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Congenital Pulmonary Lymphangiectasia: CT and Pathologic Findings

Nobre, Luiz Felipe MD*; Müller, Nestor L. MD, PhD; de Souza , Arthur Soares Jr. MD.; Marchiori, Edson MD§; Souza, Irene Vieira MD

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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.

From the *Department of Radiology, Universidade Federal de Santa Catarina, Florianopolis, Santa Catarina, Brasil; the †Department of Radiology, Vancouver General Hospital, University of British Columbia, Vancouver, BC, Canada; the ‡Faculty of Medicine, São José do Rio Preto, Sao Paulo, Brasil; the §Department of Radiology, Universidade Federal Fluminense, Rio de Janeiro, Brasil; and the Department of Pathology, Universidade Federal de Santa Catarina, São José, Santa Catarina, Brasil.

Reprints: Dr. Nestor L. Müller, Department of Radiology, Vancouver General Hospital, 899 W. 12th Ave, Vancouver, BC Canada V5Z 1M9 (e-mail: nmuller@vanhosp.bc.ca).

© 2004 Lippincott Williams & Wilkins, Inc.