Schulich school of Medicine and Dentistry logo Anesthesia and Perioperative Medicine Schulich School of Medicine & Dentistry

Anesthetic considerations in a parturient with congenital insensitivity to pain with anhidrosis

Pirani Z, Qasem F, Katsiris S.

Highlights

  • Tyrosine kinase receptor type-1 (TRKA) is the gene mutation associated with CIPA.
  • Neurotrophic Receptor Tyrosine Kinase 1 (NTRK1) is confirmatory for CIPA.
  • We discuss the the genetic transmission and pathophysiology of CIPA.
  • The primary anesthetic concern in patients with CIPA is autonomic dysfunction.

Abstract

Congenital insensitivity to pain is a rare autosomal recessive disease characterized by varying degrees of autonomic dysfunction and sensory loss, including nociceptive hyposensitivity. It is this autonomic dysfunction that makes both general and neuraxial anesthesia challenging. Testing the block during neuraxial anesthesia may be difficult and the patient may be prone to hypotension and bradycardia. This case report describes the anesthetic management of a parturient with congenital insensitivity to pain presenting for cesarean delivery.

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