Radiology of the Pancreas

Clinical Sx:
Recurrent abdominal pain since childhood or infancy, possibly precipitated by meals, lasting two to seven days. Attacks can occur every few weeks to every few years. Pain starts epigastric, but later involves the entire abdomen and radiates to the back. Severity and frequency of attacks improves with age.

Etiology/Pathophysiology: First described in 1952, autosomal dominant gene with incomplete penetrance. Males and females equally affected with all reported cases in caucasians. Etiology is unknown, but many patients have congenital malformations of the ductal system or sphincter of Oddi. It has been linked with hyperthyroidism, hyperlipoproteinemia, and aminoaciduria. Grossly, the pancreas resembles that seen in cystic fibrosis: induration, ductal dilation, fibrosis, preservation of islet cells.

Pathology:
As above.

Miscellaneous:
Diagnosis requires history of recurrent abdominal pain in at least three relatives. Course of the disease can be relatively benign if well treated, although there is reported 20-40% risk of pancreatic carcinoma in these patients. Increased risk of portal and splenic vein thrombosis.

Imaging:
similar to that or nonhereditary pancreatitis, with the exception that 50% eventually develop pseudocysts and over 50% get pancreatic calcifications and duct calculi, some as early as the second decade. pancreatic calcifications are larger and more rounded than in other conditions such as CF.

acute pancreatitis findings on US include hypoechogenicity, ductal dilatation, fluid collections, hemorrhage, ascites, and enlarged and ill-defined pancreas. CT findings include the above as well as lack of enhancement when necrosis is present. ERCP shows irregular ductal dilatation and beading.

chronic pancreatitis may show calcifications, atrophy, fibrosis, ductal dilatation, and biliary stricture, as well as any sequellae from pseudocysts.

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