Anesthetic Management of a Child with Mitochondrial Neurogastrointestinal Encephalopathy
Vianey Q Casarez1, Acsa M Zavala1, Pascal Owusu-Agyemang1
1Department of Anesthesiology & Perioperative Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 409, Houston, TX 77030, USA.
Abstract:
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.
More Related Videos
Related Concept Videos
Parenteral Anesthetics: Overview
General Anesthesia: Overview
General anesthesia induces unconsciousness in the whole body, while the others target specific areas or sensations. It is administered to minimize adverse effects, maintain...
Skeletal Muscle Relaxants: Therapeutic Uses
Inhalational Anesthetics: Overview
Stages of General Anesthesia
Local Anesthetics: Adverse Effects
Once absorbed into the systemic circulation, local anesthetics can affect the organs that depend on the functioning of sodium...


