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Transcript of 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
* 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
Purulent, with bile pigment, desquamated epithelial cells, bacteria, and debris
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?
- Chinese liver fluke, endemic in the East, particularly China, Vietnam, Taiwan, Japan, and Korea.
- Particularly O. felineus in SE Asia and central and Eastern Europe (Siberia, and former Soviet Union)
- F. hepatica: worldwide; F. gigantica: tropics
- 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)
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.
Typically 1-2 episodes per year (and history of prior biliary surgery/endoscopic procedures/percutaneous biliary drainage)
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
- Initial test; ductal dilatation and stones in 85-90%, liver abscesses
- 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)
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%