Case of glucose 6 phosphate dehydrogenase deficiency: Anaesthesia challenges and management

Dr Reshma Shenoy; Dr Usha Badole; Dr Sujata Priyadarshini

Volume : VII, Issue : VI, June - 2018

Case of glucose 6 phosphate dehydrogenase deficiency: Anaesthesia challenges and management

Dr Reshma Shenoy, Dr Usha Badole, Dr Sujata Priyadarshini

Abstract :

Glucose –6–phosphate dehydrogenase (G6PD) deficiency is an X linked recessive inborn error of metabolism causing acute haemolysis following exposure to oxidative stress. Drugs used for anaesthesia and perioperative pain management can induce haemolysis.5 years old male child with G6PD deficiency was admitted to our paediatric surgery services for urethrostomy. Patient was a diagnosed case of double urethra on cystoscopy done at another institute.All routine investigations were within normal limits . The child was premedicated with glycopyrrolate and midazolam. Induction was done with Fentanyl, Propofol and Atracurium. Anaesthesia was maintained with O2, air and sevoflurane and intermittent boluses of Fentanyl and Atracurium. At the end of the procedure, the child was reversed and extubated on table. Postoperative analgesia was given with Tramadol. Postoperative Haemoglobin done on day 2 was normal.Anaesthesia management was focused on avoiding the triggering factors for oxidative stress which can lead to haemolysis. This was achieved with good antibiotic coverage perioperatively and reducing the surgical stress with adequate anxiolysis and analgesia. We also avoided oxidative drugs and precipitants of methaemoglobinemia. Monitoring and management of acute haemolysis was also a major concern in case it occurred.