Abstract
The anesthetic implications of Joubert syndrome in an infant who required cardiac surgery using cardiopulmonary bypass (CPB) is presented. Children with Joubert syndrome present with central apnea due to malformations in the midbrain and cerebellum. These patients have a marked sensitivity to opioids. The use of dexmedetomidine along with remifentanil was effective in this case.
Keywords:
Anesthesia; Apnea; Cardiac surgery; Congenital anomalies; Dexmedetomidine; Joubert syndrome; Neonatology; Opioids.
© 2013.
MeSH terms
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Abnormalities, Multiple
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Anesthesia, Inhalation / methods*
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Anesthetics, Inhalation / administration & dosage
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Cardiopulmonary Bypass
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Cerebellar Diseases / complications
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Cerebellar Diseases / diagnosis
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Cerebellar Diseases / surgery*
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Cerebellum / abnormalities
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Eye Abnormalities / complications
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Eye Abnormalities / diagnosis
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Eye Abnormalities / surgery*
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Female
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Humans
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Infant
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Kidney Diseases, Cystic / complications
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Kidney Diseases, Cystic / diagnosis
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Kidney Diseases, Cystic / surgery*
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Magnetic Resonance Imaging
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Methyl Ethers / administration & dosage
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Pulmonary Valve Stenosis / surgery*
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Retina / abnormalities*
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Retina / surgery
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Sevoflurane
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Sleep Apnea, Central / etiology
Substances
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Anesthetics, Inhalation
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Methyl Ethers
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Sevoflurane
Supplementary concepts
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Agenesis of Cerebellar Vermis