2. DEFINITION
• Syndrome characterized by recurrent bouts of
cholangitis, caused by Intrahepatic and Extrahepatic
stones, Biliary duct strictures and parenchymal atrophy.
3. HISTORY
• Digbi in 1930 first described it in Hong Kong in 8
patients with recurrent cholangitis and
hepatolithiasis.
• Defined by Cook in 1954 as a triad of :
I). Recurrent Bacterial Cholangitis
II). Intrahepatic stones
III). Biliary strictures
4. • Stock and Fung coined the term OCH in 1962
• Mage and Moret in 1965 called it - Hong Kong Disease
• Other names :
Recurrent pyogenic cholangitis
Biliary obstruction syndrome of Chinese
Hepatolithiasis
Primary cholangitis
5. EPIDEMIOLOGY
•Predominantly seen in South East Asia
• Now widespread due to immigration and
travel to west
• Most common in Taiwan and South of China
• Equal frequency in males and females
6. • More common in Rural than urban population
• Evidence that the incidence of disease is
declining due to improving economy and living
standard
• In Kashmir, disease is endemic and contributes
12.5% of all patients with Biliary disease
7. ETIOLOGY AND PATHOGENESIS
• Exact Etiology is not known; probably multifactorial
• Clusters of OCH are seen in areas where Biliary Parasites are
common which includes Flukes and Roundworms.
• About half of patients of OCH are infected with Clonorchis
sinensis in endemic areas.
8. • Study by Khuroo et al showed that among 30 patients
of OCH 22 had round worm infestation in the bile
• Role of Biliary Ascariasis in etiology of OCH in
areas has not been well established
• Hypothesis of parasitic infestation is based on
geographical distribution of OCH which resembles that
of Ascaris lumbricoides and Clonorchis.
9. • 12-15% of OCH patients have evidence of Biliary
Ascariasis
In one Study from South Africa, 14 out of 15 patients
were having Ascaris lumbricoides in the Biliary
However, its co-existence was attributed to high
prevalence of Ascaris lumbricoides in the region
without any cause or effect relationship. Hence, strong
association between Biliary Ascariasis and OCH is yet to
be established
10. • Khuroo et al- speaks of strong association between
Biliary ascariasis and OCH. As per the study, among
patients of Biliary ascariasis, 12.6% of patients form
Brown pigment stones in Hepatic duct in when followed
for long term
11. • Pathogenesis is multifactorial
Ascaris lumbricoides invades bile ducts and carries along
with it enteric organisms
• A. lumbricoides cause portal obstruction which leads to
to inadequate biliary drainage –> stasis –> infection
• Recurrent attacks cause papillitis which lead to motor
abnormality and impaired biliary drainage
12. • Sphincter of Oddi dysfunction is seen in few patients.
• Dead worm extracts contain high activity of β-
glucuronidase which facilitates deconjugation of
pigments.
• Dead worms, ova or fragments act as a nidus for stone
formation
13. BACTERIAL AGENTS
• Bacteria as a cause or result in OCH is uncertain
• Bacteria of intestinal origin- E.coli, Klebsiella, Clostridium
perfringes, Pseudomonas are implicated in disease
pathogenesis.
