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• W1987482783 abstract "With the advent of modern technology, new diseases are described. As patients with these ‘new’ conditions may require surgical procedures, the anaesthetic implications need to be addressed [1]. Mitochondrial myopathies are disorders of fatty acid utilisation or mitochondrial function that may affect skeletal muscle, or defective aerobic metabolism that effect several organ systems. The CNS is often affected. Other organs affected include the heart, liver, kidney, endocrine glands and gastro-intestinal tract [2]. We present a 61-year-old woman, weight 69 kg, height 160 cm, scheduled for breast biopsy. Her family history included maternal myasthenia gravis. Her medical history included hypothyroidism of unknown aetiology treated with thyroxine 50 mg daily, and deafness that needed a tympanoplasty. She was diagnosed as having progressive external ophthalmoplegia (PEO) 2 months before surgery because of recent and progressive bilateral ptosis. Muscular biopsy showed ragged red fibres and subsarcoleme lipofucsine granules. Lactic acid levels were high after exercise and 55 min after cessation. No therapeutic recommendations were made. Pre-operative haematology, biochemistry and ECG were normal. We planned an anaesthetic technique avoiding triggers for malignant hyperthermia (MH), potential respiratory complications and with the initial plan of keeping the patient in an ambulatory surgery setting. Monitoring was: ECG, non-invasive blood pressure (NIBP), pulse oximetry, capnography and oesophageal temperature. Dantrolene was immediately available. The ventilator was previously purged with high flow oxygen. An intravenous saline infusion was started. Pre-anaesthetic medication consisted of i.v. midazolam 1.5 mg, followed by a remifentanil infusion rate of 0.25 μg.kg −1.min−1 Induction was performed with propofol 140 mg and a laryngeal mask size 3 was inserted. Anaesthesia was maintained with a mixture of oxygen and nitrous oxide with a remifentanil infusion and assisted manual ventilation until spontaneous ventilation started after 5 min. Duration of surgery was 40 min and the remifentanil infusion and nitrous oxide were discontinued at the completion of skin suturing. The patient awoke within 5 min. The total dose of remifentanil was 0.39 mg. At the end of surgery the patient was transferred to the recovery unit for 1 h and discharged home 6 h later. Mitochondrial myopathies (MM) are classified according to their histological appearance, inmunochemical and biochemical profile or genetic analysis. The histological hallmark of MM is the ragged red fibre. PEO is associated with sporadic deletions of DNA. A negative family history indicates that deletion occurs in embryogenesis. Age of presentation ranged from 47 to 82 years in a nine-case series [3], with asymmetrical ptosis as the commonest sign, as in our patient. In this series, CPK was normal in eight cases and myopathic changes were found in three cases. The clinical course is usually progressive and diabetes mellitus, hypothyroidism, hyperparathyroidism and short stature are often associated. The anaesthetic implication in MM and PEO is an increased susceptibility for MH, which has been described in at least one case. This requires a non- triggering anaesthetic technique. This can be addressed with propofol and remifentanil infusions [4, 5]. Depressed ventilatory response to hypercarbia or hypoxia, independent of muscle weakness or sleep apnoea, has been described in patients with various MMs including PEO [2]. Marked sensitivity to intravenous induction agents, opiates or sedative-hypnotics may be present and weaning difficulty has been described. A few cases of pregnancy and other surgical procedures successfully managed with regional anaesthesia have been described in patients with MM [6]. Ambulatory care surgery is possible in PEO patients, as possible for many other conditions whose MH susceptibility is low and a non-triggering technique is used. Total intravenous anaesthesia (TIVA) based on propofol and alfentanil has been used successfully for eye surgery [7], or ketamine with fentanyl for cardiac surgery [8]. The use of ambulatory care surgery continues to grow and increases the likelihood of encountering MH-susceptible patients or of experiencing a malignant hyperthermia crisis. The management of this population creates new challenges A comprehensive plan of patient care under these circumstances includes the ability to identify the high-risk patient and to plan their care accordingly, early recognition of the signs and symptoms of MH, and being prepared to promptly and efficiently treat a malignant hyperthermic event [9]. MM is an heterogeneous group of rare conditions that will need to be anaesthetised more often in future and we believe that new anaesthetic drug combinations offer the patient safe anaesthesia with low MH risk and allow ambulatory surgery to be possible." @default.
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• W1987482783 date "2003-05-20" @default.
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• W1987482783 title "Progressive external ophthalmoplegia and ambulatory remifentanil-propofol based anaesthesia" @default.
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• W1987482783 doi "https://doi.org/10.1046/j.1365-2044.2003.32191.x" @default.
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