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Anaesthetic management of children with Joubert syndrome
Author(s) -
HABRE WALID,
SIMS CRAIG,
D’SOUZA MICHAEL
Publication year - 1997
Publication title -
pediatric anesthesia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.704
H-Index - 82
eISSN - 1460-9592
pISSN - 1155-5645
DOI - 10.1046/j.1460-9592.1997.d01-65.x
Subject(s) - medicine , nitrous oxide , joubert syndrome , anesthesia , brainstem , respiratory system , respiratory monitoring , general anaesthesia , ventilation (architecture) , psychiatry , biology , cilium , microbiology and biotechnology , mechanical engineering , engineering
We report the anaesthetic management of two children with Joubert syndrome. Children with this syndrome have abnormalities of respiratory control due to changes in the brainstem and cerebellum. They are extremely sensitive to the respiratory depressant effects of anaesthetic agents, including nitrous oxide. Anaesthesia using inhalational induction, controlled ventilation, avoidance of opioids, and close postoperative monitoring is recommended.