• 姓名：楊x錦

• Gender:female

• Age:52

• Admission date:99/06/18

• Chief complaint:sudden onset RUQ pain

since last night

• Present illness:This 52 y/o had HTN under

regular medical control for 1 month.According

to the patient and previous medical record,

she suffered from sudden onset RUQ pain

accompany with fever and chills one month

ago.She also had nausea and vominting.She

went 松山 hospital for help.Gastritis and

acute cholecystitis were diagnosed, and then

she was transferred to our hospital for

surgical intervention.

• PE showed murphys sign positive,right CP

angle knocking pain and RUQ tenderness.CT

showed gallstones with in multiseptated

enlogated gallbladder. Under the impression

of acute cholecystitis with gallstone , she is

admitted for furher evaluation and

management.

Physical examination

• RUQ tenderness(+)

• Murphy’s sign (+)

• Right CP angle knocking pain (+)

Lab data (0619)

WBC 13340/uL

HGB 13.2g/dL

PLT 203000/uL

glucose 113 mg/dl

creatinine 0.6 mg/dl

GOT 140 IU/L (0-40)

amylase 25 IU/L

Bilirubin T 0.9 mg/dl

Na 139 mEq/L

K 3.7 mEq/L

Image findingsMRCP and liver MRI

without and with Gd-DTPA

IV contrast study show:

A large cystic dilatation of

proximal CBD and CHD

with multiple

stones,connection with

mild cystic dilated left

main IHD and normal sized

distal CBD , consistent with

choledochal cyst

Differential diagnosis

• Biliary obstruction

– Bile duct stricture

– Cholangitis

– Choladochal cyst

– Cholesystitis and cholelithiasis

• Cholangiocarcinoma

• Acute pancreatitis

• Acute pancreatitis

– Sudden onset epigastric abdominal pain radiates

to back and flanks

– Nausea and vomiting , Fever

– Elevated serum amylase and lipase,

hyperglycemia

– An isalated left pleural effusion on chest

radiography is strongly suggestive of acute

pancreatitis

– Sentinel loop sign , colon cutoff sign

Sentinel loop sign

Colon cutoff sign

– Abdominal ultrasonography

– Abdominal CT scans

• grading scale developed by Balthazar

• A – Normal

• B - Enlargement

• C - Peripancreatic inflammation

• D - Single fluid collection

• E - Multiple fluid collections

CT scan in a patient with

abdominal pain, fever, and

jaundice shows air (thin

arrow) in the central

pancreas, which is necrotic

and largely replaced by an

acute fluid collection (thick

arrows), leaving only a

small residual pancreatic

head (P).

uptodate

Dynamic computed

tomographic image shows

a thin-walled pancreatic

pseudocyst in a patient

with chronic pancreatitis.

The cyst is compressing

the gastric antrum and an

abnormal pancreas with

ductal dilatation and

calcifications can be seen

(red arrows).

uptodate

• Cholangiocarcinoma

• intrahepatic, extrahepatic (ie, perihilar), and distal

extrahepatic

• Perihilar tumors(Klatskin tumors) are the most

common

• 95% ductal adenocarcinomas

• Related to Primary sclerosing cholangitis

• Hepatomegaly is frequent, dilatation of biliary tree can

be detected

• Jaundice, pururitus, weight loss, RUQ abdominl pain

• CA19-9, Cholangiocarcinoma does not produce alpha-

fetoprotein

• Image studies

– Ultrasound may demonstrate biliary duct

dilatation and larger hilar lesions

• Patients with underlying PSC may have limited ductal

dilatation secondary to ductal fibrosis

• vascular encasement or thrombosis

– Helical CT scans are accurate in diagnosing the

level of biliary obstruction.

– MR cholangiography enables imaging of bile ducts

and, in combination with MR angiography,

permits staging (excluding vascular involvement)

This MRCP image,

demonstrates a

circumferential narrowing

of the distal common bile

duct (CBD, arrow) due to a

focal cholangiocarcinoma.

The obstructing tumor is

causing dilation of the

CBD.

Courtesy of Jonathan

Kruskal, MD, PhD.

