Pediatric Cystic Fibrosis Pancreas

  • Etiology: Pancreatic insufficiency leads to fatty atrophy
  • Imaging:
    — Variable appearance of pancreas from totally fatty replaced to small and fibrotic
    — Can see macrocysts and calcifications
  • DDX:
  • Complications:
  • Treatment:
  • Clinical:
    — Most common hereditary cause of pancreatitis in children
    — Most common cause of pancreatic insufficiency in children

Radiology Cases of Cystic Fibrosis Pancreas

Radiology Cases of Pancreatic Insufficiency and Cirrhosis of the Liver and Esophageal Varices Due to Cystic Fibrosis

CT of cystic fibrosis causing liver cirrhosis and pancreatic insufficiency
Coronal CT with contrast of the abdomen (above) shows a marked nodular contour to the liver and multiple dilated veins around the spleen. Coronal CT with contrast of the abdomen (below) shows diffuse fatty replacement of the pancreas.

Radiology Cases of Pancreatic Insufficiency and Liver Cirrhosis Due to Cystic Fibrosis and Normal Appendix

CT of a dilated but normal appendix in a patient with cystic fibrosis
Axial CT with contrast of the abdomen (above left) shows an irregular contour to the liver and fatty replacement in the pancreatic head. Axial (below left) and coronal (right) CT show the appendix is dilated to a diameter of 12 mm but there are no inflammatory changes around it.

Radiology Cases of Chronic Pancreatitis Due to Cystic Fibrosis

AXR of cystic fibrosis with pancreatic insufficiency
AXR shows calcifications throughout the pancreas.

Radiology Cases of Acute Pancreatitis with Pseudocyst and Chronic Pancreatitis Due to Cystic Fibrosis and Fatty Infiltration of Liver and Normal Appendix and Distal Intestinal Obstruction Syndrome

CT of enlarged but normal appendix in cystic fibrosis
Axial CT with contrast of the abdomen (above left) shows a low density liver due to fatty infiltration and a low density pancreas with calcifications that has a round low density fluid collection near the pancreatic head. The appendix in the right lower quadrant posterior to the cecum is dilated in diameter up to 10 mm but has no periappendiceal inflammation (above right and below). There is stool mixed with air in the terminal ileum medial to the cecum (above right and below).