Neuropediatrics 2004; 35(4): 250-252
DOI: 10.1055/s-2004-820992
Short Communication

Georg Thieme Verlag KG Stuttgart · New York

Fatal Propofol Infusion Syndrome in Association with Ketogenic Diet

F. A. M. Baumeister1 , R. Oberhoffer1 , G. M. Liebhaber1 , J. Kunkel1 , J. Eberhardt2 , H. Holthausen3 , J. Peters1
  • 1Children's Hospital of the Technical University Munich, Munich, Germany
  • 2Department of Anesthesiology, Hospital of Vogtareuth, Germany
  • 3Department of Neuropediatrics, Hospital of Vogtareuth, Germany
Further Information

Publication History

Received: August 11, 2003

Accepted after Revision: April 8, 2004

Publication Date:
10 August 2004 (online)

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.

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Priv. Doz. Dr. F. A. M. Baumeister

Kinderklinik und Poliklinik der Technischen Universität München
Kinderklinik Schwabing

Kölner Platz 1

80804 München

Germany

Email: FAM.Baumeister@lrz.uni-muenchen.de