Spontaneous Chylothorax in Noonan Syndrome

Authors:
  • DCDavid Campbell
    David Campbell
  • JWJames W. Wiggins
    James W. Wiggins
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Abstract
• Objective. —To describe a case of spontaneous chylothorax in a child with Noonan syndrome successfully treated with prednisone. Design. —Case report. Setting. —A pediatric cardiology referral center for the Rocky Mountain region. Patient. —An 18-month-old girl with Noonan's syndrome, pulmonary stenosis, and hypertrophic cardiomyopathy who presented with spontaneous chylothorax. Interventions. —The child's chylothorax did not respond to thoracic duct ligation, tetracycline pleurodesis, and pleurectomy during a 2-month period. A low-fat diet was helpful but did not eliminate the problem. Prednisone was started orally at 1 mg/kg per dose twice daily and slowly tapered during 3 months. The chylothorax did not recur during 8 months of follow-up. Conclusions. —Prednisone may be useful in the treatment of chylothorax in Noonan syndrome. A controlled clinical trial would be helpful but would be difficult in such a rare complication of an uncommon syndrome. (AJDC. 1992;146:1453-1456)

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