We report
anesthetic management of a 22-month-old child with
Cornelia de Lange syndrome scheduled for palatoplasty because of
cleft palate.
Micrognathia and short neck of the patient suggested difficult
airway management. For
anesthetic induction, 1 microg x kg(-1)
dexmedetomidine was loaded intravenously, followed by infusion at a rate of 0.7 microg x kg(-1) x hr(-1) with incremental inhalation of
sevoflurane. During the induction, spontaneous breathing was maintained. By laryngoscopy with Machintosh blade after intravenous
propofol for the purpose of laryngeal reflex inhibition, only the tip of epiglottis was seen. Then, after another intravenous
propofol, laryngoscopy was performed with Airwayscope, which enabled operators to see the glottis. Then, a RAE endotracheal tube (ID 4.5 mm) was inserted. During the Airwayscope operation,
pharyngeal reflex, laryngeal reflex and saliva increase were inhibited resulting in good view of the larynx and the lowest Spo2 was 94% temporarily. After intubation,
anesthesia was maintained with
sevoflurane,
remifentanil and
fentanyl.
Dexmedetomidine infusion was also useful to maintain adequate spontaneous breathing and to achieve awaking before extubation.