Raff Recurrent Pyogenic Cholangitis 03012016

7/25/2019 Raff Recurrent Pyogenic Cholangitis 03012016

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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!



B: Biloma


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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B: Biloma


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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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.



D: Biliary bypass

CONTINUE WITH CASE


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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.



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

Raff Recurrent Pyogenic Cholangitis 03012016

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