Internal Medicine

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

Choledochal Cysts 303

■incidence:1/15,000 in the Western countries and as high as 1/1000

in Japan

■not familial; female to male ratio 3:1

■two thirds of the patients present before the age of 10

Signs & Symptoms

■classic clinical presentation of choledochal cyst disease Type I, II,

and IV: triad of abdominal pain (>50%), mass (50%) and jaundice

(one third)

■adults with persistent and unexplained symptoms, including jaun-

dice, recurrent abdominal pain, fever, nausea, vomiting, cholangitis

and pancreatitis

■typical presentation often includes only one or two findings of the

triad

■type III: recurrent biliary pain or pancreatitis

■type V: recurrent cholangitis and liver abscesses, pain, and fever

tests

Laboratory

■elevated total bilirubin, liver enzymes, amylase and lipase

■tumor markers: CA19–9, alpha-fetoprotein

Imaging

■ultrasound: best way to establish diagnosis of choledochal cyst

■ERCP: confirm diagnosis, help classify cyst, and may be therapeutic

(type III)

■abnormal pancreatobiliary duct junction suggestive of choledochal

cysts

differential diagnosis

■cholangitis, Caroli’s disease, CBD obstruction secondary to stone or

stricture

management

What to Do First

■rule out biliary obstruction

■classify and confirm diagnosis (ultrasound and/or ERCP)

■rule out cholangiocarcinoma: ERCP, CT scan, tumor markers

■identification of pancreatic duct insertion by ERCP critical to plan

surgical management