Anesthetic management of congenital insensitivity to pain with anhydrosis

Abstract

In one patient with CIPA, we found that plasma norepinephrine and epinephrine concentrations were very low, that the urine excretion ratio of HVA to VMA was elevated during enflurane anesthesia, and that an abnormal biosynthesis or catabolism of catecholamines was probably present. Careful management, including premedication to avoid apprehension, light anesthesia, maintenance of body temperature, and avoidance of joint extension, lead to a relatively safe anesthetic. Thus, with careful attention to clinical details as described above, administration of anesthesia should be without problems.