Congenital Lobar Emphysema Developing Massive Hemoptysis in Adulthood Treated by Thoracoscopic Lobectomy.

Congenital lobar emphysema (CLE) is a rare developmental lung disorder characterized by lobar hyperinflation secondary to bronchopulmonary obstruction. Half of patients are symptomatic at birth, with many requiring urgent or emergent surgical resection to treat respiratory distress. Meanwhile, patients achieving late childhood or adolescence without symptoms usually never require surgery. We present a case of a 26 year old otherwise healthy female with known CLE who developed massive hemoptysis and required urgent videoscopic (VATS) resection of her right lung upper lobe. We know of no other report of CLE causing life-threatening bleeding at any age, and herein review pathology and pathophysiology of the condition.

Vanishing Lung Syndrome in a Young Male With Chronic Marijuana Use: A Case Report.

Vanishing lung syndrome (VLS) also known as type I bullae disease or idiopathic bullous disease is characterized by giant emphysematous bullae that commonly develop in the upper lobes, occupying at least one-third of a hemithorax. It is a progressive and irreversible condition that involves pulmonary parenchymal destruction and alveolar dilation. It is commonly associated with middle-aged tobacco smokers, habitual marijuana users, and those with alpha-1-antitrypsin deficiency. This case involves an incarcerated male in his 30s with chronic marijuana smoking who presented with a three-month history of right-sided chest pain accompanied by cough, hemoptysis, fever, and weight loss. The patient reported month-long atypical chest discomfort associated with a cough productive of bloody sputum and was brought to the ED after developing acutely worsening right-sided chest pain. The patient underwent a chest X-ray that revealed a large pneumothorax on the left. Subsequently, CT chest imaging showed extensive bilateral bullous disease, left upper lobe consolidation, and enlarged mediastinal lymph nodes. This case illustrates a rare presentation of VLS in the setting of a young patient who other than reported regular marijuana use had no other risk factors and a negative workup for possible etiologies that could cause his severe bullous emphysema, including alpha-1 antitrypsin, HIV, Sjogren's syndrome, pulmonary Langerhans cell histiocytosis, two sputum Mycobacterium tuberculosis tests, and acid-fast bacteria sputum cultures, which were all negative. Identifying and assessing the degree of disease early in this progressive disease helps guide treatment while preventing further deterioration of lung parenchyma.