ABSTRACT

Congenital pain insensitivity syndrome (CPIS) is a very rare autosomal recessive neuropathy, characterized by insensitivity to pain which is seen in early childhood. In all patients the pain sensation is absent from birth. Systemic anhydrosis and mental retardation are also characteristic findings of this syndrome. Pediatric patients with CPIS frequently present for multiple orthopaedic surgeries requiring general anesthesia. Although there is insensitivity to pain, patients have tactile hyperesthesia that can cause unpleasant feelings during surgical procedures. We report our anaesthetic management of a 12 years old male patient with CPIS undergoing right knee arthrodesis undergoing general anaesthesia.

Keywords: Anesthesia, general, congenital pain insensitivity syndrome, bispectral index