Airway management in a patient with Hecht's syndrome

Hecht's syndrome, described in 1969, is a rare autosomal dominant phenotype that includes trismus, pseudocamptodactyly, and somewhat short stature. A 5-year-old white boy with Hecht's syndrome and frequent otitis media, nasal obstruction, and sleep apnea is described to illustrate the otolaryngologic manifestations of this syndrome. Airway management in these patients is complicated by trismus, which does not improve after the induction of anesthesia. Mask anesthesia with spontaneous ventilation has been used successfully in these patients but may be difficult in the majority of otolaryngologic procedures. Blind nasotracheal intubation is an alternative, but in this case it was impossible because of adenoid hypertrophy. In this patient, laryngoscopy and orotracheal intubation were done using a Bullard laryngoscope, and transnasal KTP laser adenoidectomy was done to relieve the obstruction and apnea. The details of Hecht's syndrome and its management are presented in this case review.