Mitochondrial diseases represent a wide range of disorders caused by impairment of mitochondrial metabolism and energy production. We report a case of an uneventful perioperative management of a pregnant patient with early childhood-onset mitochondrial encephalopathy, attributed to a novel AIFM1 (Apoptosis-Inducing Factor, Mitochondrion-associated 1) mutation. After a multidisciplinary team consultation, it was decided to be posted for elective caesarean delivery at 36+2 weeks of gestation, under titrated combined spinal-epidural anaesthesia and multimodal analgesia. Unique anaesthetic challenges were to ensure normoglycaemia, normothermia and adequate hydration while avoiding perioperative stress and acidosis. Continue reading