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Central Alveolar Hypoventilation Syndrome (Ondine's Curse) With Gastroesophageal Reflux

Shin-ichi Takeda; Yoshitaka Fujii; Hikaru Matsuda; Hisayoshi Kawahara; Kazuya Nakahara
Author and Funding Information

Affiliations: From the First Department of Surgery, Osaka University Medical School, Osaka, Japan,  From the Department of Pediatric Surgery, Kure National Hospital, Osaka, Japan,  From the Department of Surgery, Ootemae Hospital, Osaka, Japan

Affiliations: From the First Department of Surgery, Osaka University Medical School, Osaka, Japan,  From the Department of Pediatric Surgery, Kure National Hospital, Osaka, Japan,  From the Department of Surgery, Ootemae Hospital, Osaka, Japan

Affiliations: From the First Department of Surgery, Osaka University Medical School, Osaka, Japan,  From the Department of Pediatric Surgery, Kure National Hospital, Osaka, Japan,  From the Department of Surgery, Ootemae Hospital, Osaka, Japan


1996 by the American College of Chest Physicians


Chest. 1996;110(3):850-852. doi:10.1378/chest.110.3.850
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Abstract

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.


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