Spontaneous chylothorax in Noonan syndrome. Treatment with prednisone.

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.

• Authors: M B Goens, D Campbell, J W Wiggins
• Journal: American journal of diseases of children (1960) (Am J Dis Child) Vol. 146 Issue 12 Pg. 1453-6 (Dec 1992) ISSN: 0002-922X [Print] United States
• PMID: 1456257 (Publication Type: Case Reports, Journal Article, Review)

Chemical References

• Prednisone

Topics

• Chylothorax (diagnostic imaging, therapy)
• Drainage
• Female
• Humans
• Infant
• Noonan Syndrome (complications)
• Prednisone (therapeutic use)
• Radiography