Anesthetic Effects in Mitochondrial Disease

Summary. At the present, the investigators do not have the perfect anesthetic for
mitochondrial patients. When possible, consideration should be given to the use of local
anesthetics in small amounts. When a general anesthetic is necessary, they each carry
significant risks and have been associated with poor outcomes. At present it is not
possible to eliminate one group as less safe than others. What is clear is that these
patients must be monitored more closely than other patients. The advent of the bispectral
index (BIS) monitor may allow us to monitor their depth of anesthesia more closely and thus
expose these patients only to the minimum amount of drug necessary to carry out the surgical
procedure.

Purpose. The investigators hypothesize that specific mitochondrial diseases, in particular
those that decrease complex I function, make certain children hypersensitive to volatile
anesthetics. These same patients may be at increased risk for adverse outcomes following
general anesthesia. The specific aims of this application are:

1. Determine which molecular defects in mitochondrial function lead to alter sensitivity
to the VA sevoflurane.

2. Establish the relative safety of sevoflurane in treatment of patients with
mitochondrial disease.

The investigators plan to monitor patients with mitochondrial disease using expanded
measures of cardiovascular stability and measurements of brain electrical activity while
slowly inducing general anesthesia. The investigators will use those measurements to limit
the amount of anesthetic these patients receive in an attempt to minimize their risk. In
addition, the investigators will correlate their sensitivity to the type of mitochondrial
defect so that the investigators may be able to predict which patients are likely to have an
increased sensitivity.