Mele Patricia, Sridhar Shanthy
Division of Neonatology, Department of Pediatrics, Stony Brook Long Island Children's Hospital, New York 11794, USA.
Adv Neonatal Care. 2012 Jun;12(3):166-71. doi: 10.1097/ANC.0b013e3182569c61.
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.
先天性肺淋巴管扩张症（CPL）是一种罕见的肺部发育障碍疾病。CPL在产前可表现为伴有胸腔积液的非免疫性水肿，常伴有羊水过多。CPL在新生儿期表现为严重的呼吸窘迫，可并发乳糜胸。由于影像学表现不具特异性，诊断较为困难。在一些严重病例中，需要进行肺活检以诊断CPL。大多数患有这种疾病的婴儿很少能存活至新生儿期，往往在尸检时才被诊断出来。最终诊断通过肺活检组织学检查或死后检查得以证实。我们报告了一例罕见的CPL病例，该病例为一名早产女婴，表现为严重的非免疫性胎儿水肿，存活至新生儿期，并报告了至幼儿期的临床随访情况。
