Anesthetic management of two siblings with congenital insensitivity to pain with anhidrosis syndrome

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare syndrome characterized by a lack of sensitivity to pain due to congenital sensory and autonomic neuropathies, anhidrosis, an inability to regulate body temperature, growth retardation, mental retardation at different levels of severit...

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Bibliographic Details
Published inAǧrı Vol. 31; no. 4; p. 202
Main Authors Destegul, Dilek, Kocaöz, Fazilet, Sarı, Ahmet Sinan
Format Journal Article
LanguageEnglish
Published Turkey 01.10.2019
Subjects
Adolescent
Anesthesia
Ankle Joint - surgery
Arthritis, Infectious - surgery
Female
Hereditary Sensory and Autonomic Neuropathies
Humans
Hypnotics and Sedatives - therapeutic use
Male
Midazolam - therapeutic use
Pain Measurement
Siblings
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Summary:Congenital insensitivity to pain with anhidrosis (CIPA) is a rare syndrome characterized by a lack of sensitivity to pain due to congenital sensory and autonomic neuropathies, anhidrosis, an inability to regulate body temperature, growth retardation, mental retardation at different levels of severity, and inadvertent self-harm. It is an autosomal recessive disorder that is result of a mutation in the neurotrophic receptor tyrosine kinase 1 gene, which encodes neurotrophic tyrosine kinase. CIPA patients are frequently admitted to hospitals with unrecognized traumatic fractures and unhealed wounds due to the lack of a pain response. Presently described is the method of anesthetic management used for 2 siblings, aged 17 and 14 years, with a generalized lack of pain, anhidrosis, mental retardation, and septic arthritis. Sedation with midazolam alone provided satisfactory surgical comfort without causing hemodynamic instability in these 2 patients with CIPA syndrome.
ISSN:1300-0012
DOI:10.14744/agri.2019.91297