Anaesthesia and the Kearns‐Sayre syndrome

Estes, Richard C.; Ginsberg, Brian; Bloch, Edmond C.

doi:10.1111/j.1460-9592.1993.tb00094.x

Cited by 6 publications

(4 citation statements)

References 16 publications

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“…Lauwers et al . ( 20) has recommended that isoflurane is the agent of choice for this purpose in the Kearns–Sayre syndrome, as rhythm disturbances have been reported with halothane in this subset of patients ( 21). Indeed, in this particular subset, it has been recommended that an isoprenaline infusion and equipment necessary for artificial pacing be readily available ( 20).…”

Section: Introductionmentioning

confidence: 99%

Anaesthesia and mitochondrial disease

Wallace

Perndt

Skinner

1998

Pediatric Anesthesia

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Mitochondrial diseases, or encephalomyopathies, are an uncommon, heterogeneous group of disorders with variable clinical course and presentation. Many of these patients present for surgery, or undergo anaesthesia in the course of investigation of their illness. Unfortunately, little information exists on their management in anaesthetic texts and the literature. We report on the anaesthetic management of a paediatric patient with mitochondrial disease, and briefly discuss the pathophysiology and anaesthetic implications of these disorders.

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Section: Introductionmentioning

confidence: 99%

Anaesthesia and mitochondrial disease

Wallace

Perndt

Skinner

1998

Pediatric Anesthesia

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“…The unexpected high rate of myopathy (70"/0) found in the muscle biopsies, taken from 17 orthopaedic patients, apparently without overt neuromuscular disorder, supports our belief that precautions should always be taken in pacdiatric patients presenting for clubfoot surgery, to prevent MH related problems. We believe that regional anaesthesia techniques and complete monitoring should be preferentially utilized, when possible, as well as in patients with idiopathic neuromuscular scoliosis, where a number of other neuromuscular conditions may occur such as rigid spine syndrome, Duchenne or Becker dystrophy, Friedreich's ataxia, spinal muscular atrophy and congenital myopathies Estes, Ginsberg & Bloch 1993;. Anaesthesia should avoid exposure to triggering anaesthetics, succinylcholine and halogenated gases.…”

Section: Discussionmentioning

confidence: 99%

Prevalence of unsuspected myopathy in infants presenting for clubfoot surgery

Zanette

Manani

Pittoni

et al. 1995

Pediatric Anesthesia

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The objective of this study was an evaluation of the prevalence of myopathies in paediatric patients scheduled for orthopaedic surgery (clubfoot) performed under regional anaesthesia. Seventeen infants scheduled for lower limb orthopaedic surgery were studied to verify coexisting neuromuscular disorders with electromyography and muscle biopsy during surgery. All surgical procedures were performed under caudal block or spinal anaesthesia, associated with light general anaesthesia. No major cardiorespiratory, neurological or malignant hyperthermic complications (muscle rigidity, arrhythmias, hyperpyrexia) were observed. Combined neurological, electromyographic and biopsy studies showed a high rate of myopathic changes (70%). Performance of clubfoot surgery under light general anaesthesia with regional techniques was free from any problems. The high rate of myopathic changes (70%) observed in the muscle biopsies suggests that precautions should be taken with paediatric patients for clubfoot surgery and a regional anaesthesia technique with adequate monitoring may be helpful to prevent possible malignant hyperthermia related problems.

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“…Therefore, we did not use any neuromuscular blockade and selected sevoflurane for induction and maintenance of anesthesia. Although arrhythmias with halothane have been reported in patients with mitochondrial myopathies [ 5 ], sevoflurane has been used uneventfully in patients with these diseases [ 3 ]. Its low solubility and low pungency allow a rapid, smooth inhalational induction without the need for neuromuscular blocking drugs.…”

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confidence: 99%