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Congenital pulmonary lymphangiectasia: an unusual presentation of nonimmune hydrops in a preterm infant.
MedLine Citation:
PMID:  22668688     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Congenital pulmonary lymphangiectasia (CPL) is a rare developmental disorder of the lung. CPL can present antenatally as nonimmune hydrops with pleural effusions, which are frequently associated with polyhydraminos. CPL presents in the neonatal period with severe respiratory distress and can be complicated by chylothorax. Diagnosis is difficult, as radiographic findings are nonspecific. Lung biopsy is indicated in some severe cases to diagnose CPL. The majority of infants with this condition rarely survive the neonatal period and are often diagnosed at autopsy. The final diagnosis is confirmed histologically by lung biopsy or at postmortem. We report a rare case of CPL in a preterm female infant presenting with severe nonimmune Hydrops fetalis who survived the neonatal period, and report clinical follow-up to early childhood.
Authors:
Patricia Mele; Shanthy Sridhar
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Advances in neonatal care : official journal of the National Association of Neonatal Nurses     Volume:  12     ISSN:  1536-0911     ISO Abbreviation:  Adv Neonatal Care     Publication Date:  2012 Jun 
Date Detail:
Created Date:  2012-06-06     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101125644     Medline TA:  Adv Neonatal Care     Country:  United States    
Other Details:
Languages:  eng     Pagination:  166-71     Citation Subset:  IM    
Affiliation:
Division of Neonatology, Department of Pediatrics, Stony Brook Long Island Children's Hospital, New York.
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