Congenital insensitivity to pain with anhidrosis (CIPA) is a rare, autosomal-recessive disorder characterized by the clinical triad of indifference of
pain,
anhidrosis and
heat intolerance.Because of their lack of autonomic response to noxious stimuli, the
determination of adequate depth of
anesthesia in the CIPA
patient undergoing
surgery is a major challenge.We experienced a
patient with CIPA
who had
minor procedures three times under the
general anesthesia, in which bispectral index (BIS) was maintained at 40-50 by adjusting
sevoflurane concentrations with 50%
nitrous oxide.The low end-tidal
sevoflurane concentrations (<1.2 vol%) were required to keep the target BIS while
vital signs remained stable throughout the
surgery in each operation.BIS monitor may be a valuable tool to guide the depth of
anesthesia in
patients with CIPA.