Congenital cystic adenomatoid malformation of the lung in adults: Clinical and CT evaluation of seven patients

Abstract

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare congenital developmental abnormality that usually presents in childhood. Some associated malignancies have been reported. This study aimed to describe the clinical and multidetector CT (MDCT) image characteristics of CCAM of the lung in adults. Adult patients with congenital cystic lung diseases in association with surgery for CCAM were evaluated over a five and a half year period. Seven (four women, age range 17-64 years) of 109 congenital cystic lung disease patients were histologically confirmed as having CCAM. The most frequent symptom was productive cough (n = 5) and one patient was admitted with haemoptysis. The diagnosis was based on clinical and radiological findings and one patient was not diagnosed until surgery. MDCT images consisted of having a multiple loculated unilobar cystic mass in six patients and a cavitary mass in one and/or normal systemic arteries. The lesion was present in the right lung in four and in the left lung in three patients. The involved lobe was the upper in three, lower in three and middle lobe in one. Six patients underwent lobectomy and there was no associated malignancy or mortality. The mean length of hospital stay was 17.5 +/- 7.3 days. In adult patients who suffer from a recurrent productive cough and who have a multiloculated cystic mass in one lobe and normal vascular images in MDCT, CCAM, although rare, should be considered.