Congenital pulmonary airway malformation in adult: report of two rare cases and literature review

Abstract

Congenital pulmonary airway malformations (CPAMs) are a heterogeneous group of congenital, non-hereditary lesions of the lung, cystic and non-cystic type with clear developmental anomalies and evident malformative changes, which derive from the excessive proliferation of the tubular bronchial structures. We described two rare cases of type IV CPAM detected in adult patients with different clinical presentation: one of these cases was symptomatic, complaining of cough and hemoptysis. Conversely, the other case was reported as incidental findings in patient with abdominal pain without respiratory symptoms. Chest computed tomography (CT) scan was performed showing voluminous cystic formations with fibrotic septa within the lesions in both patients. The patient with hemoptysis was subjected to video assisted thoracoscopic lobectomy, while the other one was hospitalized to perform biopsy, which confirmed the diagnosis of CPAM. All patients were advised to follow-up. The incidence of type IV CPAM in adult patients is probably underestimated; the knowledge of the CT features of CPAM is crucial for a correct diagnosis and an appropriate management of asymptomatic patients, which may be conservative.