An 18 year old male presented to the emergency department at our institution with complaints of sudden onset left-sided pleuritic chest pain and dyspnea while sitting in church. His past medical history was significant for neonatal respiratory distress due to congenital lung disease requiring an emergent left lower lobe (LLL) resection the day following his birth. According to his father, he was reported to have an isolated type I CCAM. His past medical history was notable for a marfanoid body habitus, pectus excavatum, and double-curved

scoliosis requiring intervertebral rod placement. Despite these physical findings, the patient noted lifelong participation in sports activities with minimal limitation. He denied any previous symptoms of dyspnea, cough, or productive sputum. No further evaluation had been performed selleckchem since infancy based on a lack of pulmonary symptoms. At initial presentation, the patient was breathing comfortably with no respiratory distress. Vital signs demonstrated he was normotensive with a heart rate of 80 and oxygen saturation of 100% on room air. Physical examination was

only revealing for diminished breath sounds over the posterior left hemithorax. Chest radiograph revealed a left apical PTX selleck products measuring 1.9 cm from the pleural surface (see Fig. 1). A large-bore 32 French left chest tube was placed by the general surgery service and the patient was admitted for drainage of the PTX. His hospital course was unremarkable and the tube thoracostomy was removed after four days with minimal residual left apical air. During follow-up evaluation several months later in the Pulmonary Clinic, the patient had returned to college where he described a recurrent episode of pleuritic chest pain lasting one hour for which he did not seek medical attention. He otherwise continued to be asymptomatic with no pulmonary symptoms and no limitation in physical activities. Pulmonary function testing revealed

a mixed obstructive/restrictive defect with a forced expiratory volume at one second (FEV1) of 2.65 (54% predicted), forced vital capacity (FVC) of 3.78 (65% predicted), FEV1/FVC ratio of 70%, reduction in total lung capacity to 5.34 (67% predicted), and diffusing capacity of 25.2 (72% predicted). There was no bronchodilator Arachidonate 15-lipoxygenase response suggesting fixed obstruction. Repeat chest radiograph showed resolution of the left apical PTX and stable appearance of hyperexpanded lungs with a left basilar bleb. Computed tomography of the chest showed multiple left-sided peripheral and basilar bullae of varying sizes but no continued evidence of pleural air (see Fig. 2). The original pathology report was located by his father and the stated surgical pathology was the following: “Congenital cystic adenomatoid malformation (Type I) of the lung, clinically involving left lower lobe, with compressive atelectasis and acute interstitial emphysema.