• Likely initiating events is the establishment of infection by
Bowel micro-organisms
14. • Studies in support have isolated organisms from
portal venous blood, bile ducts and liver biopsy
were predominantly of bowel origin
• Bowel organisms reach liver under ordinary
circumstances but clinical infection is seen in:
Highly virulent organisms
Decreased host defence
Severe infection
15. • Once organisms are established, infection begins in
cholangioles and subsequently involves the Portal
• If infection is severe, hepatocytes show vacuolation
and necrosis. Thus, the name Cholangiohepatitis
• Hepatocellular damage will be mild if infection is
confined to cholangioles
• If cholangitis spreads to larger ducts, hepatocellular
damage will be severe
16. • Resolution of infection in early phase results in
restoration of normal morphology
• More intense and persistent or recurrent attacks may
result in fibrosis of ducts and liver damage
17. • In the affected intrahepatic ducts, the number of
mucus glands in the epithelial lining increases
• Integrated role of mucus and bacteria in the
lithogenesis of hepatolithiasis was shown in study
by Zenn and colleagues in 2020
Lipopolysaccharide overexpression of gel forming apo-
nuclei (MUC2 and MUC5AC) in the biliary epithelium –
mucus hypersecretion – due to viscous nature leads to
impaired bile flow and creation of nidus for pigment
deposition
18. Repeated / Severe Infection
Transmural Inflammation of Ducts
Recurrent Attack / Persistent Infection
Fibrosis of Ducts
Smaller Ducts Larger Ducts
Tubular narrowing Stenosis (web like structure)
Biliary stasis
19. β-glucuronidase
(derived from bacteria)
Splits Bilirubin di-glucuronide
Free Ionic Bilirubin
Passes into Duodenum
Calcium Bilirubinate
Di-glucuronide
Coagulates and Consolidates into
Stones
Insoluble
Ionic
Calcium
Soluble
20. HOST FACTORS
• Dietary factors : diet low in proteins and fats
• Low fat diet reduces levels of Cholecystokinin –> stasis –>
stone formation
• Low protein diet reduces levels of inhibitor (beta-Glucuro-
1,4- lactone) of bacteria Glucuronidase, promoting stone
formation
22. STRICTURES
• Found anywhere in biliary tree but more common in major intrahepatic bile ducts
more on left side (because of horizontal nature)
• Left duct comprises 40%
• Right duct 20%
• Both side 40%
23. • In extrahepatic ducts, strictures are web like situated
towards lower end
• In intrahepatic ducts, strictures extend over a short
length
• In smaller ducts strictures are long and more tubular
• Proximal dilatation secondary to stricture is common
• Sometimes, dilatation can be severe known as Cisterns
and little liver parenchyma remains in such affected
24. STONES
• Brown pigment stones (Bilirubinate stones) – soft, pigmented,
earthy and friable
• Stones are irregular in shape and conform the configuration of
duct in which they reside
• Size varies from few mm to 4cm
• In 10% of patients, ducts are filled with biliary debris but no
stone termed as Biliary mud
25. CHANGES IN LIVER
• Liver in Acute phase will be Cholangitic - congested, bile
stained, soft and prone to bleeding
• In Quiescent phase, avascular adhesions are formed between
liver surface and parietal peritoneum
• In Long Standing case, dense vascular adhesions are formed in
parietal peritoneum which contain pockets of pus
26. • Atrophy of affected lobe with compensatory hypertrophy
of other lobe is seen
• With persistent long standing severe disease, liver
cirrhosis and liver failure will follow.
27. CLINICAL MANIFESTATIONS
.Recurrent bouts of cholangitis ( Charcot’s triad)
Most common presenting features :
Cholangitis (44%)
Abdominal pain without overt cholangitis(32%)
Pancreatitis (17%)
Recurrent symptoms for which they have not sought
medical attention
28. CLINICAL MANIFESTATIONS
• Repeated attacks – progressive attacks to bile
ducts and liver parenchyma ,formation of liver
abscesses or cirrhosis.
29. COMPLICATIONS OF OCH
• Cholangitis and sepsis
• Biliary cirrhosis /liver failure
• Portal hypertension / portal vein thrombosis
• Liver abscess
• Pancreatitis
• Cholangiocarcinoma
• Choledochoduodenal fistula
30. COMPLICATIONS
• Sepsis and abscess formation at distant sites – lungs /
brain
• Rupture of obstructed pus filled bile ducts into the
peritoneum
• Formation of fistula into the GIT or anterior abdominal
wall
• PVT and hemobilia
31. • “Nothing is perfect in the management of OCH
while surgical and radiological techniques are jointly
beneficial, the ultimate panacea of OCH treatment
may be medical control of biliary lithogenic factors.”
• Van sonneberg et al AJR 1986
32. DIAGNOSIS
• Acute cholangitis accompanied by hepatolithiasis is
diagnosed by findings of systemic inflammation,
cholestasis and imaging showing Intra hepatic biliary
dilatation, strictures and stone formation.
34. IMAGING
US HBS :
– DUCTAL DILATION AND STONES
CAN BE SEEN IN 85 TO 90 OF %
PATIENTS – hepatic abscesses.
Calcium bilirubinate stones –
shows same or slightly higher
echogenicity than liver and weak
acoustic shadow.
Cholesterol stones show opposite.
