Airway involvement and anesthetic management in Goltz's syndrome

A 13-year-old girl with Goltz's (Goltz-Gorlin) syndrome (focal dermal hypoplasia) and cloacal exstrophy underwent day surgery cystoscopy. During the administration of inhalation anesthesia by mask, persistent partial upper airway obstruction was noted. Direct laryngoscopy unexpectedly showed variously sized verrucous lesions in the hypopharynx and supraglottic larynx, and the vocal cords and trachea could not be visualized. The patient later returned for a complete endoscopic examination, followed by cup forcep and laser excision of these verrucous and pedunculated lesions, and remained intubated in the intensive care unit (ICU) for 48 hours following this procedure. Airway management and pathology specific to this rare disorder are discussed.