Perioperative use of dexmedetomidine in an infant with familial dysautonomia.

We present a case of a 10-month-old girl with familial dysautonomia, who was scheduled for the insertion of a gastrotomy tube via laparoscopy under general anaesthesia. We used a total i.v. anaesthetic technique including dexmedetomidine and titrated the drug to patients' haemodynamic status and BIS value. Vital signs remained virtually unchanged during the entire procedure, and the tracheal tube was removed at the end of the procedure. Postoperative course was uneventful. Careful planning of the anaesthetic management, understanding the physiological consequences, and being able to titrate the medications utilized are key to the decrease of complications encountered in these patients. We report the safe use of dexmedetomidine in an infant with this extremely rare condition.