Mitochondrial disorders and general anaesthesia: a case series and review.

Patients with mitochondrial disease are at risk of metabolic decompensation and often require general anaesthesia (GA) as part of their diagnostic work up and subsequent management. However, the evidence base for the use of GA is limited and inconclusive. We have documented the practice and outcome in the use of GA in paediatric patients with mitochondrial disease using a retrospective case review study of 38 mitochondrial patients who had undergone 58 anaesthetics within the regional metabolic service for the period 1989-2005. A variety of anaesthetic agents were used and the pattern of use reflects that seen in standard paediatric practice. There were no episodes of malignant hyperthermia and no documented intraoperative events attributable to the GA. Three postoperative adverse events were noted; one episode of hypovolaemia, one episode of acute on chronic renal failure, and one episode of metabolic decompensation 12 h post-muscle biopsy. Despite theoretical concern about this group of patients, adverse events after GA are rare and in most cases unrelated to the anaesthesia. Further prospective studies of GA in mitochondrial disease are required to create evidence-based clinical guidelines for safe practice.

High-frequency oscillation in adolescents.

BACKGROUND: High-frequency oscillation (HFO) is a widely used lung-protective ventilatory strategy in paediatric and neonatal acute lung injury. Its safe and effective use has been hindered by inadequate recruitment of the lung during oscillation and, until recently, the lack of an adequately powered oscillator for use in adult practice. METHODS: We present data from three adolescents with severe acute respiratory distress syndrome (ARDS) who received HFO with the Sensormedics 3100B oscillator after failure of conventional mechanical ventilation. A manual recruitment manoeuvre was used in all patients prior to mechanical ventilation (conventional or HFO) and following tracheal suctioning or disconnection from the ventilator. Changes in oxygenation index were used to assess therapy. RESULTS: All patients showed at least a 25% reduction in oxygenation index within 2 h of HFO, with return to conventional ventilation after 27-65 h. CONCLUSIONS: We found HFO, in conjunction with manual recruitment and prone positioning, to be a well-tolerated mode of ventilation in adolescents with ARDS and who were unresponsive to conventional ventilation. Given this success we hope to renew interest in this method for adults with ARDS, together with concurrent use of manual recruitment.