Anesthetic implications of muscular dystrophies

  • Piedad Cecilia Echeverry-Marín Instituto Roosevelt, Bogotá, Colombia
  • Ángela María Bustamante-Vega Fundación Universitaria de Ciencias de la Salud, Bogotá, Colombia.
Keywords: Muscular Dystrophies, Myotonic Dystrophy, Muscular Dystrophy, Duchenne, Myotonia Congenita, Anesthesia



Muscular dystrophies are a group of genetic diseases characterized by the compromised synthesis or regeneration of the muscle contractile proteins. Although they belong to the same group of diseases, they have different characteristics in their clinical presentation and in their genetic origin. These diseases are classified as orphan as they have a low incidence among the general population, but represent a huge anesthetic challenge, particularly among the pediatric population.


To describe the main clinical aspects of muscular dystrophies, their etiology, anesthetic implications, and the major complications that may occur during the perioperative management.


A review article is discussed based on a systematic search of the literature to produce a descriptive review. The main source of information is case reports obtained from databases as PubMed, Google Scholar, and websites specialized in rare diseases, to describe the main anesthetic implications of muscular dystrophies.


A total of 65 references were identified by the authors in accordance with the relevance of the topic for the final review.


Muscular dystrophies are a heterogeneous group of diseases that share a common etiology due to direct injury of the muscle fiber with a progressive and systemic compromise. Each type of muscular dystrophy is different in terms of its clinical presentation, genetic origin, and anesthetic risks which are mainly cardiovascular complications due to malignant arrhythmias, acute rhabdomyolysis triggered by drugs used in anesthesia, and perioperative respiratory failure.


1. Hoppe K, Reyher C, Jurkatt-Rott K, et al. Distrofia miotónica 1 y 2. 2014; [Cited 2017 Feb 6]. Available at:

2. Jimenez N, Linston D. Distrofia muscular congénita por deficiencia de merosina. 2013; [Cited 2017 Feb 6]. Available at:énita-por-deficiencia-de-merosina.html.

3. Munster T. Distrofia muscular de Duchenne. 2011; [Cited 2017 Feb 6]. Available at:

4. De Boer HD, Van Esmond J, Booij L, et al. Reversal of rocuronium-induced profound neuromuscular block by sugammadex in Duchenne muscular dystrophy. Paediatr Anaesth 2009;19:1226-1228.

5. Gurnaney H, Brown A, Litman RS. Malignant hyperthermia and muscular dystrophies. Anesth Analg 2009;109:1043-1048.

6. Hayes J, Veyckemans F, Bissonnette B. Duchenne muscular dystrophy: an old anesthesia problem revisited. Paediatr Anaesth 2008;18:100-106.

7. Cheuk DK, Wong V, Wraige E, et al. Surgery for scoliosis in Duchenne muscular dystrophy. Cochrane Database Syst Rev 2007;24 1:CD005375.

8. Segura LG, Lorenz JD, Weingarten TN, et al. Anesthesia and Duchenne orBeckermuscular dystrophy: reviewof117 anesthetic exposures. Paediatr Anaesth 2013;23:855-864.

9. Driessen JJ. Neuromuscular and mitochondrial disorders: what is relevant to the anaesthesiologist? Curr Opin Anaesthesiol 2008;21:350.

10. himauchi T, Yamaura K, Sugibe S, et al. Usefulness of sugammadex in a patient with Becker muscular dystrophy and dilated cardiomyopathy. Acta Anaesthesiol Taiwan 2014;52:146-148.

11. Lerman J. Perioperative management of the paediatric patient with coexisting neuromuscular disease. Br J Anaesth 2011;107 (suppl 1):i79-i89.

12. Veyckemans F, Scholtes JL. Myotonic dystrophies type 1 and 2: anesthetic care. Paediatr Anaesth 2013;23:794-803.

13. Catena V, Del Monte DD, Rubini A, et al. Anesthesia and myotonic dystrophy (Steinert's syndrome). The role of total intravenous anesthesia with propofol, cisatracurium and remifentanyl. Case report. Minerva Anestesiol 2007;73:475-479.

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

15. Weingarten TN, Hofer RE, Milone M, et al. Anesthesia and myotonic dystrophy type 2: a case series. Can J Anaesth 2010; 57:248-255.

16. Haliloglu G, Topaloglu H. Ullrich congenital muscular dystrophy. Iran J Child Neurol 2011;5:1-13.

17. Yonekawa T, Nishino I. Ullrich congenital muscular dystrophy: clinicopathological features, natural history and pathomechanism(s). J Neurol Neurosurg Psychiatry 2015;86:280-287.

18. Bozorgmehr B, Kariminejad A, Nafissi S, et al. Ullrich congenital muscular dystrophy (UCMD): clinical and genetic correlations. Iran J Child Neurol 2013;7:15-22.

19. Park Y, Park MS, Sung DH, et al. Ullrich congenital muscular dystrophy possibly related with COL6A1 p.Gly302Arg variant. Ann Rehabil Med 2014;38:292-296.

