Results of surgical treatment of congenital cystic lung disease.

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.