What is the pathogenesis of choledochal cysts?

Updated: Jul 01, 2020
  • Author: Emily Tommolino, MD; Chief Editor: BS Anand, MD  more...
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Answer

No strong unifying etiologic theory exists for choledochal cysts. The pathogenesis is probably multifactorial. [12] In many patients with choledochal cysts, an anomalous junction between the common bile duct and the pancreatic duct can be demonstrated. This occurs when the pancreatic duct empties into the common bile duct more than 1 cm proximal to the ampulla.

Some series, such as the one published by Miyano and Yamataka in 1997, have documented such anomalous junctions in 90-100% of patients with choledochal cysts. [13] This abnormal union allows pancreatic secretions to reflux into the common bile duct, where the pancreatic proenzymes become activated, damaging and weakening the bile duct wall. Defects in epithelialization and recanalization of the developing bile ducts and congenital weakness of the ductal wall also have been implicated. The result is the formation of a choledochal cyst.

These anomalies are classified according to the system published by Todani and coworkers. Five major classes of choledochal cysts exist (ie, types I-V), with subclassifications for types I and IV (ie, types IA, IB, IC; types IVA, IVB). [10, 14]


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