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RELEVANT HISTORY
Past Medical History
Multiple episodes of cholangitis. Reported history of parasitic infection in infancy.
Review of Systems
Negative unless as stated above.
Medications: None
Allergies: NKDA
DIAGNOSTIC WORKUP
Physical Exam
T 98.4 BP 111/62 HR 96 RR 18 O2 sat 96% on RA
General: Well-appearing, lying in bed, NAD
Eyes: Mild scleral icterus
GI/ABD: Soft, nondistended, mild tenderness to palpation in the RUQ/epigastric region
w/o rebound/guarding, normoactive bowel sounds.
Ext: No LE edema, all 4 extremities w/w/p
DIAGNOSTIC WORKUP
Laboratory Data
WBC 17.7, AST 74, ALT 118, Alk phos 830, Tbil 3.0.
Non-Invasive Imaging
Ultrasound: Intrahepatic ductal dilation filled with echogenic material suspected to be
stones.
MRCP: Severe stricturing of the central intrahepatic ducts and large intrahepatic stone
burden. Transient periductal arterial hyperenhancement likely reflects cholangitis.
QUESTION SLIDE
1) Recommended first line imaging for patients with suspected recurrent pyogenic
cholangitis:
A: Contrast enhanced CT.
B: Ultrasound.
C: MRCP.
D: ERCP.
CORRECT!
1) Recommended first line imaging investigation for patients with suspected recurrent
pyogenic cholangitis:
A: Contrast enhanced CT. Provides better spatial resolution than ultrasound, but with
radiation. Similar ability to detect stones, pneumobilia and masses. Enhancement of biliary
mucosa can indicate active cholangitis.
B: Ultrasound. Quick and cost effective, ultrasound can demonstrate the general features
of RPC including intrahepatic calculi (identified in up to 90% of patients), pneumobilia,
ductal dilatation and related complications including hepatic masses (e.g., abscess,
cholangiocarcinoma). (Heffernan et al., AJR 2009)
C: MRCP. Expensive but with ability to characterize ducts proximal to an obstruction or tight
stenosis better than ERCP. No risk of aggravating biliary sepsis. Improved sequence speed
reduce motion artifacts.
D: ERCP. Allows for stone removal, cytologic but has risk for aggravation/development of
biliary sepsis. Previously the gold standard with high spatial resolution, MRCP is preferred
for given noninvasive nature.
CONTINUE WITH CASE
ABDOMINAL US
CT ABDOMEN PELVIS
CT Abdomen Pelvis: Marked central intrahepatic biliary dilatation. Several foci of high
attenuation are present compatible with stones (not seen on these images).
MRCP
MRCP images demonstrate multifocal biliary strictures and dilatation with intrahepatic filling
defects (arrow) compatible with stones. Volume rendered images (right) demonstrate diffuse
intrahepatic biliary dilatation.
ERCP
ERCP image shows diffuse intrahepatic duct dilatation with multiple stones (arrow) and
biliary sludge
DIAGNOSIS
Recurrent pyogenic cholangitis (RPC) causing secondary sclerosing
cholangitis
Differential Diagnosis
Primary sclerosing cholangitis
Peribiliary cysts
Hydatid disease
Peripheral cholangiocarcinoma
Carolis disease
AIDS cholangiopathy
QUESTION SLIDE
2) Complications of recurrent pyogenic cholangitis include
A: Cholangiocarcinoma
B: Biloma
C: Portal vein thrombosis
D: Cirrhosis
CORRECT!
2) Complications of recurrent pyogenic cholangitis include
A: Cholangiocarcinoma
B: Biloma
C: Portal vein thrombosis
D: Cirrhosis
E: All of the above. Patients with severe RPC are at risk for all of the above. These
complications should be monitored with serial imaging and cytology
examinations.
B: Biloma
C: Portal vein thrombosis
D: Cirrhosis
E: All of the above. Patients with severe RPC are at risk for all of the above. These
complications should be monitored with serial imaging and cytology
examinations.
