• Katerina Ruzkova, Toby N Weingarten, Kelly J Larson, Robert J Friedhoff, Dimitar K Gavrilov, and Juraj Sprung.
• Department of Anesthesiology, Mayo Clinic, Rochester, MN, USA.
• Paediatr Anaesth. 2015 Aug 1; 25 (8): 807-817.

BackgroundOrganic acidurias (OAs) are rare inborn errors of metabolism that can present with various neurologic manifestations, propensity for acute metabolic decompensation with anion-gap metabolic acidosis, developmental delay, poor feeding, and failure to thrive.ObjectiveIn this case series, we outline the anesthetic management and perioperative outcomes of OA patients.MethodsWe reviewed demographic characteristics, comorbidities, and perioperative course of patients with four different OAs who underwent anesthetic care at our institution between January 1, 2000, and December 31, 2013.ResultsEleven patients with OA underwent 19 anesthetic procedures, of which 13 were <2 h in duration and seven were outpatient procedures. One patient with methylmalonic acidemia developed metabolic acidosis during a 10-h procedure with substantial blood loss but lacked evidence that this acidosis could be attributed to his underlying metabolic disease. The patients who received hydration with lactated Ringer's solution and/or nitrous oxide anesthetic had a perioperative course free of metabolic complication. Two patients died within 30 days of surgery from causes likely to be unrelated to anesthetic exposure.ConclusionsOur patients with various forms of metabolically compensated OAs tolerated anesthetics for surgical procedures without metabolic decompensation, even when lactated Ringer's solution was used for hydration. Measures to prevent protein catabolism and intraoperative events that may precipitate metabolic acidosis, in addition to close monitoring of acid-base status during more extensive procedures, must be part of perioperative treatment of these patients.© 2015 John Wiley & Sons Ltd.

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