Pulmonary lymphangiectasia demonstrated by lymphangiography in 2 patients with Noonan's syndrome

Two cases of pulmonary lymphangiectasia associated with Noonan's syndrome are described. Chest films showed diffuse pulmonary intersitial infiltrates from infancy. Lymphangiograms demonstrated obstructive changes and collateral formation in the retroperitoneal, mediastinal, pulmonary, and cervical lymphatics and extensive opacification of the pulmonary and visceral pleural lymphatics. Possible mechanisms and causes of the lymphatic malformation are discussed.