Linfangiectasia pulmonar congênita unilateral: um relato de caso

Abstract

Pulmonary lymphangiectasia is a rare developmental disorder in which lymphatic vessels proliferate and dilate. Based on pathogenesis and clinical phenotype, it can be classified as: primary or congenital pulmonary lymphangiectasia (CPL), or secondary. The diagnosis is made by clinical characteristic and imaging and histopathological exams features. Lung biopsy is considered the gold standard for the diagnosis of CPL and is also useful to distinguish between interstitial lung diseases or other forms of pulmonary lymphatic dilation. The definitive treatment of the isolated form can be curative with resection of the affected area. In diffuse form, the treatment is palliative and aims to limit the production and accumulation of lymph in the pulmonary serosa. Currently, the conservative treatment is recommended for chylothorax correction. This article aims to report the case of a patient with a diagnosis of congenital pulmonary lymphangiectasia with good evolution, emphasizing the importance of early diagnosis and therapeutic approach, and to compare with what has been described in the literature.

Keywords

• pulmonary lymphangietasia
• newborn
• lymphatic vessels
• child