Internal Medicine

0521779407-C01 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 20:53

302 Cholangiocarcinoma Choledochal Cysts

➣proximal tumors: hardest to resect as the adjacent structures,

i.e., portal vein, liver are involved; procedure=resection and

Roux-en-Y hepaticojejunostomy

follow-up

■serial visits with physical examination, liver chemistries, CA 19–9

complications and prognosis

■median survival after surgery: 2 years

■median survival without surgery: 6–12 mo

■stenting of the biliary tree for palliation; metal stents patent for

8 months; operative biliary-enteric bypass can also be offered but

no difference in survival

■liver transplantation: 5-yr survival 17%; high recurrence; usually not

done

Choledochal Cysts...................................

AIJAZ AHMED, MD

history & physical

History

■biliary cysts: choledochal cysts, gallbladder cysts, and cystic duct

cysts

■choledochal cysts may involve any segment of the bile duct

■classification (Todani et al.)

➣type I A choledochal cyst

➣type I B segmented choledochal dilatation

➣type I C diffuse or cylindrical duct dilatation

➣type II extra hepatic duct diverticula

➣type III choledochocele

➣type IVA multiple intra- and extrahepatic duct cysts

➣type IVB multiple extrahepatic duct cysts

➣type V intrahepatic duct cysts (Caroli’s disease)

■type I cysts are the most common, accounting for 40% to 60% of all

cases, followed by type IV; type II, III and V are rare

■Type V divided into two subtypes:

➣simple type (Caroli’s disease) cyst, limited to the larger intrahep-

atic bile ducts without cirrhosis/portal hypertension

➣periportal fibrosis type (Caroli’s syndrome) with cirrhosis/portal

hypertension (more common than Caroli’s disease)