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A patient with a bag of pancreatic stones

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Q1: What is the diagnosis?

The diagnosis is fibrocalculus pancreatopathy; previously it was known as fibrocalcific pancreatic diabetes. This condition was first reported from Indonesia by Zuidema. This condition has been reported from many tropical countries such as India and the south west Indian state of Kerala has the highest prevalence. The clinical features of fibrocalculus pancreatopathy have been described in detail from a series of over 1700 cases by Geevarghese. The proposed guidelines for diagnosis of fibrocalculus pancreatopathy, laid down by Mohan et al are shown in box 1.1

Box 1: Proposed guidelines for diagnosis of fibrocalculus pancreatopathy

  • Occurrence in a tropical country.

  • Diabetes diagnosed by World Health Organisation criteria.

  • Evidence of chronic pancreatitis.

  • Pancreatic calculi or at least three of the following: chronic abdominal pain since childhood, steatorrhoea, abnormal pancreatic morphology on imaging, abnormal pancreatic exocrine function tests.

  • Absence of other causes of chronic pancreatitis: alcohol abuse, hepatobiliary disease, primary hyperparathyroidism.

Q2: What are the radiological features and radiological differential diagnosis?

The calcium rich calculi are multiple, large, rounded, and discrete radio-opaque shadows. These are always confined to larger ducts, mostly in the head and body of pancreas. These are usually seen to the right of first and second lumbar vertebra on plain radiography of the abdomen. Ultrasonography or contrast enhanced computed tomography helps to localise calculi within the pancreatic ductal system and to document other features of chronic pancreatitis, such as ductal dilatation. Ultrasonography findings include shrinkage of gland, increased echogenicity, and ductal dilatation.2 Contrast enhanced computed tomography in advanced disease shows variable atrophy, with irregular ducts containing stones and occasionally heavy fat infiltration of gland. In extreme cases, little parenchyma is visible and the gland is reduced to a bag of stones.3 Endoscopic retrograde cholangiopancreatography is useful to identify non-calcific fibrocalculus pancreatopathy where marked ductal abnormality occurs in the absence of stones or to delineate the gland before surgery. The most important differential diagnosis is chronic alcoholic pancreatitis. Calculi found in this condition are small and in the smaller ductules; they show as poorly defined specks of calcification with hazy margins, lying on both sides of vertebral column.4

Learning points

Fibrocalculus pancreatopathy:

  • A rare cause of secondary diabetes.

  • Prevalent in tropical countries.

  • Comprises a clinical triad of abdominal pain, steatorrhoea, and diabetes.

  • Has a radiological hallmark of calculi in the main pancreatic duct, ductal dilatation, and atrophic pancrease.

  • Ketosis or ketoacidosis is rare.

Q3: Why ae these patients ketosis resistant?

Ketosis resistance may be due to several reasons. (1) Mohan et al indicated that C peptide levels are not as low as in type I diabetes; insulin deficiency may not be profound enough to allow ketosis. (2) In malnourished patients, the adipose tissue mass may be so small that free fatty acids cannot be mobilised fast enough by lipolysis to fuel ketogenesis.3,5

Final diagnosis

Fibrocalculus pancreatopathy.

References

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