Objective : Congenital cystic adenomatoid malformation (CCAM) is a rare congenital broncho-pulmonary lesion. The purpose of this study was to review the characteristics of the patients surgically treated for CCAM at our hospital.
Methods : Between 1979 and 1998, fifty six patients were operated on for a congenital broncho-pulmonary malformation including pulmonary sequestration (n = 19), bronchogenic cyst (n = 14), congenital lobar emphysema (n = 8), and congenital cystic adenomatoid malformation (n = 15). Hospital notes of patients with CCAM were reviewed.
Results : There were 11 male and 4 female patients with a mean (± SEM) age of 2 ± 1 years (0-13 years). In seven children a pulmonary mass was suspected in utero by prenatal ultrasound. Four neonates suffered from respiratory distress. Seven newborns were asymptomatic. Four infants (3-13 years) were treated for recurrent respiratory infections. The lesion was located in the left lung in 5 cases (upper lobe n = 2, lower lobe n = 3), and in the right lung in 10 cases (upper lobe n = 3, middle lobe n = 1, lower lobe n = 6). The type of parenchymal resection was an atypical segmental resection in 5 patients, a lobectomy in 9 patients and bilobectomy in 1 patient. Two patients (13%) had prolonged airleak, one after segmental resection with a residual cystic lesion on imaging. There has been no mortality. Hospital stay was 15 ± 3 days (8 – 47 days). According to the Stocker classification, 11 patients (73%) had a type I lesion and 4 patients had a type II lesion. No type III lesions were seen in this series. All patients were doing well at latest follow up (1-8 years).
Conclusions : CCAM accounts for 27% of all congenital broncho-pulmonary malformations seen at our hospital. Prenatal diagnosis was made in 47% of the patients. Urgent resection of the space-occupying mass in the newborn with respiratory distress can be lifesaving. Surgical removal is advocated in asymptomatic infants presenting with a cystic lesion on chest-x-ray in order to prevent recurrent respiratory infections or potential malignant degeneration at later age.