Congenital pulmonary lymphangiectasia: an unusual presentation of nonimmune hydrops in a preterm infant
Autor: | Shanthy Sridhar, Patricia C. Mele |
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Rok vydání: | 2012 |
Předmět: |
Lung Diseases
Pediatrics medicine.medical_specialty Lymphangiectasis Hydrops Fetalis Autopsy Lung biopsy Infant Premature Diseases Chylothorax Rare Diseases Hydrops fetalis medicine Humans Lung Respiratory distress business.industry Infant Newborn General Medicine medicine.disease Developmental disorder medicine.anatomical_structure Pediatrics Perinatology and Child Health Female Presentation (obstetrics) business Infant Premature |
Zdroj: | Advances in neonatal care : official journal of the National Association of Neonatal Nurses. 12(3) |
ISSN: | 1536-0911 |
Popis: | 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. |
Databáze: | OpenAIRE |
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