• 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.
(Arch Otolaryngol Head Neck Surg. 1992;118:310-312)
Olson TS, Woodson GE, Heldt GP. Upper Airway Function in Ondine's Curse. Arch Otolaryngol Head Neck Surg. 1992;118(3):310–312. doi:10.1001/archotol.1992.01880030098020
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