Fatal Propofol Infusion Syndrome in Association with Ketogenic Diet

Abstract

Propofol is used for the treatment of refractory status epilepticus. When given as a long-term infusion propofol may cause a rare but frequently fatal complication, the propofol infusion syndrome. The hallmarks are metabolic acidosis, lipemia, rhabdomyolysis and myocardial failure. Propofol infusion syndrome is caused by impaired fatty acid oxidation. Beside anticonvulsants the ketogenic diet, a high-fat, low-carbohydrate, adequate-protein diet, is an effective treatment for difficult-to-control seizures. We report a 10-year-old boy with catastrophic epilepsy, who developed fatal propofol infusion syndrome when a ketogenic diet was initiated. Substances like propofol which impair fatty acid oxidation may pose an increased risk if combined with ketogenic diet.

Key words

Propofol - ketogenic diet - arrhythmia - rhabdomyolysis - hyperlipidemia

References

• 1 Best T H, Franz D N, Gilbert D L, Nelson D P, Epstein M R. Cardiac complications in pediatric patients on the ketogenic diet.  Neurology. 2000;  54 2328-2330
• 2 Bonnet D, Martin D, de Lonlay P, Villain E, Jouvet P, Rabier D. et al . Arrhythmias and conduction defects as presenting symptoms on fatty acid oxidation disorders in children.  Circulation. 1999;  100 2248-2253
• 3 Bray R J. Propofol infusion syndrome in children.  Paediatric Anaesthesia. 1998;  8 491-499
• 4 Cornfield D N, Tegtmeyer K, Nelson M D, Milla C E, Sweeney M. Continuous propofol infusion in 142 critically ill children.  Pediatrics. 2002;  110 1177-1181
• 5 Cremer O L, Moons K GM, Bouman E AC, Kruijswijk J E, de Smet A MGA, Kalkman J. Long-term propofol infusion and cardiac failure in adult head-injured patients.  Lancet. 2001;  357 117-118
• 6 Kang T M. Propofol infusion syndrome in critically ill patients.  Ann Pharmacother. 2002;  36 1453-1456
• 7 Kottlors M, Jaksch M, Ketelsen U-P, Weiner S, Glocker F X, Lücking C-H. Valproic acid triggers acute rhabdomyolysis in a patient with carnitine palmitoyltransferase type II deficiency.  Neuromuscular Disord. 2001;  11 757-759
• 8 Lefevre F, Aronson N. Ketogenic diet for the treatment of refractory epilepsy in children: A systematic review of efficacy.  Pediatrics. 2000;  105 1-7
• 9 Lowenstein D H, Alldredge B K. Status epilepticus.  N Engl J Med. 1998;  338 970-976
• 10 Stelow E B, Johari V P, Smith S A, Crosson J T, Apple F S. Propofol-associated rhabdomyolysis with cardiac involvement in adults: chemical and anatomic findings.  Clin Chem. 2000;  46 577-581
• 11 Wolf A, Weir P, Segar P, Stone J, Shield J. Impaired fatty acid oxidation in propofol infusion syndrome.  Lancet. 2001;  357 606-607