Anesthetic Considerations for Liver Transplantation in a Patient with Mitochondrial Neurogastrointestinal Encephalopathy Syndrome.

Abstract

Mitochondrial neurogastrointestinal encephalopathy (MNGIE) is a rare, complex mitochondrial disorder with variable phenotypes caused by a defect in the TYMP gene that codes for the thymidine phosphorylase enzyme. Orthotopic liver transplantation (OLT) has been proposed as a curative option for patients by using the liver as a source to restore thymidine phosphorylase levels in the body. Anesthetic considerations for this syndrome have not been clearly outlined in the past. We describe the clinical presentation of a young woman with MNGIE, her perioperative assessment, and intraoperative management during liver transplantation.

• Authors: Jai Madhok, Jason Leong, Jed Cohn
• Journal: Cureus (Cureus) Vol. 11 Issue 6 Pg. e5038 (Jun 29 2019) ISSN: 2168-8184 [Print] United States
• PMID: 31501730 (Publication Type: Case Reports)