1/5. General anaesthesia in a man with mitochondrial myopathy undergoing eye surgery.mitochondrial myopathies are rare complex multisystem disorders, which can present several potential anaesthetic problems. A 49-year-old man with mitochondrial myopathy was anaesthetized successfully for cataract extraction using propofol and alfentanil infusions. Vecuronium sensitivity was apparent, with a prolonged duration of action, despite dosage reduction.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
2/5. Anaesthetic considerations for a child with combined prader-willi syndrome and mitochondrial myopathy.We report the anaesthetic management of a child with prader-willi syndrome and mitochondrial myopathy for open heart surgery. We used ketamine, fentanyl, rocuronium and caudal morphine together with a propofol infusion with no untoward effects. The implications of both conditions for anaesthesia are discussed.- - - - - - - - - - ranking = 0.25keywords = anaesthesia (Clic here for more details about this article) |
3/5. Anaesthetic management of labour and delivery in the parturient with mitochondrial myopathy.PURPOSE: We describe the anaesthetic management for Caesarean section in a parturient with a defect in complex III of the respiratory chain who had increased lactate concentrations at rest and with exercise. CLINICAL FEATURES: We administered effective epidural anaesthesia with lidocaine for Caesarean delivery. The serum lactate concentration was less than the preoperative value both during and after surgery. shivering during the perioperative period was avoided by administering warm i.v. fluids, warm local anaesthetic solution and epidural meperidine. Pain relief after surgery was provided with i.v. PCA morphine augmented by local infiltration with bupivacaine to fascia and skin edges and epidural injection with meperidine. CONCLUSION: mitochondrial myopathies are an uncommon group of disorders in which mitochondrial dysfunction leads to clinical disease of muscle and sometimes of other organs with high energy requirements. The management of labour and delivery in women with mitochondrial myopathies should be individualized according to severity of disease and formulated by consultation between attending physicians and the anaesthetist. Epidural analgesia reduces stress and work associated with labour and reduces oxygen demand during labour. However, parturients with defects of the respiratory chain with documented increased lactate concentrations at rest and with exercise are best managed with elective Caesarean delivery with regional anaesthesia to prevent life-threatening lactic acidosis during labour. The association between malignant hyperthermia and these disorders has not been proved, but it appears prudent to consider these women as MH susceptible until definitive data regarding this possible relationship are available.- - - - - - - - - - ranking = 0.5keywords = anaesthesia (Clic here for more details about this article) |
4/5. mitochondrial myopathies: an unusual cause of hypotonia in infants and children.Children frequently undergo muscle biopsy for the workup of hypotonia under general anaesthesia which poses unique risks in patients with undiagnosed muscle disease. mitochondrial myopathies are a relatively newly recognized cause of myopathy and multisystem disease in both adults and children. The diagnosis is complex. In addition to causing myopathy, there are metabolic derangements present in some cases that may be life-threatening. We present three cases of children with hypotonia where the diagnosis was suspected in two patients, and confirmed in the third. The question of whether patients with mitochondrial myopathies are at increased risk for developing malignant hyperthermia is discussed.- - - - - - - - - - ranking = 0.25keywords = anaesthesia (Clic here for more details about this article) |
5/5. Increased sensitivity to rocuronium and atracurium in mitochondrial myopathy.PURPOSE: To describe the prolonged effect of the intermediate-acting, non-depolarising neuromuscular blocking agents rocuronium and atracurium in a 29-yr-old apparently healthy woman. CLINICAL FEATURES: Because of abdominal pain the patient was scheduled for explorative laparoscopic pelvic examination. General anaesthesia was induced with fentanyl, midazolam and propofol. muscle relaxation was achieved with 0.6 mg.kg-1 rocuronium. Anaesthesia was maintained with nitrous oxide and propofol. Two Hz train-of-four stimulation every 15 sec evoked no twitch responses until 60 min after rocuronium. Further relaxation was achieved with 0.075 mg.kg-1 atracurium after which twitch responses recurred after 45 min. Fifteen minutes later neuromuscular blockade was successfully reversed with atropine and neostigmine. The postanaesthetic course was uneventful. Because of the increased sensitivity to rocuronium and atracurium the patient was re-evaluated postoperatively. history revealed occasional double vision, fatigue, muscle cramps, stiffness and myoglobinuria. Clinical neurological examination showed ptosis, tremor, ataxia and bradydiadochokinesia. A standardised lactate stress testing on a bicycle was pathological and, after muscle biopsy, the diagnosis of mitochondrial myopathy was established. CONCLUSION: An increased sensitivity to rocuronium and atracurium may occur in patients with mitochondrial myopathy. In these patients appropriate dosing of muscle relaxants and adequate monitoring of the neuromuscular blockade are required. If an increased sensitivity to rocuronium and atracurium occurs in an apparently healthy subject, further neurological investigations should follow.- - - - - - - - - - ranking = 0.25keywords = anaesthesia (Clic here for more details about this article) |