Our patient is a 15-year old Caucasian basketball player with a well-established diagnosis of SJMS. In the age of five years he had a serious pulmonary infection with Mycoplasma Pneumoniae for which he had to hospitalize. After his exit from the hospital and up to the age of ten years he had repeated episodes of pulmonary infections with bronchitis and dyspnea. It was that time were the diagnosis SJMS was made due to bronchiolitis obliterans and resulting emphysema. The radiologic exams were unremarkable. Chest X- ray showed a hyperlucency on the left pulmonary field with the left lung being relatively smaller than the right. Upon lung function tests the child exhibited measurements of lung obstructive disorder with decreased forced expiratory volume in 1 sec (FEV1) and forced vital capacity (FVC) in relation to the normal value considering his age and somatometric parameters. Moreover, the ratio of (FEV1) to FVC (FEV1/FVC) was decreased, indicating airway obstruction. Pulmonary scintigraphy with 99mTc showed decreased perfusion of the affected lung mainly at the apex, whereas the right lung remained unaffected. The patient continued to exhibit recurrent episodes of bronchial infections up to the age of ten. Since then and for the next five years he remained asymptomatic.
One year ago the patient was admitted in the emergency department complaining of sudden onset of dyspnea accompanied by pleuritic chest pain on the left side. He has been feeling well over the past days, was afebrile and reported no history of recent trauma. Auscultation revealed decreased breath sounds on the left. A chest X-ray followed revealing spontaneous pneumothorax on the left side. Immediate insertion of an 18 Fr thoracic chest tube in an apical position for the evacuation of air and the patient was transferred to the ward for further hospitalization. During the next few days the patient continued to exhibit significant amount of air in the underwater seal mainly during coughing and the chest X-rays showed that the pneumothorax still existed; although significantly decreased in size. Chest CT angiography followed showing an emphysematous bulla 3 cm in diameter in the left upper lobe with partially expanded lung and small remaining pneumothorax. Additionally, hyperlucency of the left upper lobe with decreased vascularity in concordance with SJMS were also present (Figures  1 and 2). Patient's underwater seal drain was changed and connected to negative suction in order to remove the remaining air and expand the lung. He remained on negative suction for the following seven days with no radiological or clinical improvement; therefore, surgical therapy was decided.
Under general anaesthesia using double lumen endotracheal tube, VAT- bullectomy of the left upper lobe was carried out through 3 access ports that were 5.5, 5.5- and 20-mm in diameter. Bovine pericardial sleeve used to buttress and reinforce the staple line. Apical pleurectomy just over 5 cm followed to achieve mechanical pleurodesis.
Postoperatively patient's underwater seal continued to remain connected to mild negative suction (10-15 cmH2O) for five days in order to accomplish full pulmonary expansion since he was emphysematous with associated obstructive bronchiolitis and decreased pulmonary elasticity. No postoperative air leak was observed. During his stay we observed minimal apical space when the patient was off suction. Therefore we decided to connect the intercostal drain to a one flutter valve bag (Portex bag) and we discharge him home to optimize his mobilization and treat the minimal apical space. The chest drain was on the 15th postoperative day. Chest X ray after removal showed fully expanded pulmonary parenchyma. One year postoperatively he is in excellent clinical condition and able to cope fully with his basketball activities. No recurrence of spontaneous pneumothorax is observed.
CT scan angiography of the same patient. Note the decreased vascularity of the left lung with hyperlucent upper lobe and presence of apical bulla. The left lung is smaller in comparison to the right.