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Anesthesia management in congenital pain insensitivity syndrome: a case report

Author(s): Hakan Erkal | Yaman ozyurt | Feriha Temizel | Gulten Arslan

Journal: Medical Journal of Bakirköy
ISSN 1305-9319

Volume: 2;
Issue: 9;
Start page: 78;
Date: 2013;
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Keywords: Anesthesia | general | congenital pain insensitivity syndrome | bispectral index

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.

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