Desflurane anaesthesia in myotonic dystrophy

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Abstract

Myotonic dystrophy (MD) is a rare genetic disorder with multisystem involvement characterised by myotonia and progressive muscle weakness and wasting. These patients pose significant challenges to the anaesthesiologist in view of the muscular and extramuscular involvement and sensitivity to anaesthetic drugs. The literature is replete with reports of postanaesthetic respiratory and cardiovascular complications in these patients. But an ideal anaesthetic technique in MD patients remains to be determined. Rapid recovery is desirable to reduce postoperative respiratory complications. Though there are a few case reports of maintenance of anaesthesia with isoflurane and sevoflurane, there are scanty reports of use of desflurane in these patients. We present successful management of a patient with MD for laparoscopic cholecystectomy using a carefully titrated desflurane-based anaesthesia and discuss the perioperative considerations.

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The myotonias and susceptibility to malignant hyperthermia.

Jerome Parness, Oliver Bandschapp, Thierry Girard (2009)

Malignant hyperthermia (MH) is a pharmacogenetic disorder of skeletal muscle in which volatile anesthetics trigger a sustained increase in intramyoplasmic Ca(2+) via release from sarcoplasmic reticulum and, possibly, entry from the extracellular milieu that leads to hypermetabolism, muscle rigidity, rhabdomyolysis, and death. Myotonias are a class of myopathies that result from gene mutations in various channels involved in skeletal muscle excitation-contraction coupling and sarcolemmal excitability, and unusual DNA sequence repeats that result in the inability of many proteins, including skeletal muscle channels that affect excitability, to undergo proper splicing. The suggestion has often been made that myotonic patients have an increased risk of developing MH. In this article, we review the physiology of muscle excitability and excitation-contraction coupling, the pathophysiology of MH and the myotonias, and review the clinical literature upon which the claims of MH susceptibility are based. We conclude that patients with these myopathies have a risk of developing MH that is equivalent to that of the general population with one potential exception, hypokalemic periodic paralysis. Despite the fact that there are no clinical reports of MH developing in patients with hypokalemic periodic paralysis, for theoretical reasons we cannot be as certain in estimating their risk of developing MH, even though we believe it is low.

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Risk factors for perioperative adverse events in children with myotonic dystrophy.

James Sinclair, P Reed (2009)

This study was conducted to identify patient-related, surgical, and anesthetic factors that would help predict adverse events and allow for better planning of perioperative care in children with myotonic dystrophy. This is a retrospective chart review from a large tertiary pediatric hospital. Data were collected on demographics, disease severity, surgical procedure, and anesthetic technique. Perioperative adverse events were recorded. Records on 27 patients having 78 anesthetics over a 17.5-year period were reviewed. The overall frequency of postoperative respiratory complications was 10%. Significant risk factors were high muscular impairment rating scale (MIRS) grade (P = 0.007), at least 2300 cytosine, thymine, guanine (CTG) repeats on the protein kinase gene of chromosome 19q (P = 0.009), a longer duration of surgery (RR = 14.0 for surgery lasting at least 1 h; P = 0.002), perioperative morphine use (RR = 7.7, 95% CI 2.2-12.8; P = 0.005), intubation (P = 0.02), and the use of muscle relaxant without reversal (RR = 15.5, P = 0.0002). Using a multivariate risk model, only MIRS grade and the use of muscle relaxant without reversal were shown to be significant independent risk factors (RR = 24.9, P < 0.0001). The MIRS is a statistically significant and clinically useful tool for predicting high perioperative risk. Patients with a high MIRS grade should therefore be considered for postoperative intensive care. The use of muscle relaxant without reversal was also shown to be a significant risk factor. Patients who require morphine infusions postoperatively might also be most safely managed in a high dependency unit.

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Anaesthetic problems in myotonic dystrophy. A case report and review of the Aberdeen experience comprising 48 general anaesthetics in a further 16 patients.

Cameron Aldridge (1985)

A previously undiagnosed case of myotonic dystrophy presenting with apnoea of 2.5 h duration following thiopentone is described. A review of the anaesthetic outcome from 49 operations in 17 patients with myotonic dystrophy in the Aberdeen area is presented. The type of operation and intra- and postoperative problems are analysed. The results reveal a 52% complication rate in previously diagnosed cases and a 35% complication rate in undiagnosed cases. In the series, 29% of the anaesthetics were administered to symptomatic patients before formal diagnosis. To avoid potential hazards it behoves the anaesthetist to remain alert to the possibility of the undiagnosed disease. The symptomatology and associated findings of the 17 patients at initial diagnosis are presented. The literature has been reviewed and anaesthetic implications noted.

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Author and article information

Journal

Journal ID (nlm-ta): Indian J Anaesth

Journal ID (publisher-id): IJA

Title: Indian Journal of Anaesthesia

Publisher: Medknow Publications (India )

ISSN (Print): 0019-5049

ISSN (Electronic): 0976-2817

Publication date (Print): Jan-Feb 2011

Volume: 55

Issue: 1

Pages: 61-63

Affiliations

Department of Anaesthesiology, Pain and Perioperative Medicine, Sir Ganga Ram Hospital, New Delhi, India

Author notes

Address for correspondence: Dr. Ranju Gandhi, C-1/1065, Vasant Kunj, New Delhi - 110070, India E-mail: icu_era@ 123456yahoo.co.in

Article

Publisher ID: IJA-55-61

DOI: 10.4103/0019-5049.76599

PMC ID: 3057249

PubMed ID: 21431056

SO-VID: 33086c66-7857-4093-982c-278b8dbec3e4

License:

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

Desflurane anaesthesia in myotonic dystrophy

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Abstract

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Irish Journal of Paramedicine

Most cited references 13

The myotonias and susceptibility to malignant hyperthermia.

Risk factors for perioperative adverse events in children with myotonic dystrophy.

Anaesthetic problems in myotonic dystrophy. A case report and review of the Aberdeen experience comprising 48 general anaesthetics in a further 16 patients.

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