Congenital Cystic Adenomatoid Malformation and Extralobar Pulmonary Sequestration in an Infant

Abstract

Although congenital cystic adenomatoid malformation(CCAM) and extralobar pulmonary sequestration(EPS) are different entities, they sometimes share common morphologic features, raising a possibility of a morphogenetic link. We report an autopsy case of a neonate who had features of both EPS and CCAM. An abnormal left lung with three separate lobes was found in the left thoracic cavity after removal of herniated abdominal viscera through the Bochdalek foramen. Two lobes without bronchial connections were directly connected with the descending aorta, and showed typical histologic features of CCAM. The lung tissue with normal bronchial connection and 'pulmonary artery supply showed identical features to these accessory lobes. Our case provides an evidence that CCAM and EPS can occur in a single individual and in the same side of the lung, and therefore raises a possibility that these two lesions could be different expressions of the same disease.