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RECURRENT PYOGENIC CHOLANGITIS
Resident(s): Evan Raff, MD MHA
Attending(s): Narasimham Dasika, MD
Program/Dept(s): University of Michigan Health System, Departmen
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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 wpresents with one week history of increased systemic itching and yellowing in
She reports sharp midepigastric pain that lasted for about 30 minutes starting
with subjective fevers, chills and sweats.She also reports dark urine, light colored stools and noticed her skin was yelloalso has intermittent nausea without vomiting.
Patient reports several year history of intermittent fevers and chills without apain, nausea or vomiting which began during pregnancy.
Work up included several ERCPs with findings interpreted as primary sclerosincholangitis.
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RELEVANT HISTORY
Past Medical History
Multiple episodes of cholangitis. Reported history of parasitic infection in infa
Past Surgical History
None
Family & Social History
Born in China and moved to USA in the late 1970s. No tobacco or drug use, ra
Review of Systems
Negative unless as stated above.
Medications: None
Allergies: NKDA
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DIAGNOSTIC WORKUP
Physical ExamT 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/epigastw/o rebound/guarding, normoactive bowel sounds.
Ext: No LE edema, all 4 extremities w/w/p
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DIAGNOSTIC WORKUP
Laboratory DataWBC 17.7, AST 74, ALT 118, Alk phos 830, Tbil 3.0.
Non-Invasive Imaging
Ultrasound: Intrahepatic ductal dilation filled with echogenic material suspectstones.
MRCP: Severe stricturing of the central intrahepatic ducts and large intrahepa
burden. Transient periductal arterial hyperenhancement likely reflects cholan
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QUESTION SLIDE
1) Recommended first line imaging for patients with suspected recurrentcholangitis:
A: Contrast enhanced CT.
B: Ultrasound.
C: MRCP.
D: ERCP.
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CORRECT!
1) Recommended first line imaging investigation for patients with suspected recurpyogenic cholangitis:
A: Contrast enhanced CT. Provides better spatial resolution than ultrasound, but wradiation. Similar ability to detect stones, pneumobilia and masses. Enhancement mucosa can indicate active cholangitis.
B: Ultrasound. Quick and cost effective, ultrasound can demonstrate the generaof RPC including intrahepatic calculi (identified in up to 90% of patients), pneumductal dilatation and related complications including hepatic masses (e.g., absce
cholangiocarcinoma). (Heffernan et al., AJR 2009)C: MRCP. Expensive but with ability to characterize ducts proximal to an obstructiostenosis better than ERCP. No risk of aggravating biliary sepsis. Improved sequencreduce motion artifacts.
D: ERCP. Allows for stone removal, cytologic but has risk for aggravation/developmbiliary sepsis. Previously the gold standard with high spatial resolution, MRCP is prfor given noninvasive nature.
CONTINUE WITH CASE
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SORRY, THATS INCORRECT!
1) Recommended first line imaging investigation for patients with suspected recurpyogenic cholangitis:
A: Contrast enhanced CT. Provides better spatial resolution than ultrasound, but wradiation. Similar ability to detect stones, pneumobilia and masses. Enhancement mucosa can indicate active cholangitis.
B: Ultrasound. Quick and cost effective, ultrasound can demonstrate the generaof RPC including intrahepatic calculi (identified in up to 90% of patients), pneumductal dilatation and related complications including hepatic masses (e.g., absce
cholangiocarcinoma). (Heffernan et al., AJR 2009)C: MRCP. Expensive but with ability to characterize ducts proximal to an obstructiostenosis better than ERCP. No risk of aggravating biliary sepsis. Improved sequencreduce motion artifacts.
D: ERCP. Allows for stone removal, cytologic but has risk for aggravation/developmbiliary sepsis. Previously the gold standard with high spatial resolution, MRCP is prfor given noninvasive nature.
CONTINUE WITH CASE
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ABDOMINAL US
Abdominal US: Several shadowfoci (arrow) are present in the csystem compatible with intrahe
stone with diffuse biliary intrahdilatation.
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CT ABDOMEN PELVIS
CT Abdomen Pelvis: Marked central intrahepatic biliary dilatation. Several foci of hattenuation are present compatible with stones (not seen on these images).
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MRCP
MRCP images demonstrate multifocal biliary strictures and dilatation with intrahepatic fdefects (arrow) compatible with stones. Volume rendered images (right) demonstrate diintrahepatic biliary dilatation.
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ERCP
ERCP image shows diffuse intrahepatic duct dilatation with multiple stones (arrow) andbiliary sludge
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DIAGNOSIS
Recurrent pyogenic cholangitis (RPC) causing secondary sclecholangitis
Differential Diagnosis
Primary sclerosing cholangitis
Peribiliary cystsHydatid disease
Peripheral cholangiocarcinoma
Carolisdisease
AIDS cholangiopathy
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QUESTION SLIDE
2) Complications of recurrent pyogenic cholangitis include
A: Cholangiocarcinoma
B: Biloma
C: Portal vein thrombosis
D: Cirrhosis
E: All of the above
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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 abovcomplications should be monitored with serial imaging and cytologyexaminations.
CONTINUE WITH CASE
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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 abovcomplications should be monitored with serial imaging and cytologyexaminations.
