What are choledochal cysts?

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

Choledochal cysts are congenital bile duct anomalies. These cystic dilatations of the biliary tree can involve the extrahepatic biliary radicles, the intrahepatic biliary radicles, or both. They may occur as a single cyst or in multiples within the biliary tree. In 1723, Vater and Ezler published the anatomic description of a choledochal cyst. Douglas wrote the clinical report involving a 17-year-old girl presenting with jaundice, fever, intermittent abdominal pain, and an abdominal mass. [9] The patient died a month after an attempt was made at percutaneous drainage of the mass. (See image below.)

Operative specimen of a type I choledochal cyst. Operative specimen of a type I choledochal cyst.

In 1959, Alonzo-Lej produced a systematic analysis of choledochal cysts, reporting on 96 cases. He devised a classification system, dividing choledochal cysts into 3 categories, and outlined therapeutic strategies. Todani has since refined this classification system to include 5 categories. [10] This article reviews the incidence, pathophysiology, diagnosis, and management of choledochal cysts.

Based on findings from a retrospective analysis of 32 children and 47 adults with choledochal cysts, Shah et al concluded that because of differences with regard to the presentation, management, and histopathology of, as well as the outcomes related to, these lesions, choledochal cysts in children should be considered separate entities from those in adults. [11] The authors reported the following findings [11] :

  • A history of biliary surgery, pancreatitis, cholangitis, early postoperative complications, and late postoperative complications occurred, respectively, 5.1, 5.4, 6.4, 2.0, and 3.3 times more frequently in adults than they did in children.

  • The classic triad of abdominal pain, jaundice, and a palpable right upper quadrant abdominal mass occurred 6.7 times more frequently in children than in adults.

  • Fibrosis of the cyst wall was peculiar to children.

  • Signs of inflammation and hyperplasia were primarily seen in adults.

  • Long-term complications occurred in 29.7% of adults but in only 9.3% of children.

For patient education resources, see the Digestive Disorders Center, as well as GallstonesPancreatitisCirrhosis, and Abdominal Pain in Adults.


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