Methyl malonyl coenzyme A mutase deficiency is a rare autosomal inherited inborn error in branched-chain amino acid metabolism characterised by the accumulation of methylmalonic acids. There is relative paucity of literature regarding anaesthetic management of these children presenting for incidental major abdominal surgery. Preoperative management includes goal-directed correction of dehydration, metabolic acidosis and hyperammonemia. Anaesthetic goals include avoidance of factors that can trigger metabolic crisis like hypercapnia, hypothermia, hypoxia, surgical stress, hypovolaemia, hypotension and so on. Herein, we are reporting the anaesthetic management of a 17-month-old child with methylmalonic acidemia (MMA) posted for a major upper abdominal surgery for excision of an adrenal mass, which was incidentally diagnosed during admission for an episode of metabolic crisis. We aim to highlight the specific nuances of pathophysiology of the disease, preoperative optimisation, anaesthetic considerations, role of advanced monitoring and regional anaesthesia and current literature on the management of patients with MMA.
LMA CTrachTM , a newer airway conduit, with its ability to ventilate and intubate is the ideal device for airway management for difficult airways. A retrospective study was conducted to evaluate the efficacy of this airway device in patients with anticipated difficult airway. 250 patients in whom LMA CTrach TM was used for endotracheal intubation was analyzed. These patients were analyzed for anticipated difficult airway, success rate of intubation with LMA CTrach TM , difficulties encountered and corrective measures applied. Fifty five patients had predictors of difficult airway and LMA CTrach TM was successful in all but one. Although successful intubation was achieved with LMA CTrach TM , difficulty in intubation was encountered in six patients. This could be overcome with excessive pressure, correctional manoeuvers or a larger size LMA CTrach TM . The most common predictors of difficult airway encountered were Modified Mallampati class 3, restricted extension, restricted mouth opening of two finger breadths and retrognathia. LMA CTrach TM is a useful airway device in a difficult airway scenario with the ability to ventilate and intubate the patient.
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