20. Prottengeier J, Shammas K, Smith J. Collagen VI-related myopa-thy. 2015; [Cited 2017 Feb 6]. Available at:

21. Miscione MT, Bruno F, Ripamonti C, et al. Body composition, muscle strength, and physical function of patients with Bethlem myopathy and Ullrich congenital muscular dystrophy. Sci World J 2013;2013:152684.

22. Kang PB, Morrison L, Iannaccone ST, et al. Evidence-based guideline summary: evaluation, diagnosis, and management of congenital muscular dystrophy Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Issues Review Panel of the American Association of Neuromuscular & Electrodiagnostic Medicine. Neurology 2015; 84:1369-1378.

23. Lopez-Alvarez A, Roman-Fernandez A. Limb-girdle muscular dystrophy. 2015; [Cited 2017 Feb 6]. Available at:

24. Allen T, Maguire S. Anaesthetic management of a woman with autosomal recessive limb-girdle muscular dystrophy for emergency caesarean section. Int J Obstet Anesth 2007;16:370-374.

25. Richa FC. Anaesthetic management of a patient with limb-girdle muscular dystrophy for laparoscopic cholecystectomy. Eur J Anaesthesiol 2011;28:72-73.

26. Kim OM, Elliott D. Elective caesarean section for a woman with Emery-Dreifuss muscular dystrophy. Anaesth Intensive Care 2010;38:744-747.

27. Shende D, Agarwal R. Anaesthetic management of a patient with Emery-Dreifuss muscular dystrophy. Anaesth Intensive Care 2002;30:372-375.

28. Ozer Y, Medical A. An anaesthetic approach to a case with Emery-Dreifuss muscular dystrophy. J Neurol Sci Turk 2005;22: 195-199. 26.

29. Funnell A. Recomendaciones para anestesia en pacientes que sufren de Distrofia muscular de Emery-Dreifuss (DMED). 2014; [Cited 2017 Feb 6]. Available at:

30. Aldwinckle RJ, Carr AS. The anesthetic management of a patient with Emery-Dreifuss muscular dystrophy for orthopedic surgery. Can J Anaesth 2002;49:467-470.

31. Funnell A, Morgan J, McFadzean W. Anaesthesia and orphan disease: management of cardiac and perioperative risks in a patient with Emery-Dreifuss muscular dystrophy. Euro J Anesthesiol 2012;29:593-604.

32. Schuster F, Wessig C, Schimmer C, et al. In vitro contracture test results and anaesthetic management of a patient with Emery Dreifuss muscular dystrophy for cardiac transplantation. Case Rep Anesthesiol 2015;2012:349046.

33. Jimenez N, Song K, Lynn AM. Hemodynamic instability during prone spine surgery in a patient with merosin-deficient congenital muscular dystrophy. Paediatr Anaesth 2013;23:294-296.

34. Scrivener TA, Ross SM, Street NE, et al. A case series of general anesthesia in children with laminin alpha2 (merosin)-deficient congenital muscular dystrophy. Paediatr Anaesth 2014;24: 464-465.

35. Apiliogullari S, Oc B, Kara I, et al. Unilateral spinal anesthesia in a pediatric patient with Duchenne muscular dystrophy: a case report. Paediatr Anaesth 2013;23:1106-1107.

36. Pickard A, Lobo C, Stoddart PA. The effect of rocuronium and sugammadex on neuromuscular blockade in a child with congenital myotonic dystrophy type 1. Paediatr Anaesth 2013; 23:871-873.

37. Vandenberghe W, Jacobs TF, Plasschaert FS, et al. Anesthesia and perioperative management for a patient with Ullrich syndrome undergoing surgery for scoliosis. Acta Anaesthesiol Belg 2010; 61:43-47.

38. Carrasco-Marina ML, Quijano-Roy S, Iglesias-Escalera G, et al. Ullrich congenital muscular dystrophy. The usefulness of muscular magnetic resonance imaging in its diagnosis. Rev Neurol 2015;61:44-46.

39. Khirani S, Dabaj I, Amaddeo A, et al. The value of respiratory muscle testing in a child with congenital muscular dystrophy. Respirol Case Rep 2014;2:95-98.

40. Anta Redondo D, Ruiz Lopez JJ, Gredilla Diaz E, et al. Anesthesia for cesarean section in a patient with limb-girdle muscular dystrophy. Rev Esp Anestesiol Reanim 2008;55:651-652.

41. Miles F, Dare T. Scoliosis repair in a teenager with Duchenne's muscular dystrophy: who calls the shots? Paediatr Anaesth 2009;19:1022-1024.

42. Bisinotto FM, Fabri DC, Calcado MS, et al. Anesthesia for video-laparoscopic cholecystectomy in a patient with Steinert disease. Case report and review of the literature. Rev Bras Anestesiol 2010;60:105-110.

43. Hopkins PM. Anaesthesia and the sex-linked dystrophies: between a rock and a hard place. Br J Anaesth 2010;104: 397-400.

