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이윤숙 ( Yoon Sook Lee ),김운영 ( Woon Young Kim ),김경근 ( Kyoung Gun Kim ),장문석 ( Moon Seok Chang ),김재환 ( Jae Hwan Kim ),박영철 ( Young Cheol Park ) 대한마취과학회 2009 Korean Journal of Anesthesiology Vol.57 No.1
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. (Korean J Anesthesiol 2009; 57: 96~9)