J.Jpn. Surg. Soc.. 97(8): 589-593, 1996

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Feature topic

PANCREATICO-BILIARY MALJUNCTION AND CONGENITAL DILATATION OF BILE DUCT

Congenital dilatation of bile duct (CDBD) or choledochal cyst has been demonstrated to be associated with pancreatico-biliary maljunction (PBM), and its various clinical signs and symptoms have been shown to be closely related with the presence of PBM.
In almost all of the patients who were less than one year of age, the disease was of the cystic type and patients presented with either a palpable mass or jaunce as the main symptom. In patients with more than one year of age, the disease was of either the cystic or cylindrical type. A history of episodes of abdominal pain accompanied by elevated levels of serum amylase was present in most patients with both types of diseases.
Histologic sections from the patients showed glandular formation with chronic inflammation, a result of refluxed activated pancreatic juice (possibly phospholipase A₂) : in contrast, histologic sections from the remaining patients of all ages showed only thickening of the fibrous layer. Thus, such variable morphologic features and clinical signs and symptoms in CDBD are highly dependent on two factors-the age at oset and the reflux of pancreatic juice into the bile duct through the common channel.

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