• Kamath Sriganesh, Byrappa Vinay, Sritam Jena, Venkataramaiah Sudhir, Jitender Saini, and Ganne S Umamaheswara Rao.
• Department of Neuroanaesthesia, National Institute of Mental health and Neurosciences (NIMHANS), Bangalore, India.
• Paediatr Anaesth. 2014 Nov 1; 24 (11): 1180-4.

ObjectiveTo 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.BackgroundJoubert 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 MethodsThe records of ten patients with JS undergoing MRI study with different anesthetic techniques were retrospectively reviewed over the last 5 years.ResultsThe 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.ConclusionAlpha-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.© 2014 John Wiley & Sons Ltd.

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