Advances in neonatal care : official journal of the National Association of Neonatal Nurses
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Review Case Reports
Congenital Chylothorax: A Unique Presentation of Nonimmune Hydrops Fetalis in a Preterm Infant.
Nonimmune hydrops fetalis secondary to congenital chylothorax (CC) is a rare disease process associated with high morbidity and mortality related to abnormal formation of the lymphatic system and disrupted management of fetal fluid. Hydrops fetalis is typically diagnosed prenatally by the presence of pleural effusions or other fluid collection on ultrasonography. Congenital chylothorax is diagnosed when the analysis of pleural fluid is deemed chylous. Neonatal presentation is often respiratory distress secondary to lung compression or pulmonary hypoplasia. Management ranges from supportive medical management such as high-frequency ventilation, chest drainage, and nutrition support, to controversial therapies such as octreotide administration and chemical pleurodesis, to surgical interventions such as thoracic duct ligation and mechanical pleurodesis. ⋯ Nonimmune hydrops fetalis secondary to CC presents a complex challenge for neonatal providers, as no definitive treatment strategy currently exists. Further research is needed to determine the safety and efficacy of controversial therapies including octreotide.