Case Report: General Anesthetic Management for Laparoscopic Cholecystectomy in Paramyotonia Congenita

  • Analise McGreal Mercer University School of Medicine, Macon, Georgia, United States.
  • Daniel Slagle Mercer University School of Medicine, Macon, Georgia, United States.
  • Andrew Dickens Anesthesia Associates of Columbus. Emory-St. Francis Hospital in Columbus, Georgia, United States.
Keywords: Anesthesia, Paramyotonia congenita, Laparoscopic cholecystectomy, General anesthesia, Sodium channel muscle disorders

Abstract

Background: Paramyotonia congenita (PC) is a rare disorder affecting skeletal muscle. Patients with this non-progressive condition experience intermittent episodes of sustained myotonia. Due to the predisposition for prolonged muscle contraction, special attention must be given to anesthetic management during operative procedures to prevent complications similar to those experienced in patients with malignant hyperthermia. To date, however, limited reports of anesthetic management in paramyotonia congenita are available.

The Case: The present report describes successful general anesthetic management given to a patient with paramyotonia congenita using propofol and ketamine for induction, non-depolarizing rocuronium for muscle paralysis, and continuous nitrous oxide and IV propofol infusion for sedation. The patient remained stable throughout the case without myotonic episodes or other complications.

Conclusion: Our report describes successful anesthetic management in a patient with paramyotonia congenita. This provides a potential management plan that may be applied to PC patients undergoing a variety of surgical procedures and eliminates risk associated with succinylcholine and possibly volatile anesthetics. Further research is needed to determine whether this approach is superior to previously reported techniques and should also identify which agents may be effectively utilized to reverse an intra-operative myotonic episode in PC.

Author Biographies

Analise McGreal, Mercer University School of Medicine, Macon, Georgia, United States.

Analise McGreal is a third year medical student at Mercer University School of Medicine in Columbus, GA. She is a member of the Alpha Omega Alpha Honor Medical Society. She plans to pursue training in internal medicine.

Daniel Slagle, Mercer University School of Medicine, Macon, Georgia, United States.

Third-year Medical Student.

Andrew Dickens, Anesthesia Associates of Columbus. Emory-St. Francis Hospital in Columbus, Georgia, United States.

MD, Staff anesthesiologist

References

Finsterer J. Primary periodic paralyses. Acta Neurol Scand. 2008: 117: 145–158.

Schieren M, Defosse J, Böhmer A, Wappler F, Gerbershagen MU. Anaesthetic management of patients with myopathies. Eur J Anaesthesiol. 2017; 34(10): 641 – 649.

Parness J, Bandschapp O, Girard T. The myotonias susceptibility to malignant hyperthermia. Anesth Analg. 2009; 109 (4); 1064-1054.

Bandchapp O, Iaizzo PA. Pathophysiologic and anesthetic considerations for patients with myotonia congenita or periodic paralyses. Paediatr Anaesth. 2013; 23. 824-833.

Ay B, Gerçek A, Doğan VI, Kiyan G, Göğüş YF. Pyloromyotomy in a patient with paramyotonia congenita. Anesth Analg. 2004; 98(1): 68 – 69.

Matsumoto N, Nishimoto R, Matsuoka Y, Takeda Y, Morimatsu H. Anesthetic management of a patient with sodium-channel myotonia: a case report. JA Clin Rep. 2019; 5(1): 77.

Vevckemans F, Scholtes JL. Myotonic dystrophies type 1 and 2: anesthetic care. Paediatr Anaesth. 2013 Sep; 23(9):794-803.

Elsharydah A, Kaminski AC. Propofol reduces succinylcholine-induced muscle rigidity in a patient with paramyotonia congenita. Anesth Essays Res 2017 Jan-Mar, 11(1) 274-273.

Haeseler G, Stormer M, Bufler J, Dengler R, Hecker H, Piepenbrock S, Leuwer M. Propofol blocks human skeletal muscle sodium channels in a voltage-dependent manner. Anesth Analg. 2001; 92 (5): 1198-1192.

Statland JM, Bundy BN, Wang Y, Rayan DR, Trivedi JR, Sansone VA, et al. Mexiletine for symptoms and signs of myotonia in nondystrophic myotonia: a randomized controlled trial. JAMA. 2012;308(13):1357–65.

Published
2020-12-09
How to Cite
McGreal, A., Slagle, D., & Dickens, A. (2020). Case Report: General Anesthetic Management for Laparoscopic Cholecystectomy in Paramyotonia Congenita. International Journal of Medical Students, 8(3), 288-290. https://doi.org/10.5195/ijms.2020.604
Section
Case Report