کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
4159333 | 1273825 | 2008 | 5 صفحه PDF | دانلود رایگان |
![عکس صفحه اول مقاله: Diaphragmatic pacing for the treatment of congenital central alveolar hypoventilation syndrome Diaphragmatic pacing for the treatment of congenital central alveolar hypoventilation syndrome](/preview/png/4159333.png)
PurposeThe congenital central alveolar hypoventilation syndrome (CCAHS), also known as Ondine curse, is characterized by the absence of adequate autonomic control of respiration.The purpose of our study is to review our 20-year experience with diaphragmatic pacing as a treatment modality for CCAHS.MethodsAfter obtaining institutional review board approval, the medical records of all 6 patients who currently are in the diaphragmatic pacing program at the Montreal Children's Hospital, Montreal, QC, Canada were reviewed. In addition to demographic details, associated anomalies were noted. Data regarding age at surgery, technical approach, and short- and long-term complications, including equipment failure and replacement, were noted. We were interested in the long-term outcome for this group of patients, particularly their quality of life.ResultsOur group of patients is composed of 4 females and 2 males. Their age ranges between 4 and 23 years. They were operated on at an average age of 47.8 months. The surgical approach used was mainly bilateral axillary thoracotomy. Internal component failure is the most common complication. All patients are ventilator free during the day. They all are active and productive, either attending school or working full time.ConclusionDiaphragmatic pacing is an effective treatment of Ondine disease. As equipment improved, there is much less need for replacement of components. Patients can lead a much more normal existence by being ventilator free at least during the day, enabling them to participate in normal daily activities.
Journal: Journal of Pediatric Surgery - Volume 43, Issue 5, May 2008, Pages 792–796