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Case Reports in Anesthesiology
Volume 2015 (2015), Article ID 453714, 3 pages
Case Report

Anesthetic Management of a Child with Mitochondrial Neurogastrointestinal Encephalopathy

Department of Anesthesiology & Perioperative Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 409, Houston, TX 77030, USA

Received 3 March 2015; Accepted 21 May 2015

Academic Editor: Renato Santiago Gomez

Copyright © 2015 Vianey Q. Casarez et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is an autosomal recessive disorder associated with deficiency of thymidine phosphorylase (TP). Associated manifestations include visual and hearing impairments, peripheral neuropathies, leukoencephalopathy, and malnutrition from concomitant gastrointestinal dysmotility and pseudoobstruction. Given the altered metabolic state in these patients, specific consideration of medication selection is advised. This case report will describe the anesthetic management used in a 10-year-old girl with MNGIE. She had multiple anesthetics while undergoing allogeneic hematopoietic stem cell transplantation. This case report will discuss the successful repeated use of the same anesthetic in this pediatric patient, with the avoidance of volatile anesthetic agents, propofol, and muscle relaxant.