—To describe a case of spontaneous chylothorax in a child with Noonan syndrome successfully treated with prednisone.
—A pediatric cardiology referral center for the Rocky Mountain region.
—An 18-month-old girl with Noonan's syndrome, pulmonary stenosis, and hypertrophic cardiomyopathy who presented with spontaneous chylothorax.
—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.
—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)
Goens MB, Campbell D, Wiggins JW. Spontaneous Chylothorax in Noonan SyndromeTreatment With Prednisone. Am J Dis Child. 1992;146(12):1453–1456. doi:10.1001/archpedi.1992.02160240063021
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