Congenital pulmonary lymphangiectasia: CT and pathologic findings.
Congenital pulmonary lymphangiectasia is a rare disease characterized by dilation of lymphatic channels without lymphatic proliferation. The disease is seen almost exclusively in infancy and early childhood. The authors report 2 cases of pulmonary lymphangiectasia. The patients were a 12- and a 25-year-old male who presented with progressive dyspnea and hemoptysis. The diagnosis was confirmed by open lung biopsy. The radiographic findings consisted of bilateral reticular changes, peribronchial cuffing, and bilateral pleural effusions. High-resolution CT demonstrated extensive bilateral septal and peribronchovascular interstitial thickening, areas of ground-glass attenuation, and bilateral pleural effusions. The histologic findings consisted of ectatic and tortuous lymphatic channels in the interlobular septa, bronchovascular sheaths, and pleura.
Nobre
Luiz Felipe
LF
Department of Radiology, Universidade Federal de Santa Catarina, Florianopolis, Santa Catarina, Brasil.
Müller
Nestor L
NL
de Souza Júnior
Arthur Soares
AS
Marchiori
Edson
E
Souza
Irene Vieira
IV
D000328:Adult; D001706:Biopsy; D002648:Child; D006801:Humans; D008171:Lung Diseases / Q000151:congenital / Q000000981:diagnostic imaging* / Q000473:pathology; D008200:Lymphangiectasis / Q000151:congenital / Q000000981:diagnostic imaging* / Q000473:pathology; D008297:Male; D014057:Tomography, X-Ray Computed / Q000379:methods*