[Ondine's syndrome (alveolar hypoventilation)].
Ondine's syndrome is a rare condition characterised by alveolar hypoventilation during sleep on account of an abnormality in the automatic control of respiration. The respiration centre does not react adequately to the carbon dioxide tension in the blood required to maintain normal ventilation. We present a description of the course in an infant with congenital Ondine's syndrome because a therapeutic possibility is now available, viz, implantation of a phrenic nerve pacemaker. The infants showed respiratory insufficiency shortly after delivery. This disappeared on tactile stimulation. The infant had generalised hypotonia with absent patellar reflexes and weak sucking reflexes and, at the age of one week, seizures developed. The infant was treated with assisted ventilation but this could be reduced to use only during the period when the infant slept. The infant was in good health with normal psychomotor development until the age of two years. Plans had been made to implant a phrenic nerve pacemaker. Unfortunately, the infant developed Syncytial-virus pneumonia complicated by bacterial superinfection and developed severe anoxic brain damage which subsequently proved fatal.
Hansen
H
H
Aalborg Sygehus, børneafdelingen.
Thomsen
K D
KD
Ebbesen
F
F
D002675:Child, Preschool; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012119:Respiration / Q000502:physiology; D012891:Sleep Apnea Syndromes / Q000151:congenital* / Q000503:physiopathology / Q000628:therapy
Undine's syndrom (alveolaer hypoventilation).