QUESTION SLIDE
3) Benefit of MRCP over ERCP in the evaluation of RPC includes:
1. Decreased risk of biliary sepsis
A: 2 and 3
B: 1 and 3
C: 1 and 4
D: 2 and 4
CORRECT!
3) Benefits of MRCP over ERCP in the evaluation of RPC include:
A: 2 and 3
B: 1 and 3
C: 1 and 4. MRCP allows for improved visualization of ducts distal to obstructions
but has a lower spatial resolution than ERCP. ERCP may be used for stone removal,
analysis and cytology but results in increased risk for aggravation of bacteremia.
D: 2 and 4
B: 1 and 3
C: 1 and 4. MRCP allows for improved visualization of ducts distal to obstructions
but has a lower spatial resolution than ERCP. ERCP may be used for stone removal,
analysis and cytology but results in increased risk for aggravation of bacteremia.
D: 2 and 4
INTERVENTION
Bilateral PTC tube placement for recurrent cholangitis with extensive intrahepatic
stone burden.
Biliary culture: Positive for Klebsiella, Enterococci and Pseduomonas.
The biliary system was accessed under ultrasound guidance using a 22 gauge Chiba needle through which a wire
was passed. Fluoroscopic images demonstrate moderate to severe bilateral central and intrahepatic ductal
dilatation with associated central and intrahepatic biliary duct strictures. In addition, there are multiple filling
defects seen throughout the bilateral biliary ducts, consistent with sludge, debris, and stones.
CHOLEDOCHOSCOPY
(6 weeks post presentation)
Fluoroscopic images show placement of bilateral Amplatz superstiff guidewires through existing biliary
drainage tube tracts and dilatation of PTC tracts using two kissing 8 x 4 mm balloons. 20 Fr peel away sheaths
were placed through which a 16.5 Fr choledochoscope was advanced into the right and left hepatic ducts.
CHOLEDOCHOSCOPY
(6 weeks post presentation)
Extensive right and left intrahepatic biliary calculi were seen involving almost all the segmental ducts
including the common hepatic duct and CBD. Small casts and debris were removed by scope and Nitinol
Zero tip 4 wire basket. Large CBD stone was fragmented using electrohydraulic lithotripsy. Bilateral 14Fr
pigtail PTC tubes with additional sideholes were placed for additional external and internal drainage.
CLINICAL FOLLOW UP
Patient has returned for multiple PTC exchanges with balloon clearance of
CBD, right and left main hepatic ducts, and segmental/subsegmental ducts
Labs:
Stone analysis: calcium bilirubinate
Repeat common bile duct/hepatic duct brushing cytology negative for malignant cells
QUESTION SLIDE
4) Treatment option for localized lobar disease when atrophy has occurred
includes:
A: Segmental hepatic resection
B: Orthotopic liver transplant
C: Endoscopic intervention
D: Biliary bypass
CORRECT!
4) Treatment option which should be considered for localized RPC:
A: Segmental hepatic resection. May be considered when calculi are isolated to
the a single lobe generally after atrophy has occurred. This can reduce the risk for
hepatic abscess formation and cholangiocarcinoma.
B: Orthotopic liver transplant
C: Endoscopic intervention
D: Biliary bypass
D: Biliary bypass
Presentation
Fever, RUQ pain, leukocytosis, elevated alkaline phosphatase and bilirubin
Incidence in Asia decreasing due to improved nutritional standards, but prevalence in
the West increasing due to migration from endemic areas
Recurrent episodes of cholangitis lead to secondary biliary sclerosis and eventually
biliary cirrhosis and portal hypertension in later stages
Treatment:
Requires repeated multidisciplinary approach
Antibiotic therapy for recurrent episodes; equivocal evidence for ursodial therapy
Biliary drainage and stone removal via ERCP and PTC
Surgical hepatico-jejunostomy or lobectomy for advanced or isolated left lobe disease
Complications
Liver abscess formation (20%) and risk for septic emboli
Secondary biliary cirrhosis, portal vein thrombosis
Biloma
Cholangiocarcinoma (1.5-11%) and inflammatory pseudotumor
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