Congenital cystic adenomatoid malformation of the lung associated with bronchial atresia involving a different lobe in an adult patient: a case report

• Published in: Journal of medical case reports 2010-05, Vol.4 (1), p.164-164, Article 164
• Main Authors: Discioscio, Valerio, Feraco, Paola, Bazzocchi, Alberto, Femia, Rayka, Romeo, Chiara, Fasano, Luca, Pacilli, Angela M, Zompatori, Maurizio
• Format: Article
• Language: English
• Subjects: Care and treatment; Case report; Case studies; Diagnosis; Genetic disorders; Respiratory distress syndrome; Risk factors
• ISSN: 1752-1947; 1752-1947
• DOI: 10.1186/1752-1947-4-164

Congenital 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. We 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. Although 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.