44. Caliskan E, Sener M, Kocum A, et al. Duchenne muscular dystrophy: how I do it? Regional or general anesthesia? Paediatr Anaesth 2009;19:624-625.

45. Van Obbergh LJ, Corteel J, Papadopoulos J, et al. Anesthesia for a child suffering from a deletion in the Xp21 loci resulting in Duchenne disease, glycerol kinase deficiency and congenital adrenal hypoplasia. Paediatr Anaesth 2011;21:1085-1087.

46. Ferschl M, Moxley R, Day JW, et al. Practical suggestions for the anesthetic management of a myotonic dystrophy patient. Myotonic dystrophy foundation toolkit. 2013;73-80. [Cited 2017 Feb 6]. Available at:

47. Muenster T, Mueller C, Forst J, et al. Anaesthetic management in patients with Duchenne muscular dystrophy undergoing orthopaedic surgery: a review of 232 cases. Eur J Anaesthesiol 2012;29:489-494.

48. Schwartz D. Regarding: muscular dystrophy and the safety of inhalational agents. Paediatr Anaesth 2007;17:96 author reply 96-97.

49. Yemen TA, McClain C. Muscular dystrophy, anesthesia and the safety of inhalational agents revisited; again. Pediatr Anesth 2006;16:105-108.

50. Veyckemans F. Can inhalationagents be used in the presence ofa child with myopathy? Curr Opin Anaesthesiol 2010;23:348-355.

51. Baticon Escudero PM, Marcos Vidal JM, Ramos Fernandez R. Incomplete axillary block and sedation with ketamine in a patient with myotonic dystrophy. Rev Esp Anestesiol Reanim 2008;55: 60-61.

52. El-Dawlatly A, Aldohayan A, Nawaz S, et al. Anesthetic management of a patient with myotonic dystrophy for laparoscopic cholecystectomy: a case report. Middle East J Anaesthesiol 2008;19:1135-1140.

53. Muenster T, Schmidt J, Wick S, et al. Rocuronium 0.3mgkg-1 (ED95) induces a normal peak effect but an altered time course of neuromuscular block in patients with Duchenne's muscular dystrophy. Paediatr Anaesth 2006;16:840-845.

54. Stourac P, Krikava I, Seidlova J, et al. Sugammadex in a parturient with myotonic dystrophy. Br J Anaesth 2013;110:657-658.

55. Birnkrant DJ, Panitch HB, Benditt JO, et al. American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing anesthesia or sedation. Chest 2007;132: 1977-1986.

56. Sivathondan D. Myotonic dystrophy and pain management of a patient undergoing total abdominal hysterectomy in a metropolitan general hospital. Anaesth Intensive Care 2006; 34:506-509.

57. Chuang MC, Duggan LV, Van Heest RD, et al. Laparoscopic cholecystectomy under spinal anesthesia in a patient with limb-girdle muscular dystrophy. Can J Anaesth 2013;60:1276-1277.

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

59. Kocabas S, Yedicocuklu D, Askar F, et al. Anesthetic management of a child with Duchenne muscular dystrophy undergoing correction of Fallot’s Tetralogy. Paediatr Anaesth 2008;18:448-450.

60. Vieito M, Plaja I, Vilaplana J, et al. Anesthesia with sevoflurane for tonsillectomy in a boy with Duchenne muscular dystrophy. Rev Esp Anestesiol Reanim 2006;53:437-441.

61. Vandepitte C, Gautier P, Bellen P, et al. Use of ultrasound-guided intercostal nerve block as a sole anaesthetic technique in a high-risk patient with Duchenne muscular dystrophy. Acta Anaesthesiol Belg 2013;64:91-94.

62. Errando CL, Perez-Caballero P. Anaesthetic management in patients with Duchenne muscular dystrophy. Eur J Anaesthesiol 2013;30:257.

63. Sinclair JL, Reed PW. Risk factors for perioperative adverse events in children with myotonic dystrophy. Paediatr Anaesth 2009;19: 740-747.

64. Kocum A, Sener M, Caliskan E, et al. Anesthetic management for a child with unknown type of limb-girdle muscular dystrophy. Pediatr Int 2010;52:e37-e38.

65. Mathieu J, Boivin H, Meunier D, et al. Assessment of a diseasespecific muscular impairment raiting scale in myotonic dystrophy. Neurology 2001;56:336-340.
How to Cite
Echeverry-Marín PC, Bustamante-Vega Ángela M. Anesthetic implications of muscular dystrophies. Colomb. J. Anesthesiol. [Internet]. 2018 Jul. 1 [cited 2024 Apr. 18];46(3):230-41. Available from:


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How to Cite
Echeverry-Marín PC, Bustamante-Vega Ángela M. Anesthetic implications of muscular dystrophies. Colomb. J. Anesthesiol. [Internet]. 2018 Jul. 1 [cited 2024 Apr. 18];46(3):230-41. Available from:
Systematic review


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