HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

Anesthetic Management of Patients with Congenital Insensitivity to Pain with Anhidrosis: A Retrospective Analysis of 358 Procedures Performed Under General Anesthesia.

AbstractBACKGROUND:
Congenital insensitivity to pain with anhidrosis (CIPA) is a rare autosomal recessive disorder characterized by recurrent episodic fevers, anhidrosis, absent reaction to noxious stimuli, self-mutilating behavior, and mental retardation. The anesthetic management of patients with CIPA is challenging. Autonomic nervous system abnormalities are common, and patients are at increased risk for perioperative complications.
METHODS:
In this study, we describe our experience with 35 patients with CIPA who underwent 358 procedures requiring general anesthesia between 1990 and 2013.
RESULTS:
During surgery, 3 patients developed hyperthermia intraoperatively (>37.5°C) without prior fever. There were no cases of intraoperative hyperpyrexia (>40°C). Aspiration was suspected in 2 patients, and in another patient aspiration was prevented by the use of endotracheal tube, early detection of regurgitation, and aggressive suctioning. One patient had cardiac arrest requiring cardiopulmonary resuscitation. Intraoperative bradycardia was observed in 10 cases, and postoperative bradycardia was observed in 11 cases.
CONCLUSIONS:
Regurgitation, hyperthermia, and aspiration were uncommon, but the incidence of bradycardia was higher than has been reported in previous studies. CIPA remains a challenge for anesthesiologists. Because of the rare nature of this disorder, the risk of various complications is difficult to predict.
AuthorsAlexander Zlotnik, Dmitry Natanel, Ruslan Kutz, Matthew Boyko, Evgeny Brotfain, Benjamin F Gruenbaum, Shaun E Gruenbaum, Lipa Bodner
JournalAnesthesia and analgesia (Anesth Analg) Vol. 121 Issue 5 Pg. 1316-20 (Nov 2015) ISSN: 1526-7598 [Electronic] United States
PMID26484462 (Publication Type: Journal Article)
Chemical References
  • Anesthetics
Topics
  • Adolescent
  • Anesthesia, General (methods)
  • Anesthetics (administration & dosage)
  • Child
  • Child, Preschool
  • Disease Management
  • Female
  • Hereditary Sensory and Autonomic Neuropathies (diagnosis, drug therapy, surgery)
  • Humans
  • Infant
  • Male
  • Postoperative Complications (diagnosis, prevention & control)
  • Retrospective Studies
  • Young Adult

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research graph!


Choose Username:
Email:
Password:
Verify Password:
Enter Code Shown: