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      Myotonic Dystrophy and Anesthetic Challenges: A Case Report and Review

      case-report
      Author(s): , ,
      Publication date (Electronic):
      Journal: Case Reports in Anesthesiology
      Publisher: Hindawi

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          Abstract

          Providing anesthesia to patients with myotonic dystrophy (DM) can be very challenging due to the multisystemic effects of the disease and extreme sensitivity of these patients to sedatives, opioids, and anesthetic agents. Other factors such as hypothermia, shivering, or mechanical or electric stimulation during surgery can precipitate myotonia which is difficult to abolish and can lead to further complications. Generally, local or regional anesthesia is preferred to avoid the complications associated with general anesthesia in this group. However there are several case reports of successful use of general anesthesia (with or without volatile agents and with or without opioids). These general anesthetic cases led to postoperative admission to the regular floor or ICU. We present a case of a woman with a history of DM who underwent robotic assisted laparoscopic hysterectomy under general anesthesia and was discharged home on the same day.

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          Inhibition of adrenal steroidogenesis by the anesthetic etomidate.

          Irene P Kan, Aaron P White, Kathryn Wagner … (1984)
          The use of the intravenous anesthetic etomidate for prolonged sedation has been associated with low levels of plasma cortisol and increased mortality. We measured the cortisol and aldosterone responses to ACTH stimulation in five patients receiving etomidate, and we also studied the direct effects of etomidate on enzymes in the rat steroidogenic pathway. One patient who was receiving a 20-hour infusion of etomidate (1.3 to 1.5 mg per kilogram of body weight per hour) had marked adrenocortical suppression that was still evident four days after etomidate was discontinued. Four surgical patients receiving etomidate during their operations were all found to have adrenal suppression four hours after the operation; mean (+/- S.D.) increases in cortisol and aldosterone after ACTH stimulation were only 1.8 +/- 0.5 micrograms per deciliter and 0.5 +/- 1.1 ng per deciliter, respectively. In rat adrenal cells, etomidate produced a concentration-dependent blockade of the two mitochondrial cytochrome P-450-dependent enzymes, cholesterol-side-chain cleavage enzyme, and 11 beta-hydroxylase, without evident inhibition of the microsomal enzymes in the glucocorticoid pathway. Physicians should be aware that etomidate inhibits adrenal steroidogenesis, and they should consider treating selected patients with corticosteroids if etomidate is used.
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            Anesthetic and surgical complications in 219 cases of myotonic dystrophy.

            M. Fusco Girard, M Braekeleer, G Gobeil … (1997)
            The objective of this study was to assess the frequency, type, and severity of perioperative complications after a first surgery under general anesthesia in patients with myotonic dystrophy (DM) and to measure the association with suspected risk factors. Numerous cases of perioperative complications in DM patients have been reported. Hazards have been associated with the use of thiopentone, suxamethonium, neostigmine, and halothane. A retrospective study of perioperative complications was conducted for 219 DM patients who had their first surgery under general anesthesia at the Chicoutimi Hospital. The overall frequency of complications was 8.2% (18 of 219). Most complications (16 of 18) were pulmonary, including five patients with acute ventilatory failure necessitating ventilatory support, four patients with atelectasis, and three patients with pneumonia. Using multivariate analysis, we found that the risk of perioperative pulmonary complications (PPC) was significantly higher after an upper abdominal surgery (odds ratio (OR), 24.4; 95% CI, 4.0 to 149.3) and for patients with a severe muscular disability, as assessed by the presence of proximal limb weakness (OR, 14.1; 95% CI, 1.5 to 134.4). The likelihood of PPC was not related to any specific anesthetic drug. Because of the increased risk of PPC, careful monitoring during the early postoperative period, protection of upper airways, chest physiotherapy, and incentive spirometry are mandatory in all symptomatic DM patients, particularly those with a severe muscular disability or those who have undergone an upper abdominal surgery.
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              Long-term follow-up of arrhythmias in patients with myotonic dystrophy treated by pacing: a multicenter diagnostic pacemaker study.

              Frederic Anselme, Jean Varin, Denis Duboc … (2002)
              We hypothesized that pacemaker (PM) implantation in patients with myotonic dystrophy (MD) with a prolonged HV interval, even asymptomatic, may protect them against sudden death related to atrioventricular (AV) block. We sought to prospectively document the true incidence of AV block episodes in this high-risk population and accurately trace, in the long term, by the PM, the occurrence of arrhythmias that may remain undetected during conventional follow-up. Myotonic dystrophy is associated with a high risk of sudden death, commonly attributed to AV block or ventricular arrhythmias, but cardiac pacing is only recommended as a secondary prevention. Patients with MD with an HV interval > or =70 ms, even in the absence of related symptoms, prospectively received a cardiac PM, including an algorithm capable of diagnosing episodes of bradycardia and tachyarrhythmias. The population consisted of 49 patients (45.5 +/- 8.9 years old) followed for 53.5 +/- 27.2 months. Paroxysmal arrhythmias were recorded in 41 patients (83.7%), consisting of complete AV block (n = 21), sino-atrial block (n = 4), or atrial (n = 25) or ventricular (n = 13) tachyarrhythmias. No patient died of AV block during follow-up, but 10 deaths occurred, 4 of them sudden. An arrhythmic cause could be excluded by postmortem PM interrogation in two cases of typical sudden death. Arrhythmias are common in patients with MD with infrahisian conduction abnormalities. The prophylactic implantation of a pacing system when the HV interval is > or =70 ms seems appropriate. The PM protects the patient against the clinical consequences of paroxysmal profound bradycardia and facilitates the diagnosis and management of frequent paroxysmal tachyarrhythmias.
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                Author and article information

                Contributors
                Chanchal Mangla:
                ORCID: http://orcid.org/0000-0001-9129-737X
                Journal
                Journal ID (nlm-ta): Case Rep Anesthesiol
                Journal ID (iso-abbrev): Case Rep Anesthesiol
                Journal ID (publisher-id): CRIA
                Title: Case Reports in Anesthesiology
                Publisher: Hindawi
                ISSN (Print): 2090-6382
                ISSN (Electronic): 2090-6390
                Publication date Collection: 2019
                Publication date (Electronic): 20 March 2019
                Volume: 2019
                Electronic Location Identifier: 4282305
                Affiliations
                Department of Anesthesiology, New York Presbyterian Brooklyn Methodist Hospital, Brooklyn, New York, USA
                Author notes

                Academic Editor: Jian-jun Yang

                Author information
                http://orcid.org/0000-0001-9129-737X
                http://orcid.org/0000-0002-1448-9623
                Article
                DOI: 10.1155/2019/4282305
                PMC ID: 6446115
                PubMed ID: 31016049
                SO-VID: 615f8653-0212-45ea-966a-d1877baad9ef
                Copyright © Copyright © 2019 Chanchal Mangla et al.
                License:

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 5 December 2018
                Date revision received : 6 February 2019
                Date accepted : 6 March 2019
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Anesthesiology & Pain management
                Data availability:
                ScienceOpen disciplines: Anesthesiology & Pain management

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