Pulmonary lymphangiectasia: Diagnosis and clinical course

Pulmonary lymphangiectasia (PL) is a rare disease characterized by dilation of the normal lung lymphatics. The disease was previously associated with a poor prognosis. However, several recent case reports and case studies indicate that survival is possible in the severe neonatal form of PL, and the prognosis is good for infants who present after the neonatal period. In this report, we describe the etiology, clinical presentation, diagnosis, management, and outcome of PL. A revised classification of PL is proposed, based on the current state of knowledge of its etiology and presentation.