Diagnostic study, level III. Prognostic study, level III.

Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency is an X-linked recessive enzymopathy responsible for acute haemolysis following exposure to oxidative stress. Drugs which induce haemolysis in these patients are often used in anaesthesia and perioperative pain management. Neurosurgery and few drugs routinely used during these procedures are known to cause stress situations. Associated infection and certain foodstuffs are also responsible for oxidative stress. Here, we present two patients with G-6-PD deficiency who underwent uneventful neurosurgical procedures. The anaesthetic management in such patients should focus on avoiding the drugs implicated in haemolysis, reducing the surgical stress with adequate analgesia, and monitoring for and treating the haemolysis, should it occur.