Diagnostic imaging of digestive tract involvement in cystic fibrosis. Part 2: pancreatic and gastrointestinal disease

Cystic fibrosis (CF) is the most common fatal, autosomal recessive disease among the white population. Although recurrent pulmonary infections and pulmonary insufficiency are the major causes of morbidity and mortality, gastrointestinal symptoms generally present earlier and may suggest the diagnosis in the newborn or even prior to birth. The changes are attributed to the secretion of an abnormally thick mucous into the intestinal lumen, leading to the hallmark of diseases of the digestive tract: obstruction. This can be detected at birth in the form of mecanium ileus, ileal atresia, mecanium peritonitis and mecomiun plug, or present later on in childhood and adolescence as distal bowel obstruction syndrome or fibrosing colonopathy. This thick mucous can also trigger intussusception or acute appendicitis. Pancreatic insufficiency or pancreatic enzyme replacement therapy is the direct cause of most of these disorders. Plain radiography is of the utmost utility in assessing the digestive tract in CF. When the disease is detected in a newborn, the recommended approach is to perform plain abdominal X-ray, followed by barium enema, always accompanied by ultrasound. In older children and adolescents, enema and ultrasound are usually sufficient, although computed tomography and magnetic resonance may sometimes be necessary. (Author) 52 refs