Report a case of congenital pulmonary lymphangiectasia with fetal pleural effusions
10.3969/j.issn.1000-3606.2015.08.008
- VernacularTitle:先天性肺淋巴管扩张症伴胎儿胸腔积液1例报告并文献复习
- Author:
Xiaolei ZHUANG
;
Jimei WANG
;
Xiaoyun ZHOU
;
Xiaoxia AN
;
Beiqian QIAN
;
Yingliu YAN
;
Min JI
- Publication Type:Journal Article
- Keywords:
pulmonary lymphangiectasia;
hydrops fetalis;
pleural effusions;
newborn
- From:
Journal of Clinical Pediatrics
2015;(8):710-714
- CountryChina
- Language:Chinese
-
Abstract:
ObjectiveTo summarize and review the clinical characteristics of congenital pulmonary lymphangiectasia with fetal bilateral pleural effusions.MethodsThe clinical data of a newborn diagnosed as congenital pulmonary lymphangiec-tasia with bilateral pleural effusions in Obstetrics and Gynecology Hospital, was summarized. The clinical, radiographic features, treatment and prognosis of this case are discussed in the context of the literature review.ResultsThe premature infants present-ed with rapid progression bilateral pleural effusions, respiratory distress, chylothorax, hypoalbuminemia and persistent pulmo-nary hypertension at birth. The pulmonary surfactant was given and mechanical ventilation was used for respiratory support. NO was inhaled, high-frequency mechanical ventilation was applied and albumin was repeatedly administered. After treatment for 3 months in the NICU, the patient was discharged. After 10 days, the patient was administered to the PICU with severe pneumo-nia, chronic lung disease, mechanical ventilation and anti-infection treatments were applied for 2 months. After living for six months, the baby died.ConclusionsCongenital pulmonary lymphangiectasia is extremely rare and prenatal diagnosis was dif-ifcult. The disease should be considered in patients presented with progressive dyspnea, interstitial emphysema after birth. Lung tissue biopsy and radioisotope scanning should be performed in time to get diagnosis.
