Primary congenital pulmonary lymphangiectasia--a case report.

We report on a 22 year-old women with recurrent pleural effusions and shadowing of the right lower lobe, which was refractory to antibiotic treatment. Histologic examination (open lung biopsy) was interpretated as indicating an early stage of lymphangioleiomyomatosis. Because of progression of the pulmonary changes and development of a pericardial effusion in spite of antiestrogen treatment to achieve pharmacological castration, and in view of the atypical findings in high resolution computed tomography (lack of cysts) a second open lung biopsy was performed, confirming the diagnosis of pulmonary lymphangiectasia. The patient was given oral corticosteroids postoperatively and showed almost complete resolution of the pleural and pericardial effusions.