Perioperative care of an infant with pyruvate dehydrogenase deficiency

Abstract

The authors present the anaesthetic management of two infants with pyruvate dehydrogenase complex deficiency (PDCD), a rare genetic disorder of carbohydrate metabolism leading to lactic acidosis and neurological impairment. In the first case, a seven-month-old infant, undergoing closed reduction of a dislocated hip, received general anaesthesia with a volatile agent. In the second case, spinal anaesthesia was administered to a six-month-old infant undergoing Achilles tendon lengthening. There were no adverse outcomes in both cases. Key components of perioperative care included minimising perioperative stress, and avoiding exacerbation of the lactic acidosis. Previous reports regarding the perioperative care of such patients are reviewed, and recommendations for anaesthetic care discussed.