Regional anesthetic techniques have long
been denied in these patients, even though no scientific data are available.
As for all demyelinating disorders, as the disease progresses, nerve
stimulation becomes technically more difficult, thus making nerve location
more unreliable, and less concentrated local anesthetic solutions are
sufficient. Epidural and spinal anesthesias have been performed without
adverse effects in many patients. FRDA patients have a marked sensitivity to
depolarizing muscle relaxants, which induces hyperkalemia. The sensitivity
to nondepolarizing muscle relaxants is increased, and proper neuromuscular
monitoring is essential. However, monitoring of muscle relaxation may be
difficult, depending on the progress of the disease. If general anesthesia
is used, transesophageal echocardiographic monitoring is helpful to maintain
continuous evaluation of cardiac contraction. As with any hypertrophic
cardiomyopathy, maintain adequate volume status and avoid tachycardia,
vasoplegia, and adrenergic stimulation during the intraoperative and
postoperative periods. Postoperative supervision must emphasize hemodynamic
and cardiac rhythm monitoring, adequate analgesia, and careful clinical
muscular monitoring, especially of the pharyngeal and respiratory muscle.
Postoperative mechanical ventilation must be considered to ensure adequate
ventilation and facilitates adequate pain management.