Central alveolar hypoventilation syndrome (Ondine's curse) with gastroesophageal reflux.
Congenital central hypoventilation syndrome (Ondine's curse) is a rare disorder with lack of automatic control of ventilation during sleep. We have reported a case of Ondine's curse in a patient who underwent Nissen's fundoplication for gastroesophageal reflux (GER) at age 5 months. Ventilatory challenge test during sleep was done to confirm central alveolar hypoventilation. This female patient, without cor pulmonale, was a good candidate for diaphragm pacing. Thus, the patient underwent implantation of a diaphragm pacer at age 3 years; she had required mechanical ventilation since birth. Diagnosis, pathogenesis, and problems in the setting of diaphragm pacing for an infant are discussed.
Takeda
S
S
First Department of Surgery, Osaka University Medical School, Japan.
Fujii
Y
Y
Kawahara
H
H
Nakahara
K
K
Matsuda
H
H
D002675:Child, Preschool; D005260:Female; D018662:Fundoplication; D005764:Gastroesophageal Reflux / Q000150:complications* / Q000601:surgery; D006801:Humans; D012121:Respiration, Artificial; D012891:Sleep Apnea Syndromes / Q000150:complications* / Q000628:therapy