Is radical lobectomy required in congenital cystic adenomatoid malformation?

The extent of surgical resection in the management of congenital cystic adenomatoid malformation (CCAM) located in 1 pulmonary lobe remains controversial. Although lobectomy seems to be the best treatment to avoid recurrent pulmonary infection and neoplasia, neither retrospective nor prospective studies have been done to our knowledge. Besides, many reports claim that there are advantages of parenchyma-saving resections. The aim of our study was to determine whether it was necessary to perform an extended resection in these cases.

We retrospectively reviewed all patients who had undergone a thoracoscopic lobectomy for CCAM located to 1 pulmonary lobe between January 2007 and January 2009. A thoracic radiologist performed a single blind review of all preoperative computed tomographic (CT) scans, mentioning the presence or absence of distant lesions from the main cysts of CCAM within the pulmonary lobe. The pathologist who analyzed the pulmonary lobectomy specimen was aware of the diagnosis but not the CT report.

Twenty-five patients underwent thoracoscopic lobectomy for congenital lung malformations, including 12 with CCAM. All had a positive prenatal sonogram. The median age at surgery was 12 months (range, 2-24 months). The preoperative CT showed only 2 cases with distant lesions within the affected pulmonary lobe, whereas the histologic study of the surgical specimen identified 6 cases. The sensitivity of the CT scan was low, 33%, whereas its specificity was high, 100%. Furthermore, the preoperative CT negative predictive value was 60%. The median time of follow-up was 7.5 months.

Adequate treatment of CCAM in children requires a lobectomy because of poor sensitivity and very poor negative predictive value (60%) of the preoperative CT to determine distal adjacent lesions. A prospective study on a larger number of patients is required to confirm these observations.