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Pulmonary lymphangiectasis in Noonan syndrome
AJR. American Journal of Roentgenology
|January 1, 1980
Summary
Noonan syndrome patients with pulmonary lymphangiectasia often present with specific cardiac and radiographic findings. Surgical intervention requires careful consideration due to potential lymphatic complications.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Medical Genetics
Background:
- Noonan syndrome is a genetic disorder associated with various congenital anomalies.
- Pulmonary lymphangiectasia is a rare, potentially life-threatening condition characterized by dilated lymphatic vessels in the lungs.
Observation:
- Four patients with Noonan syndrome and pulmonary lymphangiectasia were analyzed.
- Common findings included superior QRS axis deviation, pulmonary stenosis, and atrial septal defects.
- Radiographs revealed prominent pulmonary interstitial markings and possible pleural effusions.
Findings:
- All patients exhibited a distinct electrocardiogram pattern (superior QRS axis deviation).
- Valvular pulmonary stenosis was a consistent cardiac anomaly, with septal defects also noted.
- Pulmonary lymphangiectasia manifested as dilated lymphatics on chest imaging.
Implications:
- Surgical repair of cardiac defects in these patients carries risks.
- Interruption of engorged pleural or mediastinal lymphatics during surgery can lead to increased morbidity.
- Understanding these associations is crucial for surgical planning and patient management.
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