Congenital cystic adenomatoid malformation of the lung.

Congenital cystic adenomatoid malformation of the lung (CCAM) has been reported with increasing frequency since it was first described in 1949. In a review of the literature by Halloran and co-workers in 1972, 61 cases were reported. After ten years, we collected from the literature 279 cases. The malformation is characterized by a multicystic mass of lung tissue with bronchiolar proliferation and alveolar impairment. The clinical picture of this pulmonary malformation varies with the age at presentation. In the neonatal period the respiratory distress syndrome is often very serious and may require an urgent treatment. In the older infant, the unrecognized disease has more often a chronic course characterized by recurrent respiratory infections and failure to thrive. Sometime, the malformation may be completely silent and be disclosed during a routine examination of the thorax for others reasons. Both types of presentations of the cystic adenomatoid malformation require a surgical therapy and the lobectomy is the treatment of choice. The prognosis following resection in the older patients is generally good, while in the neonatal period the remaining hypoplastic pulmonary tissue may represent a problem in the postoperative ventilatory management. Two cases of congenital cystic adenomatoid malformation observed in a 5 months old and in a 10 months old infants are reported. The clinical, pathologic and radiologic features of this pulmonary malformation are discussed. The hypothesis of a future "in utero" correction of the most severe cases, in order to prevent the pulmonary hypoplasia of the healthy parenchyma, is also suggested.