Anesthesia for the patient with myasthenia gravis
- Riki Kveraga, MD
Riki Kveraga, MD
- Instructor in Anesthesiology
- Harvard Medical School
- John Pawlowski, MD, PhD
John Pawlowski, MD, PhD
- Assistant Professor of Anesthesia
- Harvard Medical School
- Section Editors
- Stephanie B Jones, MD
Stephanie B Jones, MD
- Editor-in-Chief — Anesthesiology
- Section Editor — Anesthesia with Comorbid Non-Cardiopulmonary Conditions
- Associate Professor of Anesthesia
- Harvard Medical School
- Jeremy M Shefner, MD, PhD
Jeremy M Shefner, MD, PhD
- Section Editor — Neuromuscular Disease
- Professor and Chair of Neurology, Barrow Neurological Institute
- Professor of Neurology, University of Arizona, Phoenix
- Clinical Professor of Neurology, Creighton University
Myasthenia gravis (MG) is an autoimmune disorder characterized by fatigable weakness of skeletal muscles. Weakness results from an antibody-mediated immunological attack directed at acetylcholine receptors (or receptor-associated proteins) in the postsynaptic membrane of the neuromuscular junction.
Anesthetic concerns for patients with MG include the interactions among the disease, the disease treatment, and the medications used for anesthesia, particularly neuromuscular blocking agents (NMBAs). Patients with MG are unpredictably sensitive to nondepolarizing NMBAs and are resistant to succinylcholine, a depolarizing NMBA.
Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder of the neuromuscular junction, with antibodies directed against the presynaptic voltage-gated calcium channels. It is often associated with an underlying malignancy, most commonly small cell lung cancer, though it is also associated with other autoimmune processes. Patients with LEMS are very sensitive to both depolarizing and nondepolarizing NMBAs.
This topic will discuss the anesthetic management of patients with MG and LEMS. Diagnosis, clinical manifestations, and management of MG and LEMS are discussed in detail separately. (See "Diagnosis of myasthenia gravis" and "Clinical manifestations of myasthenia gravis" and "Treatment of myasthenia gravis" and "Clinical features and diagnosis of Lambert-Eaton myasthenic syndrome" and "Treatment of Lambert-Eaton myasthenic syndrome".)
Preoperative preparation for elective surgery for patients with myasthenia gravis (MG) should be coordinated with the patient’s neurologist. Elective surgery should be performed during a stable phase of the disease, when the patient requires minimal immunomodulatory medication or glucocorticoids, to minimize the chance of postoperative myasthenic crisis. In addition to routine preoperative evaluation, assessment of patients with MG should focus on bulbar and respiratory symptoms, as well as prior history of exacerbations or myasthenic crisis. Surgery should be scheduled as early in the day as possible, when the patient is strongest .
- Jamal BT, Herb K. Perioperative management of patients with myasthenia gravis: prevention, recognition, and treatment. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009; 107:612.
- Loach AB, Young AC, Spalding JM, Smith AC. Postoperative management after thymectomy. Br Med J 1975; 1:309.
- Leventhal SR, Orkin FK, Hirsh RA. Prediction of the need for postoperative mechanical ventilation in myasthenia gravis. Anesthesiology 1980; 53:26.
- Lee HS, Lee HS, Lee HE, et al. Predictive factors for myasthenic crisis after videoscopic thymectomy in patients with myasthenia gravis. Muscle Nerve 2015; 52:216.
- Watanabe A, Watanabe T, Obama T, et al. Prognostic factors for myasthenic crisis after transsternal thymectomy in patients with myasthenia gravis. J Thorac Cardiovasc Surg 2004; 127:868.
- Lacomis D. Myasthenic crisis. Neurocrit Care 2005; 3:189.
- Gracey DR, Divertie MB, Howard FM Jr, Payne WS. Postoperative respiratory care after transsternal thymectomy in myasthenia gravis. A 3-year experience in 53 patients. Chest 1984; 86:67.
- Gritti P, Sgarzi M, Carrara B, et al. A standardized protocol for the perioperative management of myasthenia gravis patients. Experience with 110 patients. Acta Anaesthesiol Scand 2012; 56:66.
- Tripathi M, Kaushik S, Dubey P. The effect of use of pyridostigmine and requirement of vecuronium in patients with myasthenia gravis. J Postgrad Med 2003; 49:311.
- Gramstad L. Atracurium, vecuronium and pancuronium in end-stage renal failure. Dose-response properties and interactions with azathioprine. Br J Anaesth 1987; 59:995.
- Dretchen KL, Morgenroth VH 3rd, Standaert FG, Walts LF. Azathioprine: effects on neuromuscular transmission. Anesthesiology 1976; 45:604.
- Glidden RS, Martyn JA, Tomera JF. Azathioprine fails to alter the dose-response curve of d-tubocurarine in rats. Anesthesiology 1988; 68:595.
- Dillon FX. Anesthesia issues in the perioperative management of myasthenia gravis. Semin Neurol 2004; 24:83.
- Nitahara K, Sugi Y, Higa K, et al. Neuromuscular effects of sevoflurane in myasthenia gravis patients. Br J Anaesth 2007; 98:337.
- Gissen AJ, Karis JH, Nastuk WL. Effect of halothane on neuromuscular transmission. JAMA 1966; 197:770.
- Rowbottom SJ. Isoflurane for thymectomy in myasthenia gravis. Anaesth Intensive Care 1989; 17:444.
- Kiran U, Choudhury M, Saxena N, Kapoor P. Sevoflurane as a sole anaesthetic for thymectomy in myasthenia gravis. Acta Anaesthesiol Scand 2000; 44:351.
