Cystic Fibrosis

Clinical

Cystic fibrosis represents an abnormality in exocrine gland secretion.  It presents in the GI tract with varying degrees of pancreatic insufficiency, viscous intestinal mucus secretions, and meconium ileus. Pulmonary manifestations and infertility are also common manifestations.

Radiological

Imaging findings are varied, depending on the severity of disease.  Neonates may present with distal small bowel obstruction from meconium ileus.  In less severe forms, fluoroscopy may demonstrate impacted secretions and bowel wall edema (fold thickening), particularly in the duodenum and terminal ileum. Impacted secretions show up as poorly defined filling defects within the bowel lumen on fluoroscopic studies. Fold thickening within the terminal ileum is described as “jejunalization”, in which the usually featureless mucosal pattern becomes more prominent, resembling the normal mucosal pattern of the jejunum.

Cystic fibrosis.  Note the diffuse, irregular fold thickening.