CYSTIC-FIBROSIS - SPECTRUM OF THORACIC AND ABDOMINAL CT FINDINGS IN THE ADULT PATIENT

Cystic fibrosis (CF) is an autosomal recessive disorder that is charac terized by an abnormality of exocrine gland function. Adult patients r epresent a rapidly growing percentage of the CF population. Pulmonary changes are seen in nearly every case and are the most serious complic ation of CF. In advanced lung disease, bronchiectasis, emphysematous b ullae, and subpleural blebs can frequently develop. Although pulmonary disease is the most common cause of death and morbidity among CF pati ents, there also can be involvement of other organs, particularly in a dults, which show characteristic signs on CT and spiral CT. Pancreatic abnormalities are present in 85-90% of CF patients. The degree of pan creatic involvement varies, ranging from accumulations of mucus in the small ducts to totally plugged ducts, which can cause atrophy of the exocrine glands and progressive fibrosis. Pancreatic dysfunction on CT is demonstrated as fatty replacement and fibrosis of the pancreas. Ho wever, there may be scattered foci of pancreatic calcifications that c an be detectable on plain radiographs. Hepatobiliary involvement follo ws the same pattern as pancreatic abnormalities. Bile canaliculi are p lugged by mucinous material and when this plugging is of long duration , biliary cirrhosis with diffuse hepatic nodularity may develop. Such severe hepatic involvement is seen in only about 2-5% of patients, alt hough minor hepatic alterations, such as diffuse fatty changes, are fa irly common. Hepatobiliary involvement is characterized by hepatic nod ularity, compatible with cirrhosis, splenomegaly and ascites. Complete obstruction of the ileum by meconium occurs in approximately 10% of n ewborns with CF. Intestinal findings on CT include obstruction, althou gh this is more common in children. These CT signs should be evaluated carefully in adult patients since they may be suggestive of CF. Compu ted tomography offers unique information about or gan involvement (oth er than pulmonary) that can alter diagnosis and patient management. (C ) Elsevier Science Inc., 1998.