Congenital cystic adenomatoid malformation of the lung type II: Two case report

Congenital cystic adenomatoid malformation (CCAM) is a rare hamartomatous lesion of the lung, characterized by a cystic mass of dysplastic pulmonary tissue with proliferation of bronchial structures and loss of alveoli. From a clinical point of view, CCAM may present at birth (30-35%) with respiratory distress sometimes lifethreatening; another group of patients have a late onset (60-65%), with recurrent pulmonary infections or growth retard; the lesion can sometimes be completely asymptomatic and may only be observed with a fortuitous radiography. The evolution of the lesion may be various. Spontaneous prenatal reduction or resolution of CCAM is well reported in the literature, as well as postnatal spontaneous and complete resolution. The prognosis of neonatal CCAM is related to early diagnosis and relative treatment, and therefore is also related to the prognosis of pulmonary resection. The authors describe 2 cases of type II CCAM, observed at the Section of Pediatric Surgery of the University of Siena. The purpose of this study is to review the management in relation to the clinical onset and the type of the lesion.