The Case of Vanishing Lung

Abstract

INTRODUCTION: Idiopathic giant bullous emphysema, or “Vanishing Lung syndrome” (VLS), is a rare disease characterized by radiographic findings of bullae occupying at least one third of the thorax. The syndrome was first described in 1937 by Burke, with no clear consensus on treatment or management of this clinical entity. Very few case reports exist in the literature regarding vanishing lung syndrome. Here we are describing a case of a pregnant, asymptomatic, nonsmoking patient who was found to have this condition during a routine prenatal exam. CASE PRESENTATION: A 31 year old non-smoker female G3P2 presented for routine prenatal care. She complained of mild nonproductive cough, which had been progressively worsening during pregnancy. She denied fever, chest pain, or dyspnea. Her prenatal testing included negative PPD and HIV test. She rejected history of any respiratory diseases. On physical exam, blood pressure was 120/86, heart rate-76, respiratory rate-12, and O2 Sat. of 100% on room air. She had diminished breath sounds on the left with normal breath sounds on the right. A chest X-ray was done which was interpreted by radiology as a tension pneumothorax. Patient was immediately transferred to the Emergency department with a pulmonary and thoracic surgery consultation. Patient remained hemodynamically stable and a CT scan of the chest was performed, which revealed a giant bullous disease involving, almost, the entire left hemithorax. Spirometry did not show obstruction, but FEV1 and FVC were diminished. Patient was taken for an elective Cesarean section with a scheduled resection of the bullae. Complete absence of perfusion was noted on ventilation-perfusion (VQ) scan. Bronchoscopy revealed normal airways without any obstructions. Patient was taken to the operating room and resection of bullae and chest tube placement were performed. The patient was discharged home after chest tube removal on day 5 of the surgery. She returned for follow-up in two weeks with complaints of fever, chest pain, and dyspnea on exertion. A repeat CT scan showed a complex hydropneumothorax with loculations, bronchopleural fistula, and a mediastinal shift to the opposite side. The patient was taken back to the operating room, where she was found to have an empyema with one liter of purulent fluid. A pneumonectomy was performed with prolonged irrigation of thoracic cavity with antibiotics. Pathology findings were consistent with empyema, but all cultures were negative. Patient symptoms improved and she was discharged home with follow-up in pulmonary clinic. DISCUSSIONS: Here we have described a case of VLS in an asymptomatic nonsmoking, pregnant woman, though this uncommon disease usually affecting young cigarette smoking men. Our findings were initially confused for a tension pneumothorax. Since the patient's remained stable, further imaging was able to be obtained. Despite the absence of progressing symptoms, the danger of potential complication of VLS in woman on 32nd week of gestation justified elective C-section followed by bullae resection. Unfortunately, two weeks later the patient returned to clinic with hydropneumothorax and empyema. That worsened her quality of life and ultimately led to pneumonectomy. To date, few case reports and retrospective studies on VSL appear in the literature. Standards of treatment have not been established. Limited published data indicate that resection of the bullae should be performed only if adjacent lung tissue might reexpand. The best results are seen in symptomatic patients with poor exercise tolerance. CONCLUSION: VLS is a rare clinical entity with variable clinical presentation. Tension pneumothorax is a potential life threatening complication of this syndrome. In the absence of a pneumothorax, the indications to surgical intervention should be individually assessed thoroughly balancing benefits and hazards of the procedure. Specifically risks factors of spontaneous pneumothorax in VLS patients have to be identified in future.