dexmedetomidine;  Exchange catheter;  Myotonic dystrophy;  Theophylline


A Distrofia Miotônica (DM) tipo-1 (Doença de Steinert) é uma doença multissistêmica progressiva autossômica dominante em que a crise miotônica pode ser desencadeada por vários fatores incluindo dor, estresse emocional, hipotermia, tremores e estímulo mecânico ou elétrico. O presente relato descreve anestesia geral realizada com dexmedetomidina em combinação com peridural torácica para colecistectomia laparoscópica em paciente com Doença de Steinert. Para evitar laringoscopia, a intubação traqueal foi realizada utilizando cateter de intubação Aintree guiado por broncofibroscopia óptica. Os efeitos anestésicos prolongados do propofol foram revertidos e a recuperação anestésica foi acelerada pelo uso de infusão intravenosa de teofilina.


Período de recuperação da anestesia;  Despertar prolongado da anestesia;  Dexmedetomidina;  Cateter para troca de tubo traqueal;  Distrofia miotônica;  Teofilina


1 F. Veyckemans, J.L. Scholtes Myotonic dystrophies type 1 and 2: anesthetic care Paediatr Anaesth, 23 (2013), pp. 794-803

2 L. Kirzinger, A. Schmidt, C. Kornblum, et al. Side effects of anesthesia in DM2 as compared to DM1: a comparative retrospective study Eur J Neurol, 17 (2010), pp. 842-845

3 Y. Morimoto, M. Mii, T. Hirata, et al. Target-controlled infusion of propofol for a patient with myotonic dystrophy J Anesth, 19 (2005), pp. 336-338

4 C. Mangla, K. Bais, J. Yarmush Myotonic dystrophy and anesthetic challenges: a case report and review Case Rep Anesthesiol, 2019 (2019), p. 4282305

5 H. Speedy Exaggerated physiological responses to propofol in myotonic dystrophy Br J Anaesth, 64 (1990), pp. 110-112

6 F.M. Bisinotto, D.C. Fabri, M.S. Calçado, et al. Anesthesia for videolaparoscopic cholecystectomy in a patient with Steinert disease. Case report and review of the literature Rev Bras Anestesiol, 60 (2010), pp. 181-191

7 Y. Matsuki, M. Hirose, M. Tabata, et al. The use of sugammadex in a patient with myotonic dystrophy Eur J Anaesthesiol, 28 (2011), pp. 145-146

8 P. Baumgartner Rocuronium and sugammadex in myotonic dystrophy Anaesth Intens Care, 38 (2010), pp. 959-960

9 T. Gaszynski Opioid-free general anesthesia in patient with Steinert syndrome (myotonic dystrophy): case report Medicine (Baltimore), 95 (2016), p. e4885

10 E.H. Vanlinthout, L.H.D.J. Booij, J. Van Egmond, et al. Comparison of mechanomyography and acceleromyography for the assessment of rocuronium induced neuromuscular block in myotonic dystrophy type 1 Anaesthesia, 65 (2010), pp. 601-607

11 I. Azar The response of patients with neuromuscular disorders to muscle relaxants: a review Anesthesiology, 61 (1984), pp. 173-187

12 A.J. Valkenburg, T.G. De Leeuw, D. Tibboel, et al. Lower bispectral index values in children who are intellectually disabled Anesth Analg, 109 (2009), pp. 1428-1433

13 E.L. De Vito, A.J. Roncoroni, G. Semeniuk, et al. Effect of aminophylline in dystrophia myotonica Med (B Aires), 46 (1986), pp. 724-728

14 M.A. Rutherford, J.Z. Heckmatt, V. Dubowitz Congenital myotonic dystrophy: respiratory function at birth determines survival Arch Dis Child, 64 (1989), pp. 191-195

15 Q. Wang, R. Fong, P. Mason, et al. Caffeine accelerates recovery from general anesthesia J Neurophysiol, 111 (2014), pp. 1331-1340

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