Congenital cystic adenomatoid malformation of the lung in the adult - Report of 2 cases

Congenital cystic adenomatoid malformation (CCAM) of the lung is a developmental anomaly characterized by the proliferation of terminal bronchiole-like structures with formation of cysts of varying sizes. The vast majority of cases are diagnosed in the early neonatal period as respiratory distress develops soon after birth. Adult cases are very rare. Only about 24 cases have been reported in the English literature to date. We report two adult cases of CCAM: one with multiple cystic lesions in the right lower lobe, the other with repeated infections in the right upper lobe. Progressive air trapping in the malformed lung led to impaired ventilatory performance in the first case. The lesion caused repeated episodes of respiratory infection in the second case. Both patients underwent surgical resections: right side pneumonectomy and right upper lobectomy respectively. Good clinical results were achieved. According to our experiences and review of the literature, we conclude that although CCAM is very rare in adults, it should be included in the differential diagnosis of cystic lesions of lung. Complete surgical resection of the involved lobes is the only effective treatment.