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Pulmonary lymphangiectasia: Diagnosis and clinical course
Author(s) -
Esther Charles R.,
Barker Pierre M.
Publication year - 2004
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.20100
Subject(s) - medicine , etiology , lymphangiectasia , presentation (obstetrics) , disease , lymphatic system , rare disease , respiratory disease , pediatrics , lung , intensive care medicine , pathology , radiology
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. Pediatr Pulmonol. 2004; 38:308–313. © 2004 Wiley‐Liss, Inc.