Anesthetic management in Joubert syndrome

Paediatr Anaesth. 2004 Oct;14(10):871-3. doi: 10.1111/j.1460-9592.2004.01286.x.


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.

Publication types

  • Case Reports

MeSH terms

  • Agenesis of Corpus Callosum
  • Anesthesia*
  • Anesthesia, Caudal
  • Apnea / complications
  • Apnea / physiopathology
  • Ataxia / physiopathology*
  • Brain / abnormalities
  • Corpus Callosum / pathology
  • Developmental Disabilities / physiopathology*
  • Hernia, Inguinal / surgery
  • Humans
  • Hypnotics and Sedatives
  • Infant
  • Magnetic Resonance Imaging
  • Male
  • Ocular Motility Disorders / physiopathology*
  • Propofol
  • Respiratory Mechanics
  • Respiratory Tract Diseases / physiopathology*
  • Syndrome


  • Hypnotics and Sedatives
  • Propofol