title: Spontaneous pneumothorax in a teenager with prior congenital pulmonary airway malformation.
abstract: Congenital pulmonary airway malformation (CPAM), previously referred to as congenital cystic adenomatoid malformation (CCAM), is a developmental malformation of the lower respiratory tract and the most commonly reported congenital lung lesion. Affected patients typically present with respiratory distress in the neonatal period from expanding cysts and resulting compression of surrounding lung parenchyma. However, some patients also remain asymptomatic until later in life. In this report, we present a case of CPAM requiring emergent left lower lobectomy at the first day of life that remained asymptomatic until the patient developed a spontaneous pneumothorax 18 years later. Our patient's presentation with an isolated spontaneous pneumothorax at age 18 does not appear to have been previously reported. In addition, there are several aspects of this case that represent atypical features of CPAM. After an extensive literature search, few reports exist describing any long-term complications of CPAM following neonatal lobectomy. Chest imaging in our patient demonstrated residual left basilar bullae and there was a moderate fixed obstructive/restrictive defect on pulmonary function testing. His risk for recurrent pneumothorax or infectious complications is unknown based on minimal published information on long-term outcomes or complications in patients with resected CPAM lesions. We conclude that follow up of all CPAM patients should include an evaluation for evidence of residual lung disease both with spirometric testing and chest imaging. Furthermore, concern for infectious complications or symptomatic obstructive lung disease should likewise be considered.
authors.affiliation: Internal Medicine Residency, Department of Medicine, San Antonio Military Medical Center, 3851 Roger Brooke Drive, Fort Sam Houston, TX 78234, USA.
authors.forename: Matthew P
authors.initials: MP
authors.lastname: Shupe
authors.affiliation: Pulmonary/Critical Care Service, Department of Medicine, Womack Army Medical Center, Fort Bragg, NC, USA.
authors.forename: Herbert P
authors.initials: HP
authors.lastname: Kwon
authors.affiliation: Pulmonary/Critical Care Service, Department of Medicine, Brooke Army Medical Center, Fort Sam Houston, TX, USA.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Morris
keywords: CCAM, congenital cystic adenomatoid malformation; CPAM, congenital pulmonary airway malformation; CT, computed tomography; Congenital pulmonary airway malformation; FEV1, forced expiratory volume at one second; FVC, forced vital capacity; LLL, left lower lobe; PTX, pneumothorax; Pulmonary; Spontaneous pneumothorax