Imaging findings in pulmonary lymphangiectasia

We present the imaging findings of 4 newborns and 1 child affected by pulmonary lymphangiectasis (PL). Congenital PL is a rare developmental disorder involving the lung. Pulmonary lymphangiectasis is characterized by pulmonary subpleural, interlobar, perivascular, and peribronchial lymphatic dilatation. Both frequency and etiology are unknown. At birth, PL presents with severe respiratory distress, tachypnea, and cyanosis, with a very high mortality rate at or within a few hours of birth. Bilateral lung reticular appearance, peribronchial cuffing, and bilateral pleural effusions on radiographic chest evaluation are very suggestive of PL. Bilateral septal and peribronchial interstitial thickening are well evidentiated by high-resolution CT. Radiological studies, together with history and clinical data may lead to a diagnosis of PL in most cases. Lymphoscintigraphy, bronchoscopic and pleural effusion evaluation, and if necessary, lung biopsy are useful tools for confirming PL diagnosis.