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Anesth Analg 2008; 107:648-650
© 2008 International Anesthesia Research Society
doi: 10.1213/ane.0b013e3181770fcc
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GENERAL ARTICLE

Anesthetic Management of a Patient with 3-Methylcrotonyl-CoA Carboxylase Deficiency

Karen A. Robbins, MD, and Elias N. León-ruiz, MD

From the Department of Anesthesiology and Perioperative Medicine, University Hospitals Case Medical Center, Cleveland, Ohio.

Address correspondence to Karen A. Robbins, MD, Children’s National Medical Center, 111 Michigan Ave NW, Suite W3.5-600, Washington, DC 20010-2970. Address e-mail to robbinska{at}gmail.com.

Patients with inborn errors of metabolism require special considerations in perioperative care. In the following case report, we describe the successful management of a patient with 3-methylcrotonyl-CoA carboxylase deficiency, a deficit that causes a secondary carnitine deficiency and impaired β oxidation. Patients may have significant underlying cardiomyopathy, and are at risk for metabolic decompensation, acidosis, and hypoglycemia during periods of stress.






Lippincott, Williams & Wilkins Anesthesia & Analgesia® is published for the International Anesthesia Research Society® by Lippincott Williams & Wilkins and Stanford University Libraries' HighWire Press®. Copyright 2008 by the International Anesthesia Research Society. Online ISSN: 1526-7598   Print ISSN: 0003-2999 HighWire Press
Copyright © 2008 by the International Anesthesia Research Society.