Congenital Lobar Emphysema (CLE) is an uncommon pulmonary disorder in which one or more pulmonary lobes is over-inflated and distended. Fewer than 5% of cases are diagnosed beyond the first 6 months of life. We describe CLE that presented in a 25 yr old female who was successfully treated with minimally invasive video assisted thoracoscopic surgery (VATS).
Case ReportA 25-year old Puerto Rican female presented with increased shortness of breath, cough and chest pain. Her past medical history included diagnosis of asthma In Puerto-Rico at the age of five, treated with advair. During her third pregnancy, at the age of 23, the patient experienced shortness of breath and reported "fainting". A chest CT at that time diagnosed her with lobar emphysema.In the United States, she presented to an outside hospital with exacerbation of shortness of breath, where she was thought to have a pneumothorax, and a right chest tube was placed. She was then transferred to the Medical Center ICU. At presentation, She was afebrile, exhibiting tachycardia (117 bpm), with 28 respirations per minute, oxygen saturation of 91%, and decreased breath sounds on the right chest field.Pulmonary function test suggested of Restrictive Lung Disease (RLD) ( Table 1). The patient's initial X-rays, as seen in Figure 1, indicate hyperlucency on the right side, flattening of the right diaphragm, and a slight shift of mediastinum to the left. The right lung is collapsed inferiorly. There are a severe number of bullae within the right hemithorax, with some consolidation or collapse in the region of the right perihilar region.CT taken before surgery documented large bullae, mediastinal shift, and compressed right lung. (Figure 2 and 3).
Treatment and Surgical FindingsSince the patient's shortness of breath was not successfully managed with Oxygen support and chest tube drainage, she was scheduled for a bronchoscopy and a Video Assisted Thoracoscopic Surgery (VATS) -right upper lobectomy.
On BronchoscopyThe Right Upper Lobe (RUL) takeoff was a slit-like lumen, slightly distorted -confirms the diagnosis of CLE.VATS right upper lobectomy was performed with general endotracheal anesthesia with a double lumen tube and one lung ventilation. We used 3 ports; 10mm port in the seventh intercostal space in anterior axillary line, 10mm trocar in posterior axillary
Using a 10 mm 30 degree Olympus scopeThe right upper lobe composed entirely of large bullae. The bullae did not deflate and were densely adherent to the chest wall. There were also aberrant blood vessels from the chest wall to the bullae, with no apparent superior pulmonary vein, pulmonary artery, or bronchi going to the bullous portion. However, the segmental bronchi and major pulmonary vessels were found attached to the area of the hilum of the atritic lobe. An endoscopic EndoGIA (Covidian) used to divide the vessels and the rudimentary bronchus. The lobe removed with an endobag. A 28 french chest tube was left in the chest.
PathologyMicroscopic examination of the resected lobe showed dense fi...