Adult congenital lung disease

Abstract

Congenital lung anomalies are increasingly diagnosed in adults, because they are missed in childhood, or as a consequence of incomplete resections at a younger age. They have unique manifestations, often mimic other thoracic pathology, and can present acutely and necessitate emergent evaluation and management. Misdiagnosis is common. This article deals with the patterns of presentation, clinical and radiological diagnosis, and the management of these disorders in adults. The lesions analysed include congenital cystic adenomatoid malformation, sequestration, congenital lobar emphysema and bronchogenic cysts. It is important to include these malformations in the differential diagnosis of adult thoracic pathology and to understand the modes of presentation, potential complications and management strategies. The radiological diagnosis is reliably made by computed tomography and/or magnetic resonance imaging. The potential for malignancy in these lesions is an important consideration. The management of asymptomatic lesions was considered controversial, but more lesions are now resected early, in recognition of the potential for subsequent complications. Complete resection is recommended. In contradistinction to childhood malformations, these lesions are more amenable to thoracoscopic resections, provided they are completely benign. Congenital lung malformations call for well-organized cooperation between paediatric and adult pulmonologists to ensure a smooth medical transition from childhood to adulthood.