Drug management in emergent liver transplantation of mitochondrial disorder carriers: review of the literature.
BACKGROUND
Mitochondrial respiratory-chain disorders (MRCD) lead to progressive disabling of neurological and cellular conditions that involve muscles, brain, kidney, and liver dysfunction. Affected individuals may need surgery, including orthotopic liver transplantation (OLT). Surgery poses anesthesia challenges because of the prolonged use of anesthetic drugs and sedatives, which may inhibit oxidative phosphorylation, mimic mitochondrial cytopathic disorders, or unveil them ex novo.


METHODS
We conducted a multilingual PubMed search of surgical and non-surgical anesthesia reports between the years 1992 and 2008, where anesthetic drugs were used in MRCD patients, especially for those undergoing urgent OLTs.


RESULTS
There were 51 case reports of 210 anesthesia and critical care interventions in patients with MRCD, a large part of them were children. Data pertaining to the safe usage of anesthesia and perioperative drugs were limited and conflicting. We found no article that addressed the issue of perioperative handling of urgent OLT in MRCD patients. We therefore suggest our own - although limited - experience for such occasions.


CONCLUSIONS
There are no randomized, controlled, trial-based indications regarding safe anesthetic drugs to be used perioperatively in MRCD carriers. Consultation among geneticists, anesthesiologists, intensivists, and surgeons is essential in patients with known/suspected metabolic syndrome for planning appropriate perioperative care.
Vater
Youri
Y
Department of Anesthesiology and Pain Medicine, University of Washington School of Medicine, Seattle, WA, USA.
Dembo
Gregory
G
Martay
Kenneth
K
Klein
Yifat
Y
Vitin
Alex
A
Weinbroum
Avi A
AA
D000758:Anesthesia; D004632:Emergency Medical Services; D006801:Humans; D016031:Liver Transplantation; D028361:Mitochondrial Diseases / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D009422:Nervous System Diseases / Q000209:etiology; D013312:Stress, Physiological / Q000502:physiology