Produção Nacional
Revisado por pares
Congenital Pulmonary Lymphangiectasia
2004; Lippincott Williams & Wilkins; Volume: 19; Issue: 1 Linguagem: Inglês
10.1097/00005382-200401000-00012
ISSN1536-0237
AutoresLuiz Felipe Nobre, Nestor L. M ller, Arthur Soares Souza, Edson Marchiori, Irene Vieira Souza,
Tópico(s)Tracheal and airway disorders
ResumoCongenital 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.
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