Anesthetic management of patients with Joubert syndrome: a retrospective analysis of a single-institutional case series

Published in Paediatr Anaesth. 2014 Nov;24(11):1180-4.

Authors: Sriganesh K et al

OBJECTIVE:
To analyze the anesthetic techniques used for sedation during magnetic resonance imaging (MRI) study of patients with Joubert syndrome (JS) and assess the safety and efficacy of these anesthetic regimens in these children.

BACKGROUND:
Joubert syndrome is a rare neurological disorder with significant anesthetic implications. This study describes the anesthetic management of children with JS undergoing MRI study with different anesthetic agents and implications of various anesthetic techniques in these patients.

MATERIALS AND METHODS:
The records of ten patients with JS undergoing MRI study with different anesthetic techniques were retrospectively reviewed over the last 5 years.

RESULTS:
The patients were aged between 6 months and 21 years. The most commonly used sedation technique involved use of alpha-2 agonists, and this technique had least complications such as apnea and patient movement during imaging. None of the patients had postanesthetic respiratory problems, although one patient receiving propofol had apnea and desaturation on induction requiring airway intervention.

CONCLUSION:
Alpha-2 agonist based anesthetic technique appears to be most suitable for sedation during MRI study in patients with JS with respect to adverse events and outcome.