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Spontaneous chylothorax in Noonan syndrome. Treatment with prednisone
M B Goens1, D Campbell, J W Wiggins
1University of Colorado School of Medicine, Denver Children's Hospital.
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.