Craniotomy Complicated by Severe Metabolic Acidosis Requiring Massive Transfusion in an Infant on Ketogenic Diet Therapy for Intractable Epilepsy

The induction of a ketotic state through dietary manipulation, known as the ketogenic diet (KD), is an alternative or supplementary treatment to drug-resistant epilepsy. By sustaining a ketogenic state, the KD results in various biological adaptations which contribute to its success as an anti-seizure therapy. While the induction and maintenance of ketosis generally results in only a low-grade metabolic acidosis, various exogenous stresses such as surgery and anesthetic care may disrupt homeostasis resulting in exaggerated ketosis and severe metabolic acidosis. Metabolic acidosis may have significant effects on various physiologic functions including cardiovascular performance, coagulation function, and electrolyte balance. We present a 7-month-old patient receiving a KD who presented for craniotomy and resection of an epileptogenic focus. During intraoperative care, progressive acidosis and hyperchloremia were noted with ongoing tissue fragility and hyperemia, parenchymal friability, and coagulopathy. Though the acidosis was temporarily blunted by administration of sodium bicarbonate and a change to sodium acetate containing fluids, ultimately poor hemostasis resulted requiring significant blood product transfusion. The metabolic effects of the KD are reviewed with emphasis on acid-base disturbances and impact on coagulation function.