Important Aspects of Anesthetic Management of a Patient with Hereditary Sensorial and Autonomic Neuropathy Type 4: A Case Report

Abstract

Hereditary Sensorial Autonomic Neur opathy (HSAN) type IV or congenital insensitivity to pain and anhidrosis (CIPA) is a rare congenital disorder characterized by recurrences of unexplained fever, extensive anhidrosis, insensitivity to pain, hypotonia and mental retardation in some cases. These patients develop corneal scarring, teeth eruptions, multiple fractures, osteomyelitis, buccal and lower limb wounds, injuries of finger tips so they undergo surgery. In this paper we report a patient with HSAN-IV, who developed tibia fracture and undergone successful operation under monitored anesthesia care. A 7 year old patient with HSAN-IV, admitted with left tibia fracture, obviously painless. He was operated under monitored anesthesia care without any complications and discharged home after a night follow-up. No anesthetic or analgesic drugs had been used for the surgery. Anesthetic considerations for the management of these patients are adequate perioperative sedation to reduce tactile hyperesthesia and excitement, close monitoring of cardiac rhythm and blood pressure, monitoring of body temperature and observation of nausea and vomiting.