Presumptive Intrahepatic Congenital Cystic Adenomatoid Malformation

Abstract

ongenital cystic adenomatoid malformation (CCAM) and extralobar pulmonary sequestration (EPS) are 2 rare types of bronchopulmonary malformations that typically appear in infancy and childhood. A CCAM is a hamartomatous lesion of the lung, thought to be the result of the cessation of bronchiolar maturation with overgrowth of mesenchymal elements. An EPS is a discrete mass of lung tissues separate from the tracheobronchial tree and is supplied by a systemic vascular source (usually aortic). As well, it forms outside the normal pleural investment. Conran and Stocker 1 analyzed 50 cases of EPS and found that 50% (23 of 46) were associated with a coexistent CCAM. Rare cases of intra-abdominal EPS (IEPS) have been reported in the literature, and in these cases, CCAMs commonly occur within the pulmonary sequesterant. [2][3][4][5][6][7][8][9][10][11] We present an interesting case of CCAM manifesting within the liver of a patient treated prenatally for a large pulmonary type II CCAM and review the reported cases of IEPS with CCAM.