RECURRENT PYOGENIC CHOLANGITIS

Resident(s): Evan Raff, MD MHA

Attending(s): Narasimham Dasika, MD
Program/Dept(s): University of Michigan Health System, Department of Radiology

CHIEF COMPLAINT & HPI

Chief Complaint and/or reason for consultation
Itching, jaundice, fever, and abdominal pain for 1 week

History of Present Illness

44-year-old Chinese woman with history of recurrent episodes of cholangitis who
presents with one week history of increased systemic itching and yellowing in her eyes.
She reports sharp midepigastric pain that lasted for about 30 minutes starting 1 day ago
with subjective fevers, chills and sweats.
She also reports dark urine, light colored stools and noticed her skin was yellow. She
also has intermittent nausea without vomiting.
Patient reports several year history of intermittent fevers and chills without abdominal
pain, nausea or vomiting which began during pregnancy.
Work up included several ERCPs with findings interpreted as primary sclerosing
cholangitis.

RELEVANT HISTORY
Past Medical History
Multiple episodes of cholangitis. Reported history of parasitic infection in infancy.

Past Surgical History

None

Family & Social History

Born in China and moved to USA in the late 1970s. No tobacco or drug use, rare alcohol.

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

SORRY, THATS INCORRECT!

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

Abdominal US: Several shadowing echogenic

foci (arrow) are present in the central biliary
system compatible with intrahepatic biliary
stone with diffuse biliary intrahepatic
dilatation.

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

E: All of the above

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.

CONTINUE WITH CASE

SORRY, THATS INCORRECT!

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.

CONTINUE WITH CASE

QUESTION SLIDE
3) Benefit of MRCP over ERCP in the evaluation of RPC includes:
1. Decreased risk of biliary sepsis

2. Improved spatial resolution

3. Allows for stone removal and cytological analysis
4. Ability to visualize ducts distal to central obstruction

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

CONTINUE WITH CASE

SORRY, THATS INCORRECT!

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

CONTINUE WITH CASE

INTERVENTION
Bilateral PTC tube placement for recurrent cholangitis with extensive intrahepatic
stone burden.
Biliary culture: Positive for Klebsiella, Enterococci and Pseduomonas.

Dilatation of the bilateral PTC tract with placement of 20 Fr choledochoscope

sheaths bilaterally.
Choledochoscopy and biliary stone removal of extensive stone burden in the right
and left intrahepatic ducts and exchange of PTC tubes.

INITIAL PTC PLACEMENT

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

Course has been complicated by recurrent episodes of cholangitis with bile

cultures positive for Klebsiella, Enterococci and Pseduomonas. Patient is
maintained on outpatient oral antibiotics (augmentin, PCN, & Cipro).
Given recurrent nature of disease, the patient was referred for surgical
consultation for choledochojejunostomy

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

CONTINUE WITH CASE

SORRY, THATS INCORRECT!

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

CONTINUE WITH CASE

SUMMARY & TEACHING POINTS

Pathogenesis:
Found almost exclusively in East and Southeast Asia where infection by parasitic
helminths (Ascaris) or liver flukes (Clonorchis, Opisthorchis, and Metorchis) is common.
Parasites induce biliary epithelial damage/fibrosis leading to stricturing and secondary
infection by enteric bacteria (commonly E. coli, Klebsiella, Pseudomonas, and Proteus)
Bacteria-produced gluconidases lead to pigment stone formation; low protein intake or
abnormal phospholipid metabolism may reduce natural inhibition of glucoronidases.

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

SUMMARY & TEACHING POINTS

Diagnosis:
Combination of clinical, laboratory and imaging characteristics
History of LFTs, stool O&P, serum ELISA, biliary cytology
Initial evaluation by ultrasound, followed by ERCP/MRCP

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

REFERENCES & FURTHER READING

Afagh, A, et al: Radiologic findings in recurrent pyogenic cholangitis. The Journal of Emergency Medicine, Vol. 26, No. 3, pp. 343346, 2004
Al-Sukhni, W, et al: Recurrent Pyogenic Cholangitis with HepatolithiasisThe Role of Surgical Therapy in North America. J Gastrointest Surg 12:496503, 2008
Cheung, MT, et al: Liver Resection for Intrahepatic Stones. Arch Surg.140:993-997, 2005
Harris, HW, et al: Recurrent Pyogenic Cholangitis. American Journal of Surgery. 176:35-37, 1998
Heffernan EJ et al: Recurrent pyogenic cholangitis: from imaging to intervention. AJR Am J Roentgenol. 192(1):W28-35, 2009

Jain M et al: MRCP findings in recurrent pyogenic cholangitis. Eur J Radiol. 66(1):79-83, 2008
Jeyarajah, DR: Recurrent Pyogenic Cholangitis Current Treatment Options in Gastroenterology. 7:9198, 2004
Kim JH et al: CT findings of cholangiocarcinoma associated with recurrent pyogenic cholangitis. AJR Am J Roentgenol. 187(6):1571-7, 2006
Lee, KF et al: Outcome of surgical treatment for recurrent pyogenic cholangitis: a single-centre study. HPB 11, 7580, 2009
Lee WJ et al: Radiologic spectrum of cholangiocarcinoma: emphasis on unusual manifestations and differential diagnoses. Radiographics. 21 Spec No:S97-S116, 2001
Lo CM et al: The changing epidemiology of recurrent pyogenic cholangitis. Hong Kong Med J. 3(3):302-304, 1997
Mori, T et al: Management of intrahepatic stones. Best Practice & Research Clinical Gastroenterology 20:6, 1117e1137, 2006
Nguyen, T et al: Recurrent Pyogenic Cholangitis. Dig Dis Sci (2010) 55:810
Park MS et al: Recurrent pyogenic cholangitis: comparison between MR cholangiography and direct cholangiography. Radiology. 220(3):677-82, 2001

Shoda, J et al: Molecular Pathogenesis of Hepatolithiasis A Type of Low Phospholipid-Associated Cholelithiasis. Frontiers in Bioscience 11, 669-675, 2006
Sperling RM et al: Recurrent pyogenic cholangitis in Asian immigrants to the United States: natural history and role of therapeutic ERCP. Dig Dis Sci. 42(4):865-71, 1997
Tsui WM et al: Hepatolithiasis and the syndrome of recurrent pyogenic cholangitis: clinical, radiologic, and pathologic features. Semin Liver Dis. 31(1):33-48, 2011