Editing Choledochal cyst (section)

== '''Management''' ==

* Traditionally was cystenterostomy (internal drainage procedures) and cholecystectomy
** Persistent biliary stasis, cholangitis, pancreatitis, recurrent strictures, liver fibrosis, and persisting risk of cholangiocarcinoma
* Mostly now cholecystectomy and complete resection of cyst with biliary-enteric anastomosis
* '''Type I:''' cholecystectomy then Kocher's maneuvre. Dissect out anterior wall, then ligate CBD below. Then take off portal vein and mobilise to level of hepatic duct bifurcation and divide just distal to bifurcation. Need to do frozen section here. Reconstruction with roux-en-Y hepaticojejunostomy.
* '''Type II:''' simple diverticulectomy and closure of CBD at cyst neck
* '''Type III:''' mostly small which can be managed with endoscopic sphincterotomy. Larger cysts may need transduodenal excision.
* '''Type IV:''' Challenging. Complete excision is not always possible. Excise the external components and reconstruct similar to type I cysts. Partial hepatectomy is recommended for any intrahepatic component likely to result in future complications.
* '''Type V:''' Treat symptoms first. Surveillance for cholangiocarcinoma with serial imaging and serum CA19-9.
** Hepatic resection may be used for symptoms confined to one lobe of liver.
** Complicated bilobar disease and recurrent cholangitis despite maximal medical therapy, portal hypertension, or suspicion of early cholangiocarcinoma requires liver transplant.