Anesthetic management in Joubert syndrome.
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.
Vodopich
Darko J
DJ
Department of Anesthesiology, MetroHealth Medical Center, Cleveland, OH, USA. vodopich@yahoo.com
Gordon
Gregory J
GJ
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
D006993:Hypnotics and Sedatives; D015742:Propofol