The Use of Dexmedetomidine During Nasotracheal Intubation in a Patient with CHARGE Syndrome

Abstract

CHARGE Syndrome (CS), also known as Hall-Hittner syndrome, is a congenital disorder comprised of multiple anomalies (coloboma, heart defect, atresia choanal, retarded growth and development, genital hypoplasia, ear anomalies/deafness). First described in 1979 by Hall and Hittner, it is caused by a mutation in the gene CHD7 on chromosome 8.1 Facial and upper airway features of CS, including midface hypoplasia, micrognathia, cleft lip, and palate, anterior larynx, and subglottic stenosis, make the airway management of these patients a challenge for the anesthesiologist. Because difficult airway management is a concern, spontaneous ventilation is recommended until the airway is secured. We report the use of dexmedetomidine (DEX) for deep sedation in a CS patient who required nasotracheal intubation using a fiberoptic bronchoscope (FB) while breathing spontaneously.