[Pulmonary lymphangiectasis with spontaneous chylothorax in Noonan syndrome].
We report a case of Noonan syndrome associated with pulmonary stenosis and major lymphedema of the lower extremities. At the age of 15 yr spontaneous chylothorax with increasing dyspnea occurred> Chest-x-ray demonstrated increased interstitial markings restricted to the right lower lobe representing pulmonary lymphangiectasia. The chylothorax did not respond to repeated thoracocentesis and medium-chain-triglyceride diet. When a chest tube was inserted and total parenteral nutrition was supplied, the chylous effusion decreased within 32 days. The patient is still on diet and asymptomatic effusion remained during 12 months follow up. In conclusion, pulmonary lympgangiectasia should be considered in patients with Noonan syndrome and an abnormal interstitial pulmonary pattern similar to pulmonary congestion (without any hemodynamic abnormalities). In case of pleural effusion, chylothorax should be considered.
Sailer
M
M
Universitätsklinik für Kinderheilkunde Innsbruck.
Unsinn
K
K
Fink
C
C
Covi
B
B
Gassner
I
I
D000293:Adolescent; D015505:Chest Tubes; D002916:Chylothorax / Q000000981:diagnostic imaging / Q000235:genetics / Q000628:therapy; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008171:Lung Diseases / Q000000981:diagnostic imaging / Q000235:genetics* / Q000628:therapy; D008200:Lymphangiectasis / Q000000981:diagnostic imaging / Q000235:genetics*; D008209:Lymphedema / Q000000981:diagnostic imaging / Q000235:genetics / Q000628:therapy; D009634:Noonan Syndrome / Q000000981:diagnostic imaging / Q000235:genetics* / Q000628:therapy; D010289:Parenteral Nutrition, Total; D010996:Pleural Effusion / Q000000981:diagnostic imaging / Q000235:genetics* / Q000628:therapy; D011859:Radiography