35. CT SCAN
DILATED CENTRAL INTRAHEPATIC
DUCTS,
• ABRUPT TAPERING OF PERIPHERAL
DUCTS,
• ENHANCEMENT OF THE DUCT
WALLS,
• HEPATIC ABSCESSES, BILOMAS, AND
STONES
– DETERMINE WHETHER THE DISEASE
IS LOCALIZED (USUALLY TO THE LEFT
LOBE) WHETHER ATROPHY HAS
DEVELOPED
36. CT SCAN
• Can differentiate intrahepatic stones from pneumobilia.
• Calcium bilirubinate stones appear hyperintense on CT
• Location of stones
• bile duct dilatation for strictures proximal and distal to stone
sites.
• Volumetric and contour alteration of liver can be seen.
• Segmental hepatic atrophy in hepatolithiasis appear as a
crowding of bile duct branches ,diminished portal blood flow
and loss of portal vein branches
• And is an indication for liver resection.
37. CHOLANGIOGRAPHY
• PERCUTANEOUS TRANSHEPATIC AND ENDOSCOPIC
RETROGRADE CHOLANGIOGRAPHY
• INTRA- AND EXTRA- HEPATIC DUCT DILATATION
• STRAIGHTENED INTRAHEPATIC DUCTS WITH LESS ACUTE OR
RIGHT-ANGLED BRANCHING PATTERNS (AS A RESULT OF
EXTENSIVE PERIDUCTAL FIBROSIS)
• DECREASED ARBORIZATION AND ACUTE TAPERING OF THE
PERIPHERAL DUCTS – CLASSIC “ARROWHEAD” SIGN;
“MISSING DUCT” SIGN WHERE THERE IS COMPLETE
OBSTRUCTION OF A BILE DUCT.
38. CHOLANGIOSCOPY
• Access roots to the bile duct used for imaging are
sequentially dilated,a fistula is created then cholangioscopy
can be performed.
• Stones and strictures can be directly visualised by
cholangioscopy and biopsy ,treatment such as stone removal
can be performed.
41. MANAGEMENT
• ACUTE EPISODE
• -CONTROL OF BILIARY SEPSIS
• -DRAINAGE +/- EXTRACTION OF STONES
• ERCP
• PTC
• .DEFINITIVE TREATMENT
• CORRECTION OF ANATOMIC ABNORMALITIES/
SOURCES OF CHRONIC INFECTIONS
42. MEDICAL MANAGEMENT
• Ursodeoxycholic acid has a supplementary role.offers
liver cytoprotection and leads to accelerated activity of
bile acids / bilirubin metabolising enzymes,activity of ABC
transporter proteins, increased bile flow rate and decline
of bile mucin viscosity.
• Simvastatin reduces Plasma and biliary cholesterol Levels.
43. MANAGEMENT OF ACUTE COMPLICATIONS
• CHOLANGITIS:
• – FLUID RESUSCITATION, ANTIBIOTICS, AND BILIARY DRAINAGE:
• MAY BE MORE DIFFICULT TO ACHIEVE DRAINAGE IN PATIENTS
WITH OCH SINCE MULTIPLE INTRA- AND EXTRAHEPATIC STONES
MAY BE PRESENT.
• STRICTURING, INTRAHEPATIC DUCT STONE IMPACTION, AND
DUCTAL ANGULATION CAN ADD FURTHER CHALLENGE TO
ENDOSCOPIC INTERVENTION
• ERCP FAILS – REQUIRE PERCUTANEOUS OR SURGICAL DRAINAGE
44. LONG TERM COMPLICATIONS
• STEP 1: REMOVAL OF AS MANY STONES AS POSSIBLE WITH
REGULAR SURVEILLANCE AND INTERVENTION FOR STONE
RECURRENCE.
• STEP 2: SURGICAL RESECTION OF THE AFFECTED HEPATOBILIARY
SEGMENT WITH A BILIARY-ENTERIC ANASTOMOSIS. (HCD VS HJ)
• OPTIMAL STRATEGIES HAVE NOT BEEN WELL ESTABLISHED IN
LARGE COMPARATIVE STUDIES.
• COMBINATION OF APPROACHES MAY BE REQUIRED.
