Congenital cystic disease of the tracheobronchial tree in infants and children. Experience with 44 consecutive cases.

BACKGROUND: Cystic anomalies of the tracheobronchial tree in infants and children are usually of congenital origin and consist of pulmonary sequestration (PS), cystic adenomatoid malformation (CAM), congenital lobar overinflation (CLO), and bronchogenic cyst (BC). These four anomalies present a different clinical picture, are often difficult to diagnose, and require surgical management.

PATIENTS: From December 1974 to April 1993, 44 patients were operated on for congenital cystic disease of the tracheobronchial tree. There were 25 male patients (57%) and 19 female patients (43%), ranging in age from 1 day to 18 years. Thirty-three children (75%) were younger than 1 year and 31 (70%) were younger than 6 months at the time of surgery. There were 17 CAMs (38%), nine intralobar (20%) and six extralobar (13%) PSs, seven CLOs (16%), and six BCs (13%). All patients with CAM, CLO, and intralobar PS were symptomatic and presented with either progressive respiratory distress or recurrent pulmonary infections with cystic changes noted on chest roentgenograms. Computed tomography was the most definitive diagnostic study performed, aided by selective use of ultrasonography and arteriography.

RESULTS: All patients with CAM, CLO, and intralobar PS were treated with lobectomy; no segmentectomies were performed. Extralobar PSs and BCs were managed with resection of the cyst alone. There was one death (2%) in this series in a 1-day-old infant with a CAM who died of bilateral pulmonary hypoplasia. The remaining 43 patients (98%) are alive and well to date, with follow-up ranging from 2 months to 19 years. None of the patients who underwent a lobectomy have suffered any physical limitations during subsequent growth and development.

CONCLUSIONS: Congenital cystic disease of the tracheobronchial tree can be diagnosed with computed tomography and selective use of ultrasonography and arteriography. Lobectomy is the appropriate treatment for CAM, CLO, and intralobar PS and is well tolerated without any subsequent long-term limitation in physical activity.