title: Anesthetic management of patients with Joubert syndrome: a retrospective analysis of a single-institutional case series.
abstract: 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.
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Neuroanaesthesia, National Institute of Mental health and Neurosciences (NIMHANS), Bangalore, India.
authors.forename: Kamath
authors.initials: K
authors.lastname: Sriganesh
authors.forename: Byrappa
authors.initials: B
authors.lastname: Vinay
authors.forename: Sritam
authors.initials: S
authors.lastname: Jena
authors.forename: Venkataramaiah
authors.initials: V
authors.lastname: Sudhir
authors.forename: Jitender
authors.initials: J
authors.lastname: Saini
authors.forename: Ganne S
authors.initials: GS
authors.lastname: Umamaheswara Rao
mesh_terms: D000015:Abnormalities, Multiple; D000293:Adolescent; D058647:Adrenergic alpha-2 Receptor Agonists / Q000627:therapeutic use*; D000328:Adult; D000758:Anesthesia / Q000379:methods; D014151:Anti-Anxiety Agents / Q000627:therapeutic use*; D002526:Cerebellar Diseases / Q000175:diagnosis*; D002531:Cerebellum / Q000002:abnormalities; D002648:Child; D002675:Child, Preschool; D005124:Eye Abnormalities / Q000175:diagnosis*; D005260:Female; D006801:Humans; D006993:Hypnotics and Sedatives / Q000627:therapeutic use*; D007223:Infant; D052177:Kidney Diseases, Cystic / Q000175:diagnosis*; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008991:Monitoring, Physiologic / Q000379:methods*; D012160:Retina / Q000002:abnormalities*; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D058647:Adrenergic alpha-2 Receptor Agonists; D014151:Anti-Anxiety Agents; D006993:Hypnotics and Sedatives
keywords: Joubert syndrome; alpha-2 agonists; anesthesia; magnetic resonance imaging; molar tooth sign; respiratory abnormalities