title: Anesthetic management in Joubert syndrome.
abstract: We review the anesthetic implications of Joubert syndrome and report that spinal anesthesia under intravenous propofol sedation proved satisfactory for repair of an inguinal hernia in a spontaneously ventilating infant with this syndrome. We caution that anatomical peculiarities may complicate performance of a caudal epidural block and suggest consideration of the use of caffeine to ameliorate the apneic episodes seen in early infancy in this syndrome.
authors.affiliation: Department of Anesthesiology, MetroHealth Medical Center, Cleveland, OH, USA. vodopich@yahoo.com
authors.forename: Darko J
authors.initials: DJ
authors.lastname: Vodopich
authors.forename: Gregory J
authors.initials: GJ
authors.lastname: Gordon
mesh_terms: D061085:Agenesis of Corpus Callosum; D000758:Anesthesia; D000763:Anesthesia, Caudal; D001049:Apnea / Q000150:complications / Q000503:physiopathology; D001259:Ataxia / Q000503:physiopathology*; D001921:Brain / Q000002:abnormalities; D003337:Corpus Callosum / Q000473:pathology; D002658:Developmental Disabilities / Q000503:physiopathology*; D006552:Hernia, Inguinal / Q000601:surgery; D006801:Humans; D006993:Hypnotics and Sedatives; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D015835:Ocular Motility Disorders / Q000503:physiopathology*; D015742:Propofol; D015656:Respiratory Mechanics; D012140:Respiratory Tract Diseases / Q000503:physiopathology*; D013577:Syndrome
chemical_list: D006993:Hypnotics and Sedatives; D015742:Propofol