LAUSR

Methylmalonic acidemia (MMA) occurs due to the deficiency of methylmalonyl-CoA mutase, which converts methylmalonyl coenzyme A (CoA) to succinyl-CoA, leading to the accumulation of methylmalonic acid.[1] There is also an associated deficiency of propionyl-CoA carboxylase resulting in the accumulation of propionic acid. This leads to a state of acute metabolic decompensation with acidemia and hyperammonemia, and clinically, patients present with vomiting, seizures, developmental delay, mental retardation and renal failure.[1] Anaesthetic challenges include avoiding excessive fasting, which can initiate protein catabolism leading to metabolic decompensation, adequate depth of sedation, maintenance of euvolaemia and providing enough energy to meet the metabolic demand. Apart from this, careful selection of anaesthetic drugs is important to have an uneventful outcome.[2] We present a 1-year, 8-month-old female child, weighing 7.8 kg, diagnosed with MMA. She received sedation with a combination of ketamine and dexmedetomidine (keta-dex) for magnetic resonance imaging (MRI) of the brain for the evaluation of global developmental delay. Previously, she was admitted with hyperammonaemia, hyperlactatemia and high anion gap metabolic acidosis and was metabolically optimised with a low protein diet, l-carnitine, hydroxycobalamine, lactulose, sodium benzoate, biotin and sodium bicarbonate therapy.