• Cholangitis

• choledocholithiasis was the most common cause of

biliary tract obstruction resulting in cholangitis

• fever, abdominal (right upper quadrant) pain, and

jaundice (the Charcot triad)

• Organisms typically are enteric in origin

• Abdominal Echo

• Primary sclerosing cholangitis

• autoimmune mechanism

• approximately 75-90% of patients with PSC have

inflammatory bowel disease (IBD)

• (ANCA) in 87%, anticardiolipin (aCL) antibodies in 66%,

and antinuclear antibodies (ANA) in 53%

• progressive disease that eventually culminates in portal

hypertension and cirrhosis

• 70% of patients with PSC are men with a mean age of

diagnosis around 40 years

• fatigue, jaundice, pruritus, and right upper quadrant

pain

• Recurrent febrile episodes of bacterial cholangitis occur

in 10-15% of patients and may have an acute hepatitis-

like presentation

• Elevated alkaline phosphatase

– Imaging Studies

• ERCP is considered the criterion standard for

confirming a diagnosis of PSC. Beading-like appearance

of intra and extra hepatic duct

MRCP study shows small

stones (arrows) in the

gallbladder (GB) and the

common bile duct (CBD).

Beading-like

appearance of intra

and extra hepatic duct

Surgical intervention(0619)

– 1.laparoscopic approach to gallbladder and CBD

– 2.Cystic duct connected to dilated CBD , fuciform

type

– 3.resection of gallbladder and total exis of dilated

CBD about 1.5cm

– 4.Roux-en-Y choledocho/hepaticoportal

jejunostomy

Pathological findings (0622)

臨床診斷臨床診斷臨床診斷臨床診斷 choledochal cyst

檢查診斷 common bile duct , excision ,

choledochal cyst Gallbladder,

cholecystectomy , chronic

cholecystitis and cholelithiasis

Disscusion

Choledochal cyst

• Localized cystic dilatation of all or part of the

common bile duct

• 80% present in childhood

• Most common in Japan (1 in 1000 live births)

• Relatively rare in Western Europe (1 in

100,000 live births)

• Male : female ratio is 1:4

• PATHOLOGY

– Children:densely fibrotic cyst wall with evidence

of chronic and acute inflammation

– Adults:frequently inflammatory changes, erosions,

sparse distribution of mucin glands

– Malignancy: most commonly found in the

posterior cyst wall

• Abnormal pancreaticobiliary junction(ABPJ)

– APBJ is present in about 70 percent of patients

with biliary cysts and may be a significant risk

factor for the development of malignancy in the

biliary cyst

– A long common channel may predispose to reflux

of pancreatic juice into the biliary tree, since the

ductal junction lies outside of the duodenal wall

and the sphincter of Oddi

Todani CLASSIFICATION

• Type I is a cystic dilatation of the common bile

duct (CBD) and is the most common,

comprising 50 to 85 percent of all biliary cysts .

– Type IA common type

– Type IB segmental type

– Type IC diffuse dilatation

• Type II, the rarest biliary cyst, is a true

diverticulum of the extrahepatic bile duct

located proximal to the duodenum

• Type III is a cystic dilatation limited to the

intraduodenal portion of the distal common

bile duct, also known as a choledochocele

• Type IV includes cases of multiple cysts

– Type IVA(the second most common type of biliary

cyst), both intrahepatic and extrahepatic cystic

dilations

– Type IVB, in which there are multiple extrahepatic

cysts without intrahepatic involvement.

• Type V includes isolated or multiple cystic

dilatations of the intrahepatic ducts without

extrahepatic duct disease, giving an

appearance similar to Caroli's disease

CLINICAL MANIFESTATIONS

• Triad: pain, jaundice, and abdominal mass

– Infants : conjugated hyperbilirubinemia (80

percent), failure to thrive, or an abdominal mass

(30 to 60 percent).

– in patients older than two

• chronic and intermittent abdominal pain (50 ~ 96

percent)

• Intermittent jaundice and recurrent cholangitis are also

common (34 ~ 55 percent)

• abdominal mass is less common (10 ~ 20 percent)

• Pancreatitis(20%)

• biliary lithiasis(8%)

DIAGNOSIS

• ultrasound or CT

• Direct cholangiography has long been

considered the best test for diagnosis and

evaluation.

• MRCP is useful for diagnosis. It accurately

demonstrates cystically dilated segments of

the biliary tree, and identifies APBJ in over 75

percent of cases

MANAGEMENT

• Current standard of treatment for Types I, II,

and IV biliary cysts is surgical excision

– decreasing the risk of malignant degeneration,

reduce complications such as recurrent

cholangitis, choledocholithiasis, and pancreatitis

• In the case of extrahepatic cysts, resection is

usually followed by hepaticojejunostomy for

reconstruction

– The most frequent long-term complication(25%)

of hepaticojejunostomy is stenosis of the biliary-

enteric anastomosis leading to cholangitis,

jaundice, or cirrhosis

• Type III cysts (choledochoceles) are often

amenable to endoscopic sphincterotomy.

reference

• Uptodate

• Md consult

• emedicine