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Review
. 2017 Aug 29;17(1):116.
doi: 10.1186/s12871-017-0400-9.

Anesthetic management of a pediatric patient with Electron Transfer Flavoprotein Dehydrogenase deficiency (ETFDH) and acute appendicitis: case report and review of the literature

Emmanuel Lilitsis  1 , Elisavet Astyrakaki  1 , Evaggelos Blevrakis  2 , Sofia Xenaki  2 , George Chalkiadakis  2 , Emmanuel Chrysos  3
Affiliations
Review

Anesthetic management of a pediatric patient with Electron Transfer Flavoprotein Dehydrogenase deficiency (ETFDH) and acute appendicitis: case report and review of the literature

Emmanuel Lilitsis et al. BMC Anesthesiol. .

Abstract

Background: Mitochondria are the energy producing organelles practically in every human cell except erythrocytes. Indeed mitochondria are widespread in high energy requiring organs like brain, heart and muscles. Currently there are no clinical trials supporting with clear evidence which is the most suitable surgical or anesthetic management of a patient with known mitochondrial disease presenting with surgical disorders. This condition poses possible hazardous problems to the medical attention of those patients.

Case presentation: A case of an 8 year old child with known Electron Transfer Flavoprotein Dehydrogenase deficiency (ETFDH deficiency) requiring surgery for acute appendicitis is presented. Our approach for anesthesia revealed a combination of fentanyl, low dose propofol and nitrous oxide.

Conclusion: The choice of the safest pharmacological anesthetic agents for patients with ETFDH deficiency is challenging given that most of the general anesthetic medications have multiple effects on mitochondria, fatty acids metabolism and striated muscles. Anesthetists are expected to individualize anesthetic care for the patient based on current publications for similar cases, medical history and knowledge of pharmacology and physiology.

Keywords: Deficiency; Electron Transfer Flavoprotein Dehydrogenase; Pharmacological anesthetic agents.

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Conflict of interest statement

Ethics approval and consent to participate

NA.

Consent for publication

Written informed consent was obtained from the patients of the child, for publication of these Case reports and any accompanying images/videos. A copy of the written consents is available for review by the Editor of this journal.

Competing interests

For this study we have received NO reimbursements, fees, funding, or salary from an organization that may in any way gain or lose financially from the publication of this manuscript, either now or in the future. We hold NO stocks or shares in an organization that may in any way gain or lose financially from the publication of this manuscript, either now or in the future. We DO NOT hold any patents relating to the content of the manuscript. We have NO other financial competing interests. There any NO non-financial competing interests (political, personal, religious, ideological, academic, intellectual, commercial or any other) to declare in relation to this manuscript.

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