Pediatric Cystic Fibrosis Liver

Etiology: Fatty infiltration leads to cirrhosis leads to portal hypertension
Imaging: Liver cirrhosis leads to esophageal varices
DDX:
Complications:
Treatment:
Clinical:

Radiology Cases of Cystic Fibrosis Liver

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

UGI of esophageal varices in cystic fibrosis — Lateral images from an UGI shows multiple rounded filling defects extrinsic to the distal esophageal lumen.

Radiology Cases of Fatty Infiltration Due to Cystic Fibrosis and Acute Pancreatitis with Pseudocyst and Chronic Pancreatitis 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).