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.
Olson
T S
TS
Department of Otolaryngology-Head and Neck Surgery, University of Michigan, Ann Arbor.
Woodson
G E
GE
Heldt
G P
GP
D004825:Epiglottis / Q000503:physiopathology*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007828:Laryngoscopy / Q000379:methods; D011175:Positive-Pressure Respiration; D012891:Sleep Apnea Syndromes / Q000151:congenital* / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D014140:Tracheotomy; D014741:Video Recording