Spontaneous chylothorax in Noonan syndrome. Treatment with prednisone
M. B. Goens, D. Campbell and J. W. Wiggins
University 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.
