Anaesthetic Management of a Child with Methylmalonic Acidemia Undergoing Bilateral Orchidopexy – A Case Report

Background: Methylmalonic Acidemia (MMA) is an inborn error of metabolism caused by a deficiency in methylmalonyl CoA mutase, leading to increased protein catabolism, hyperammonemia and metabolic acidosis. This condition presents challenges to the Anaesthesiologist for perioperative management due to metabolic instability, particularly during surgeries. Case Presentation: A 1-year-old male child, weighing 8.5 kg and diagnosed with MMA, was posted for bilateral orchidopexy for undescended testes. Anaesthetic management was focused on preventing hypoglycemia, managing surgical stress with good analgesia, and avoiding metabolic acidosis. L-carnitine and methylcobalamin were administered preoperatively. General anaesthesia with inhalational induction was given, and a pre-incision caudal block for analgesia was performed. The child recovered without any complications. Conclusion: We report a case of successful anaesthetic management of a child with MMA, emphasizing the importance of knowing the pathophysiology of the disease to prevent metabolic complications. Keywords: Methylmalonic acidemia, Bilateral orchidopexy, Pediatric anaesthesia, Metabolic disorder.