Peri-operative management of an adult with POLG-related mitochondrial disease

Area of Science:

• Anesthesiology
• Mitochondrial Medicine
• Genetics

Background:

• POLG-related mitochondrial disease is a rare disorder associated with potential anesthetic risks, including propofol-infusion syndrome.
• Patients with mitochondrial DNA deletions and POLG mutations require careful anesthetic management.

Observation:

• A 19-year-old male with POLG-related mitochondrial disease underwent robotic Heller-Dor myotomy.
• Anesthesia involved a minimized fasting period, rapid sequence induction with propofol, remifentanil-sevoflurane general anesthesia, and lactate-free IV fluids with dextrose.
• The patient did not develop propofol-infusion syndrome but experienced delayed extubation due to residual neuromuscular blockade.

Findings:

• Single-use, low-dose propofol was safely administered to this patient with POLG-related mitochondrial disease.
• Delayed tracheal extubation occurred, necessitating sugammadex administration for reversal of neuromuscular blockade.

Implications:

• This case suggests that propofol can be a safe anesthetic agent in select patients with POLG-related mitochondrial disease.
• Close monitoring of neuromuscular blockade is crucial in these patients to prevent prolonged effects and ensure timely extubation.
• Anesthesiologists should consider potential prolonged neuromuscular blockade when managing patients with POLG-related mitochondrial disorders.