Chapter 341.5 - Other Conditions in Pancreatitis

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Harrison's Principles of Internal Medicine, 20e
Chapter 341.5: Other Conditions in Pancreatitis
Darwin L. Conwell; Peter A. Banks; Norton J. Greenberger
ANNULAR PANCREAS
When the ventral pancreatic anlage fails to migrate correctly to make contact with the dorsal anlage, the result may be a ring of pancreatic tissue
encircling the duodenum. Such an annular pancreas may cause intestinal obstruction in the neonate or the adult. Symptoms of postprandial fullness,
epigastric pain, nausea, and vomiting may be present for years before the diagnosis is entertained. The radiographic findings are symmetric dilation of
the proximal duodenum with bulging of the recesses on either side of the annular band, effacement but not destruction of the duodenal mucosa,
accentuation of the findings in the right anterior oblique position, and lack of change on repeated examinations. The differential diagnosis should
include duodenal webs, tumors of the pancreas or duodenum, postbulbar peptic ulcer, regional enteritis, and adhesions. Patients with annular
pancreas have an increased incidence of pancreatitis and peptic ulcer. Because of these and other potential complications, the treatment is surgical
even if the condition has been present for years. Retrocolic duodenojejeunostomy is the procedure of choice, although some surgeons advocate
Billroth II gastrectomy, gastroenterostomy, and vagotomy.
PANCREAS DIVISUM
Pancreas divisum is present in 7–10% of the population and occurs when the embryologic ventral and dorsal pancreatic anlagen fail to fuse, so that
pancreatic drainage is accomplished mainly through the accessory papilla. Pancreas divisum is the most common congenital anatomic variant of the
human pancreas. Current evidence indicates that this anomaly does not predispose to the development of pancreatitis in the great majority of patients
who harbor it. However, the combination of pancreas divisum and a small accessory orifice could result in dorsal duct obstruction. The challenge is to
identify this subset of patients with dorsal duct pathology. Cannulation of the dorsal duct by ERCP is not as easily done as is cannulation of the ventral
duct. Patients with pancreatitis and pancreas divisum demonstrated by MRCP or ERCP should be treated with conservative measures. In many of these
patients, pancreatitis is idiopathic and unrelated to the pancreas divisum. Endoscopic or surgical intervention is indicated only if pancreatitis recurs
and no other cause can be found. If marked dilation of the dorsal duct can be demonstrated, surgical ductal decompression should be performed. It
should be stressed that the ERCP/MRCP appearance of pancreas divisum (i.e., a smallcaliber ventral duct with an arborizing pattern) may be mistaken
as representing an obstructed main pancreatic duct secondary to a mass lesion.
MACROAMYLASEMIA
In macroamylasemia, amylase circulates in the blood in a polymer form too large to be easily excreted by the kidney. Patients with this condition
demonstrate an elevated serum amylase value and a low urinary amylase value. The presence of macroamylase can be documented by
chromatography of the serum. The prevalence of macroamylasemia is 1.5% of the nonalcoholic general adult hospital population. Usually
macroamylasemia is an incidental finding and is not related to disease of the pancreas or other organs. Macrolipasemia has now been documented in
a few patients with cirrhosis or nonHodgkin’s lymphoma. In these patients, the pancreas appeared normal on ultrasound and CT examination. Lipase
was shown to be complexed with immunoglobulin A. Thus, the possibility of both macroamylasemia and macrolipasemia should be considered in
patients with elevated blood levels of these enzymes.
A CKNOWLEDGMENT
This chapter represents a revised version of chapters by Drs. Norton J. Greenberger, Phillip P. Toskes, and Bechien Wu that were in previous editions
of Harrison’s.
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Harrison's Principles of Internal Medicine, 20e