SUGAMMADEX AND GENARAL ANESTHESIA IN TWO PEDIATRIC PATIENTS WITH JOUBERT SYNDROME: CASE REPORT

DİLEK GÜNAY CANPOLAT, MUSTAFA DENİZHAN YILDIRIM, SALİH DOĞAN, FATMA DOĞRUEL, HÜSEYİN PER, MEHMET CANPOLAT

Journal of Clinical Practice and Research - 2017;39(1):35-36

Department of Oral Maxillofacial Surgery, Erciyes University Faculty of Dentistry, Kayseri, Turkey

 

Joubert syndrome (JS) is a rare genetic disorder that affects the cerebellum, controlling balance, and coordination, and it usually presents a molar tooth sign on magnetic resonance imaging. It is characterized by ataxia, an abnormal breathing pattern referred to as hyperpnea, sleep apnea, hypotonia, and abnormal eye and tongue movements. Because of sensitivity to respiratory depressant agents, including opiates, nitrous oxide, and neuromuscular blockers, and potential difficult airways, anesthetic management requires attention. Sugammadex binds to rocuronium and is a reverse neuromuscular blocker. In this study, two cases of JS where sugammadex was used for general anesthesia are presented. Sugammadex may be a safe general anesthetic for patients with JS.