CONGENITAL CYSTIC-DISEASE OF THE TRACHEOBRONCHIAL TREE IN INFANTS ANDCHILDREN - EXPERIENCE WITH 44 CONSECUTIVE CASES

Background: Cystic anomalies of the tracheobronchial tree in infants a nd 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 anom alies present a different clinical picture, are often difficult to dia gnose, and require surgical management. Patients: From December 1974 t o April 1993, 44 patients were operated on for congenital cystic disea se of the tracheobronchial tree. There were 25 male patients (57%) and 19 female patients (43%), ranging in age from 1 day to 18 years. Thir ty-three children (75%) were younger than 1 year and 31 (70%) were you nger than 6 months at the time of surgery. There were 17 CAMs (38%), n ine intralobar (20%) and six extralobar (13%) PSs, seven CLOs (16%), a nd six BCs (13%). All patients with CAM, CLO, and intralobar PS were s ymptomatic and presented with either progressive respiratory distress or recurrent pulmonary infections with cystic changes noted on chest r oentgenograms. Computed tomography was the most definitive diagnostic study performed, aided by selective use of ultrasonography and arterio graphy. Results: All patients with CAM, CLO, and intralobar PS were tr eated with lobectomy; no segmentectomies were performed. Extralobar PS s 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 o f bilateral pulmonary hypoplasia. The remaining 43 patients (98%) are alive and well to date, with follow-up ranging from 2 months to 19 yea rs. None of the patients who underwent a lobectomy have suffered any p hysical limitations during subsequent growth and development. Conclusi ons: Congenital cystic disease of the tracheo bronchial tree can be di agnosed 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.