Abstract
IntroductionCongenital cystic adenomatoid malformation of the lung is an uncommon cause of respiratory distress in neonates and babies. The disorder is usually diagnosed in the neonatal period and the first two years of life. This anomaly has been described in association with bronchopulmonary sequestration, extralobar intra-abdominal sequestration or bronchial atresia in live and stillborn babies. It is rarely encountered in adults, in whom the diagnosis is made incidentally from mass lesion features seen on chest radiographs. The oldest patients recorded with this malformation have been about 35 years old, and only 10% of primary diagnoses are made after the first year of life. Delayed diagnosis can be related to infection or serendipitous discovery.Case presentationWe describe the radiological findings of a 34-year-old Caucasian woman with a clinical history of recurrent pneumonia, intermittent anterior pleuritic chest pain and haemoptysis. Congenital cystic adenomatoid malformation of the lung associated with bronchial atresia involving a different lobe was discovered.ConclusionAlthough rare in adults, congenital cystic adenomatoid malformation should be suspected in adult patients who suffer from recurrent or persistent non-productive coughs. The discovery of an association of congenital cystic adenomatoid malformation with bronchial atresia in adulthood is rare but possible, even in different lobes.
Highlights
Congenital cystic adenomatoid malformation of the lung is an uncommon cause of respiratory distress in neonates and babies
Conclusion: rare in adults, congenital cystic adenomatoid malformation should be suspected in adult patients who suffer from recurrent or persistent non-productive coughs
About 46 cases of cystic adenomatoid malformation (CCAM) diagnosed in adulthood have been reported in the English literature up to now but none in association with bronchial atresia (BA) involving a different lobe [2]
Summary
The evaluation of cystic or multicystic lung disease in adults requires the consideration of a differential diagnosis and the investigation of acquired lesions, such as lung abscesses, cavitary neoplasms or inflammatory masses, bullous diseases, bronchiectases and post-inflammatory pneumatoceles. Clinical and histological correlations are essential in establishing a diagnosis but radiological studies can be definitive. This is the first report of a diagnosis in adulthood of BA and CCAM with the involvement of different lobes. 2. Morelli L, Piscioli I, Licci S, Donato S, Catalucci A, Del Nonno F: Pulmonary congenital cystic adenomatoid malformation, type I, presenting as a single cyst of the middle lobe in an adult: case report. Author Details Imaging Section, Department of Radiologic and Histocytopathologic Sciences, University of Bologna, S.
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