The unpredictable character of congenital cystic lung lesions

Background: The spectrum of congenital cystic disease of the lung ranges fr om hydrops and neonatal respiratory distress to asymptomatic lesions. Surgi cal management is dictated by the presence of symptoms, recurrent infection , and the potential risk of malignant transformation. Methods: Since 1995, all consecutive patients with congenital cystic lung l esions underwent follow-up for symptoms, treatment, and correlation of pres umptive with pathological diagnosis. Results: Twelve cystic lung lesions were identified. Seven were diagnosed w ith mediastinal shift in utero: in 6 of 7, the shift subsequently resolved. Overall, 6 of 7 lesions that were followed up serially decreased in size. Two patients were symptomatic in utero: 1 underwent thoracoamniotic shuntin g, 1 pleurocentesis for impending hydrops. Postnatally, these 2, and 2 othe r newborns required urgent surgery. Five of 8 asymptomatic patients had ele ctive resection by 16 months, and 4 await operation. In 6 of the 9 surgical cases (67%), there was a discrepancy between preoperative and pathological diagnosis. There were 4 hybrid congenital cystic adenomatoid malformation (CCAM)/sequestrations. Conclusions: At least 6 of 7 congenital cystic lung lesions decreased in si ze regardless of gestational age or presence of mediastinal shift. Antenata l intervention is therefore rarely indicated. Hybrid morphology may necessi tate resection of stable, asymptomatic lesions to prevent the theoretical c oncern for associated malignancies as well as other complications of CCAM. Copyright (C) 2000 by W.B. Saunders Company.