Report a case of congenital pulmonary lymphangiectasia with fetal pleural effusions / 临床儿科杂志

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.

Congenital Pulmonary Lymphangiectasia, Associated with Total Anomalous Pulmonary Venous Return

Congenital pulmonary lymphangiectasia (CPL) is very rare. It shows diffuse pulmonary lymphatic dilatation without lymphatic proliferation. CPL can occur as a primary disorder or arise secondarily from other diseases such as the obstruction of pulmonary veins or lymphatics. The prognosis of CPL is very poor. Approximately 50% of infants are stillborn and most others usually die within the first day of life. The present case showed diffuse lymphangiectasia in the subpleural, interlobular, and peribronchovascular areas. The flat lining cells were immunohistochemically positive for D2-40 and CD31. CPL is usually diagnosed by clinicoradiological or postmortem examinations. However, our case was diagnosed by an antemortem lung biopsy. We report a case of CPL with total anomalous pulmonary venous return.

A Case of Congenital Pulmonary Lymphangiectasia in Noonan Syndrome

Congenital pulmonary lymphangiectasia is a rare and perphaps underestimated cause of fatal respiratory distress in the neonate or infant. Pathologically, this condition is characterized by wide lymphatic channels in the subpleural and peribronchovascular spaces and the interlobular septa. Noonan syndrome is characterized by a phenotype similar to Turner syndrome but with a normal karyotype. Both pulmonary and intestinal lymphangiectasia have been reported in patients with Noonan syndrome. We have experienced a case of congenital pulmonary lymphangiectasia in Noonan syndrome, who presented at birth with cyanosis and persistent respiratory distress, and died on 93 postnatal days. We report this case, which was diagnosed by open lung biopsy and autopsy, with brief review of the related literature.