The anaesthetic management of patients with congenital insensitivity to pain with anhidrosis

Abstract

Cardiovascular complications following anaesthesia are common in patients with the southern Israel variant of CIPA. Hyperthermia, previously recognized as a major concern in patients with congenital insensitivity to pain with anhydrous, was not seen in our patients. We conclude that cardiovascular involvement is frequently encountered in CIPA patients following anaesthesia and is the major concern in their anaesthetic management.

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

“…These patients must be considered as "full stomach" regardless of the duration of nil per os status, due to autonomic nervous system abnormalities. Regurgitation and aspiration have been previously reported in patients under anesthesia (10).However, no cases of vomiting was reported in a series of 40 anesthesia records by Rozentsveig et al (5). Although not frequent, this may be a major concern for anesthesiologists.…”

“…Only few cases have been previously reported in Turkey (3,4).Some important anesthesia implications must be considered when dealing with these patients. CIPA patients have decreased peripheral and central activity of epinephrine, norepinephrine and dopamine; and this makes them prone to cardiovascular events like hypotension, bradycardia and dysrhythmias (5,6). Notwithstanding, their cardiovascular reflexes are preserved, and reflex tachycardia is observed as a response to hypotension (7).…”

Anestesia em paciente com insensibilidade congênita a dor e anidrose

“…In a review of the literature, some important aspects of those patients are emphasized, especially in relation to the type of anesthesia and perioperative temperature control 9 . Although those patients have insensitivity to pain, some of them have tactile hyperesthesia, which can cause an uncomfortable perception during surgical manipulation 10,11 . Perception of tactile stimuli and pressure can also cause complaints of postoperative pain.…”

Anesthetic Management of Congenital Insensitivity to Pain with Anhidrosis in a Child: A Case Report

“…Anesthetic management of those patients is challenging for anesthesiologist because they have the potential risks for regurgitation and aspiration, sympathetic nervous dysfunction, mandibular osteomyelitis as well as hyperthermic events [5]. In this case report we present total intravenous anesthesia (TIVA) with spontaneous respiration via facemask applied and share the management of anesthetic considerations of CIPA in a child.…”