- Della Rocca G, Coccia C, Diana L, et al. Propofol or sevoflurane anesthesia without muscle relaxants allow the early extubation of myasthenic patients. Can J Anaesth 2003; 50:547.
- Baftiu N, Hadri B, Morina M, Mustafa A. Anesthesia for trans-sternal thymectomy: modified non-muscle relaxant technique. Med Arh 2011; 65:317.
- Lorimer M, Hall R. Remifentanil and propofol total intravenous anaesthesia for thymectomy in myasthenia gravis. Anaesth Intensive Care 1998; 26:210.
- Bennett JA, Abrams JT, Van Riper DF, Horrow JC. Difficult or impossible ventilation after sufentanil-induced anesthesia is caused primarily by vocal cord closure. Anesthesiology 1997; 87:1070.
- Baraka A, Afifi A, Muallem M, et al. Neuromuscular effects of halothane, suxamethonium and tubocurarine in a myasthenic undergoing thymectomy. Br J Anaesth 1971; 43:91.
- Abel M, Eisenkraft JB, Patel N. Response to suxamethonium in a myasthenic patient during remission. Anaesthesia 1991; 46:30.
- Eisenkraft JB, Book WJ, Mann SM, et al. Resistance to succinylcholine in myasthenia gravis: a dose-response study. Anesthesiology 1988; 69:760.
- Fambrough DM, Drachman DB, Satyamurti S. Neuromuscular junction in myasthenia gravis: decreased acetylcholine receptors. Science 1973; 182:293.
- Baraka A. Suxamethonium block in the myasthenic patient. Correlation with plasma cholinesterase. Anaesthesia 1992; 47:217.
- Baraka A, Baroody M, Yazbeck V. Repeated doses of suxamethonium in the myasthenic patient. Anaesthesia 1993; 48:782.
- Gitlin MC, Jahr JS, Margolis MA, McCain J. Is mivacurium chloride effective in electroconvulsive therapy? A report of four cases, including a patient with myasthenia gravis. Anesth Analg 1993; 77:392.
- Paterson IG, Hood JR, Russell SH, et al. Mivacurium in the myasthenic patient. Br J Anaesth 1994; 73:494.
- Baraka A. Anesthesia and critical care of thymectomy for myasthenia gravis. Chest Surg Clin N Am 2001; 11:337.
- Murphy GS, Szokol JW, Marymont JH, et al. Intraoperative acceleromyographic monitoring reduces the risk of residual neuromuscular blockade and adverse respiratory events in the postanesthesia care unit. Anesthesiology 2008; 109:389.
- Plaud B, Debaene B, Donati F, Marty J. Residual paralysis after emergence from anesthesia. Anesthesiology 2010; 112:1013.
- Pavlin EG, Holle RH, Schoene RB. Recovery of airway protection compared with ventilation in humans after paralysis with curare. Anesthesiology 1989; 70:381.
- Viby-Mogensen J, Jensen NH, Engbaek J, et al. Tactile and visual evaluation of the response to train-of-four nerve stimulation. Anesthesiology 1985; 63:440.
- de Boer HD, Shields MO, Booij LH. Reversal of neuromuscular blockade with sugammadex in patients with myasthenia gravis: a case series of 21 patients and review of the literature. Eur J Anaesthesiol 2014; 31:715.
- Juel VC. Myasthenia gravis: management of myasthenic crisis and perioperative care. Semin Neurol 2004; 24:75.
- Kim JM, Mangold J. Sensitivity to both vecuronium and neostigmine in a sero-negative myasthenic patient. Br J Anaesth 1989; 63:497.
- Rolbin WH, Levinson G, Shnider SM, Wright RG. Anesthetic considerations for myasthenia gravis and pregnancy. Anesth Analg 1978; 57:441.
- Smith AG, Wald J. Acute ventilatory failure in Lambert-Eaton myasthenic syndrome and its response to 3,4-diaminopyridine. Neurology 1996; 46:1143.
- Small S, Ali HH, Lennon VA, et al. Anesthesia for an unsuspected Lambert-Eaton myasthenic syndrome with autoantibodies and occult small cell lung carcinoma. Anesthesiology 1992; 76:142.
- Brown JC, Charlton JE. A study of sensitivity to curare in myasthenic disorders using a regionaltechnique. J Neurol Neurosurg Psychiatry 1975; 38:27.
- Weingarten TN, Araka CN, Mogensen ME, et al. Lambert-Eaton myasthenic syndrome during anesthesia: a report of 37 patients. J Clin Anesth 2014; 26:648.
- PREOPERATIVE EVALUATION
- Prediction of postoperative myasthenic crisis
- - Risk factors
- - Preoperative pulmonary evaluation
- Myasthenia gravis treatments
- - Anticholinesterase agents
- - Glucocorticoids
- - Immunotherapy
- - Rapid immunomodulating therapy
- ANESTHESIA MANAGEMENT
- Choice of anesthetic technique
- Induction and maintenance of anesthesia
- Neuromuscular blocking agents (NMBAs)
- - Depolarizing NMBAs
- - Nondepolarizing NMBAs
- - Reversal of NMBAs
- Medications that may exacerbate myasthenia gravis
- POSTOPERATIVE CONSIDERATIONS FOR PATIENTS WITH MYASTHENIA GRAVIS
- Myasthenic crisis
- Cholinergic crisis
- OBSTETRIC ANESTHESIA
- LAMBERT-EATON MYASTHENIC SYNDROME
- SUMMARY AND RECOMMENDATIONS