Congenital cystic adenomatoid malformation of the lung associated with bile duct hypoplasia.

Congenital cystic adenomatoid malformation (CCAM) is a rare lesion that affects one or two lobes of the lung. Infants are either stillborn or die shortly after birth if therapy is not started immediately. The lesion itself may be solid or cystic, manifesting in an adenomatoid increase in terminal respiratory structures. Clinical, pathological and radiological findings of a child prematurely born are presented. A special feature in this case was hypoplasia of intrahepatic bile ducts, an unusual association with CCAM that has not been previously reported. Pathogenesis and embryological connotations of the two lesions are discussed. The importance of prenatal diagnosis and immediate surgical therapy after birth is stressed.