Congenital cystic adenomatoid malformation of the lung associated with abdominal musculature deficiency (prune belly)

'Prune belly' - a deficiency of abnormal musculature usually associated with urinary tract anomalies and cryptorchidism - has been explained by numerous theories. Congenital cystic adenomatoid malformation of the lung (CCAM) is a distinct morphological entity, characterized by a proliferation of cysts lined by bronchiolar like epithelium and a lack of cartilage and seromucous glands. CCAM has been postulated to be a hamartoma, a focal type of pulmonary dysplasia, or a development anomaly. The authors have observed a patient at autopsy with CCAM and prune belly, but no urinary tract abnormalities. The pathological findings suggest that the abdominal muscular deficiency may be secondary to severe abdominal distention from ascites produced by obstruction of portal venous return by the pressure of the intrathoracic mass.