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Oriental Cholangitis

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Minkyung Choe

on 14 November 2015

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Transcript of Oriental Cholangitis

Oriental Cholangitis
Intrabiliary pigment stone formation biliary tree stricturing and obstruction, with recurrent bouts of cholangitis.

Recurrent pyogenic cholangitis, Oriental cholangiohepatitis, Hong Kong disease, Oriental infestational cholangitis . . .

* Bile stasis and stone formation
within intrahepatic bile ducts

* Parasitic infection and * Bacterial infection

* Abnormal changes:

1. Acute attack of cholangitis

2. Recurrent bouts of cholangitis

3. Complications of RPC

Management and prognosis
1. Laboratory studies

2. Imaging studies

3. Other
* Almost exclusively in people who live in (or have lived in) SE Asia,
but incidence in West’s been increasing due to immigration
* Equal frequency in males and females
* Peak prevalence in 30-40’s
* Rural population
* Lower socioeconomic groups

* Etiology unknown,
but possibly environmental
Especially the left hepatic duct
Innumerable stones - especially calcium bilirubinate and brown pigment
Abnormal bile ducts:
Extra/intrahepatic ductal dilatation and focal stricturing in intrahepatic biliary tree, with fibrosis and inflammatory cell infiltration

Abnormal bile:
Purulent, with bile pigment, desquamated epithelial cells, bacteria, and debris

Other changes:
Hypertrophy of papilla of sphincter of Oddi
Multiple capsular adhesions or subcapsular abscesses
Enlargement & scarring of liver
Eventually liver may atrophy

Geographic clustering of recurrent pyogenic cholangitis where biliary parasites are endemic

Epithelial damage and biliary obstruction, possibly initiating the disease?
Clonorchis sinensis
- Chinese liver fluke, endemic in the East, particularly China, Vietnam, Taiwan, Japan, and Korea.

Opisthorchis sp.
- Particularly O. felineus in SE Asia and central and Eastern Europe (Siberia, and former Soviet Union)

Fasciola sp.
- F. hepatica: worldwide; F. gigantica: tropics

Ascaris lumbricoides
- Intestinal roundworm, most common helminthic human infections worldwide.

Infections in 20-45% of pts w/ Oriental cholangitis

Prevalence of infection & diagnostic test sensitivity

Infection may have resolved after initiating the disease (stone analysis: debris and ova)
Bacterial glucuronidase
Unconjugated bilirubin
Bilirubin glucuronide
Pigment stones and insoluble calcium bilirubinate stones
Complex with Calcium
Persistent obstruction, predisposing to stasis and recurrent infection with additional stone formation
Injury from parasitic infection, or portal bacteremia from lower intestine
1. Lee H, Kowdley KV, Chopra S, Travis AC. Recurrent pyogenic cholangitis. In: UpToDate, Bascow, DS (Ed), UpTodate, Waltham, MA, 2012.
2. Roy PK, Kanth R, et al. "Recurrent Pyogenic Cholangitis." eMedicine. Medscape, 21 Mar 2013. Web. 5 Jul 2013.
Thank you!
Typically 1-2 episodes per year (and history of prior biliary surgery/endoscopic procedures/percutaneous biliary drainage)
Gram-negative bacteremia/sepsis

Perforations of bile duct (& rupture into peritoneum)

Acute pancreatitis (epigastric rather than RUQ pain)

Pyogenic hepatic abscess

In about 1/3 of pts.
Typical presentation: ?
CBC: Leukocytosis with left shift

LFT: Elevated aminotransferases, serum bilirubin, ALP

PT: Depending on vitamin K

Blood cultures: Many pts are bacteremic; abx choice

O&P: Mostly clonorchis
Non-invasive or invasive
RUQ ultrasound
- Initial test; ductal dilatation and stones in 85-90%, liver abscesses

Computed tomography
- Centrally dilated bile ducts with peripheral tapering, bile duct stones, pyogenic liver abscesses, and bilomas. Left lobe predilection and atrophy.

Magnetic resonance cholangiopancreatography
- Detailed visualization of extent of biliary involvement (comp. with ERCP/PTC)
- No therapeutic intervention

Endoscopic retrograde cholangiopancreatography
- Also therapeutic
- Intra/extrahepatic duct dilatation, straightened intrahepatic ducts (periductal fibrosis; "arrowhead"), "missing duct" sign

Percutaneous transhepatic cholangiography
- therapeutic intervention through percutaneous tract

* Prophylactic Abx needed!
Stool for O&P
Liver biopsy generally not helpful
(for diagnosis OC or for finding evidence of parasitic infection)
Acute Long-term
Fluid resuscitation, Abx, and biliary drainage;

More challenging to manage due to:
Multiple intra/extrahepatic stones, stricturing, and ductal distortion

So if ERCP won't work, percutaneous or surgical drainage

1. Remove as many stones as possible with regular surveillance and intervention for stone recurrence

2. Surgical resection of affected hepatobiliary segment with biliary-enteric anastomosis.
Stone clearance: endoscopic, percutaneous, surgical, combined.
Choledochoscope (small caliber endoscope) passed either:
1) percutaneously (through T-tube tract), 2) hepaticocutaneous jejunostomy site, or 3) via transpapillary route during ERCP, usually through multiple sessions;
Recurrence (-30%) and intrahepatic stricture; Also use UDCA
Resection of hepatobiliary segments that are the main source of complications in the minority of patients whom disease is localized (typically in the _____ hepatic ductal system)
Most common causes of death: sepsis, liver failure, portal hypertension

Increased risk for cholangiocarcinoma
(suspect in clinical deterioration; tumors frequently in atrophied left lobe)

Overall disease-related mortality: ~10%

Minkyung Choe
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