Purpose: To analyse the state of the art of diagnostic imaging in the rare disease entity known as congenital bronchial atresia (CBA) and to suggest new guidelines for diagnosis.

Materials and methods: From January 2002 to December 2003 we examined 6 patients, 4 males and 2 females. Four of them complained of relapsing bronchitis; one patient was admitted with a diagnosis of pulmonary abscess. All patients underwent chest X-ray and MDCT.

Results: Five patients presented at chest x-ray the typical association of hilar mass and distal parenchymal hyperinflation that raised the suspicion of CBA; the remaining patient presented an area of parenchymal translucency that prompted us to perform MDCT, which showed severe stenosis of the segmental bronchus. CBA involved the left upper lobe (LUL) in 4 patients and the right upper lobe (RUL) in the remaining 2. In 50% of cases there were associated anomalies: distal bronchiectasis, bronchogenic cyst, anomaly of branching of bronchial tree and vascular structure.

Conclusions: CBA is a relatively rare, generally oligosymptomatic malformation. Knowledge of the signs present at chest X-ray can prompt the radiologist to perform a CT scan. MDCT with multiplanar reconstruction is fundamental for the characterisation, localisation and study of the distribution of the lesion, helping to make a precise diagnosis. Surgery is seldom required and at any rate only performed in case of complications.