Search

Objectives Congenital pulmonary lymphangiectasia (CPL) is a rare but fatal disorder of the lung. Case presentation We describe the case of an extremely low birth weight (ELBW) infant who presented with severe respiratory distress and recurrent bilateral pneumothorax. He died on day 17. The post-mortem examination of the lungs showed numerous cystic spaces, ranging from 1 to 2 mm in size, in the visceral pleura, in the thickened interlobular septum and hilum. A diagnosis of primary CPL was made. Conclusions We intend to underline that CPL, albeit rare, must be one of the differential diagnoses in infants with severe neonatal respiratory distress not responding to intensive care. Chronic interstitial lung diseases are a challenging diagnostic clinical problem, which requires a multidisciplinary approach. Histological lung examination may be useful to demonstrate the presence of CPL.

Pulmonary pneumatocele is a thin-walled, air-filled cyst originating spontaneously within the lungs’ parenchyma, generally after infections or prolonged mechanical respiratory support. The diagnosis of pneumatocele is usually made using both chest X-ray (CXR) and computed tomography (CT) scan. Lung ultrasonography (LUS) is a promising technique used to investigate neonatal pulmonary diseases. We hereby present two cases of pneumatocele in newborns with respiratory distress syndrome (RDS) in which CXR and LUS were used to evaluate pulmonary parenchyma. LUS showed a multilobed cyst with a thin hyperechoic wall and a hypoechoic central area. Repeated LUS demonstrated a progressive reduction of the cyst’s size. After a few weeks, the small lesions were no longer detectable by ultrasound, therefore CXR was used, for follow-up, in the following months, until complete resolution. No data are available in the literature regarding ultrasonographic follow-up of neonatal pneumatocele. A larger number of patients are required to confirm our results and increase the use of LUS in the neonatal intensive care units (NICUs) to reduce neonatal radiations exposure.