CONTINUE WITH CASE
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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
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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 obstbut has a lower spatial resolution than ERCP. ERCP may be used for stoneanalysis and cytology but results in increased risk for aggravation of bacte
D: 2 and 4
CONTINUE WITH CASE
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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 obstbut has a lower spatial resolution than ERCP. ERCP may be used for stoneanalysis and cytology but results in increased risk for aggravation of bacte
D: 2 and 4
CONTINUE WITH CASE
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INTERVENTION
Bilateral PTC tube placement for recurrent cholangitis with extensive intstone burden.
Biliary culture: Positive for Klebsiella, Enterococci and Pseduomonas.
Dilatation of the bilateral PTC tract with placement of 20 Fr choledochossheaths bilaterally.
Choledochoscopy and biliary stone removal of extensive stone burden in
and left intrahepatic ducts and exchange of PTC tubes.
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INITIAL PTC PLACEMENT
The biliary system was accessed under ultrasound guidance using a 22 gauge Chiba needle through wwas passed. Fluoroscopic images demonstrate moderate to severe bilateral central and intrahepatic dilatation with associated central and intrahepatic biliary duct strictures. In addition, there are multip
defects seen throughout the bilateral biliary ducts, consistent with sludge, debris, and stones.
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CHOLEDOCHOSCOPY(6 weeks post presentation)
Fluoroscopic images show placement of bilateral Amplatz superstiff guidewires through existing bilidrainage tube tracts and dilatation of PTC tracts using two kissing 8 x 4 mm balloons. 20 Fr peel awawere placed through which a 16.5 Fr choledochoscope was advanced into the right and left hepatic
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CHOLEDOCHOSCOPY(6 weeks post presentation)
Extensive right and left intrahepatic biliary calculi were seen involving almost all the segmentincluding the common hepatic duct and CBD. Small casts and debris were removed by scope aZero tip 4 wire basket. Large CBD stone was fragmented using electrohydraulic lithotripsy. Bilapigtail PTC tubes with additional sideholes were placed for additional external and internal dr
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CLINICAL FOLLOW UP
Patient has returned for multiple PTC exchanges with balloon clearCBD, right and left main hepatic ducts, and segmental/subsegment
Labs:Stone analysis: calcium bilirubinate
Repeat common bile duct/hepatic duct brushing cytology negative for malign
Course has been complicated by recurrent episodes of cholangitis cultures positive for Klebsiella, Enterococci and Pseduomonas. Patimaintained on outpatient oral antibiotics (augmentin, PCN, & Cipro
Given recurrent nature of disease, the patient was referred for surconsultation for choledochojejunostomy
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QUESTION SLIDE
4) Treatment option for localized lobar disease when atrophy has occurreincludes:
A: Segmental hepatic resection
B: Orthotopic liver transplant
C: Endoscopic intervention
D: Biliary bypass
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CORRECT!
4) Treatment option which should be considered for localized RPC:A: Segmental hepatic resection. May be considered when calculi are isolathe a single lobe generally after atrophy has occurred. This can reduce thhepatic abscess formation and cholangiocarcinoma.
B: Orthotopic liver transplant
C: Endoscopic intervention
D: Biliary bypass
CONTINUE WITH CASE
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SORRY, THATS INCORRECT!
4) Treatment option which should be considered for localized RPC:A: Segmental hepatic resection. May be considered when calculi are isolathe a single lobe generally after atrophy has occurred. This can reduce thhepatic abscess formation and cholangiocarcinoma.
B: Orthotopic liver transplant
C: Endoscopic intervention
D: Biliary bypass
CONTINUE WITH CASE
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SUMMARY & TEACHING POINTS
Pathogenesis:Found almost exclusively in East and Southeast Asia where infection by parashelminths (Ascaris) or liver flukes (Clonorchis, Opisthorchis, andMetorchis) is c
Parasites induce biliary epithelial damage/fibrosis leading to stricturing and seinfection by enteric bacteria (commonly E. coli, Klebsiella, Pseudomonas, andP
Bacteria-produced gluconidases lead to pigment stone formation; low proteinabnormal phospholipid metabolism may reduce natural inhibition of glucoron
Presentation
Fever, RUQ pain, leukocytosis, elevated alkaline phosphatase and bilirubin
Incidence in Asia decreasing due to improved nutritional standards, but prevathe West increasing due to migration from endemic areas
Recurrent episodes of cholangitis lead to secondary biliary sclerosis and eventbiliary cirrhosis and portal hypertension in later stages
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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 PTCSurgical hepatico-jejunostomy or lobectomy for advanced or isolated left lobe disease
ComplicationsLiver 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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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 12:496
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 NoLo 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, 200
Shoda, J et al: Molecular Pathogenesis of Hepatolithiasis A Type of Low Phospholipid-Associated Cholelithiasis. Frontiers in Bioscience 11, 669-675
Sperling RM et al: Recurrent pyogenic cholangitis in Asian immigrants to the United States: natural history and role of therapeutic ERCP. Dig Dis Sci. 4
Tsui WM et al: Hepatolithiasis and the syndrome of recurrent pyogenic cholangitis: clinical, radiologic, and pathologic features. Semin Liver Dis. 31(1