Introduction:Congenital lobar emphysema (CLE) is a rare developmental lung malformation that involves the hyperaeration of one or more lung lobes due to partial obstruction and occurs at a rate of 1/20,000–30,000 live births.Here,we aimed to retrospectively examine the clinical, radiological, and bronchoscopy findings of patients with CLE who were diagnosed and treated with surgical or conservative approaches in our clinic to compare our results with those in the literature. Method:We examined the clinical, and radiological data and FB findings of the patients with CLE aged 0–18 years at our center between 2013 and 2020.We also examined the symptoms and findings recorded during the patients’ follow-up. Results:.The median age of 20 patients with CLE at diagnosis was 3.2 years (range, 1 day–17 years).Respiratory distress and mediastinal shift were more prominent in the patients who underwent surgery than the patients who were followed up conservatively and diagnosed at an early age (p = 0.001, p = 0.049, p = 0.001, respectively). Discussion: In line with studies in the literature, the pulmonary symptoms and CLE-related imaging findings in our study regressed during the conservative follow-up.We recommend clinicians consider performing a detailed anamnesis for patients with unresolved respiratory symptoms and unilateral or bilateral increased ventilation,along with appropriate imaging tests and examinations, and should consider CLE in the diagnosis.

A twenty-five days old girl, admitted to pediatric pulmonology clinic, with collapse on the anterior chest wall while breathing. Physical examination revealed total retraction of the chest wall especially on the upper part, paradoxical chest wall movements with respiration, and a raphe extending from the umbilicus to the lower end of the sternum. Hypoplasia of the inferior and non-development of the superior segment of the sternum was shown on 3-dimensional(3D) thorax CT. At the age of fifty-six days, she had been operated. Due to the large defect, primary closure couldn’t be performed and the sternum was reconstructed with the cartilage obtained from the tissue around the ribs. The sternal cleft is a rare malformation; complete assessment, based primarily on thoracic imaging is required for diagnosis. Also cardiac, abdominal and cranial imaging for concominant malformations should be performed; our patient had none. Immediate surgical management is essential to avoid possible complications.