Upper Airway Function in Ondine's Curse

Congenital central alveolar hypoventilation (Ondine's curse) is a rare disorder with absence of automatic control of ventilation but preservation of voluntary breathing. Phrenic pacing, used to treat this condition, is usually not successful without a tracheotomy. We performed fiberoptic videoendoscopy on an affected infant airway. During wakefulness and ventilation by a negative pressure ventilator, the airway was normal, but with sleep onset, passive inspiration produced phasic epiglottic collapse. This case demonstrates the importance of central control in maintaining upper airway patency.