Results of surgical treatment of congenital cystic lung disease

Abstract

Objective: To review the experience with surgical intervention for congenital cystic lung disease (CCLD).

Methods: From April 1988 to December 2001, 68 patients were diagnosed and treated surgically for CCLD. There were 27 males and 41 females ranging in age from 2 to 65 years. The majority of the cases were diagnosed by history, clinical manifestations and chest radiography or computerized tomography (CT). There were 25 (36.8 %) right-sided and 43 (63.2 %) left-sided cystic lung lesions. Four patients had massive bleeding and needed urgent or emergency operation. Pneumonectomy, lobectomy, and/or segmentectomy were performed.

Results: 29 (42.6 %) patients underwent lobectomy, 25 (36.8 %) had total pneumonectomy, 9 (13.2 %) lobectomy plus segmentectomy, and 5 (7.4 %) bilobectomy. During the operation, left-sided pulmonary sequestration was confirmed in 3 patients. No death occurred in this series. Diagnoses were confirmed by pathology in all cases. Two patients had malignant lesions arising from multicystic lung lesions.

Conclusions: A history of recurrent pneumonia, often with hemoptysis and subsequent dyspnea, should raise the suspicion of CCLD. The diagnosis can be confirmed by chest X-ray and CT preoperatively, and early surgical intervention may avoid the development of serious complications. Delayed diagnosis and surgical management usually result in significant morbidity and mortality.