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Korean J Anesthesiol > Volume 57(1); 2009 > Article
Korean Journal of Anesthesiology 2009;57(1):96-99.
DOI: https://doi.org/10.4097/kjae.2009.57.1.96   
Anesthetic management for a pediatric patient with joubert syndrome: A case report.
Yoon Sook Lee, Woon Young Kim, Kyoung gun Kim, Moon seok Chang, Jae Hwan Kim, Young Cheol Park
Department of Anesthesiology and Pain Medicine, Korea University Ansan Hospital, Korea University College of Medicine, Ansan, Korea. ckssis@korea.ac.kr
Abstract
Joubert syndrome is a rare autosomal recessive condition in which there is complete or partial agenesis of the cerebellar vermis. The clinical features are hypotonia, ataxia, mental retardation, abnormal ocular movements, typical facial features and episodic tachypnea with alternating apnea. The abnormal respiratory pattern and hypotonia may be exacerbated by anesthetics, and especially, the apneic episodes may be prolonged by administering opioids. Joubert syndrome has been associated with various airway abnormalities such as a high and arched palate, a large or protruding tongue, laryngomalacia, a variable epiglottis and micrognathia. These abnormalities may cause difficulty with tracheal intubation. In this present case, direct laryngoscope-assisted, fiberscopic guided intubation was performed. This case is the first report of anesthetic management in a patient with Joubert syndrome and who underwent palatoplasty in Korea.
Key Words: Anesthetic management; Fiberscopic intubation; Joubert syndrome


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