• INCIDENCE OF RETAINED STONES AFTER OPERATION IS 48 TO 77%,
RECURRENCE >30%
45. STONE REMOVAL
• CHOLEDOCHOSCOPE PASSED:
• – PERCUTANEOUSLY THROUGH THE T-TUBE TRACT,
• – A HEPATICOCUTANEOUS-JEJUNOSTOMY SITE
• – TRANSPAPILLARY ROUTE DURING ERCP
• PERMIT DILATION OF INTRAHEPATIC STRICTURES
• THE FRAGMENTATION OF STONES THAT ARE DIFFICULT TO REMOVE
WITH CONVENTIONAL MEANS– MECHANICAL, ELECTROHYDRAULIC,
OR LASER LITHOTRIPS
46. ENDOSCOPIC TREATMENT
• PRESENCE OF STRICTURES, PERIPHERAL STONE IMPACTION, DUCTAL
ANGULATION –CHALLENGES OF ENDOSCOPIC EXTRACTION
• Definitive treatment of intrahepatic stones generally includes complete
clearance of stones and elimination of bile stasis.
• Biliary strictures which are found in 35 to 96 % of patients with
hepatolithiasis are major factors in stone recurrence.
47. SURGERY FOR OCH
• HEPATIC RESECTION OF AFFECTED HEPATOBILIARY SEGMENTS
• – FEASIBLE IN THE MINORITY OF PATIENTS IN WHOM THE DISEASE
IS LOCALIZED (TYPICALLY IN THE LEFT HEPATIC DUCTAL SYSTEM)
• – BILATERAL PARTIAL HEPATECTOMY HAS ALSO BEEN DESCRIBED
• – THE GOAL OF SURGERY IS TO RESECT THE AREA OF RECURRENT
INFECTION, BILIARY STASIS, AND HEPATIC ATROPHY.
48. • AS A GENERAL RULE, THESE REPORTS HAVE SUGGESTED
HIGHER RATES OF RESIDUAL BILIARY STRICTURES AND
MORE FREQUENT STONE RECURRENCE IN PATIENTS WHO
UNDERWENT PTC LITHOTOMY WITHOUT HEPATIC
RESECTION, EVEN WITH COMPLETED STONE REMOVAL,
COMPARED TO THOSE WHO HAD LEFT LOBECTOMY WITH A
BILIARY DRAINAGE PROCEDURE [67-69].
• BETTER QUALITY OF LIFE, LOWER RATES OF SECONDARY
BILIARY CIRRHOSIS, CHOLANGIOCARCINOMA, AND
MORTALITY HAVE ALSO BEEN SUGGESTED IN PATIENTS
TREATED SURGICALLY
49. • FREQUENTLY REQUIRE A BILIARY ENTERIC ANASTOMOSIS
(SUCH AS A HEPATICOJEJUNOSTOMY),– THE EFFICACY AND
SAFETY OF THIS APPROACH REMAIN CONTROVERSIAL.
• STANDARD BILIARY DRAINAGE PROCEDURES (SUCH AS
CHOLECHODUODENOSTOMY, ROUX-EN-Y
CHOLEDOCHOJEJUNOSTOMY, OR SPHINCTEROPLASTY)
ARE GENERALLY CONTRAINDICATED – RESIDUAL
STRICTURED BILIARY SEGMENTS MAY NOT BE DRAINED
ADEQUATELY
• LONG-TERM BILIARY ACCESS HAS BEEN ACHIEVED BY
CREATION OF A CUTANEOUS STOMA FROM A ROUX LIMB
OF A HEPATICOJEJUNOSTOMY (HUTSON-RUSSELL LOOP) IN
SOME CASE SERIES
50. SUBPARIETAL HJ BILIARY ACCESS LOOP
• RECOGNIZED TECHNIQUE FOR LONG TERM MX OF PRIMARY IH STONE
DISEASE:
• SURGICAL REMOVAL OF STONES WITH AN EXTRAHEPATIC DRAINAGE
PROCEDURE
• LONG TERM ACCESS LOOPS/ROUTES – REPEATED BILIARY
INSTRUMENTATION: STONE RETRIEVAL OR STRICTURE DILATATION
• PROVIDES GOOD ACCESS TO THE BILIARY TREE; STONE REMOVAL
AROUND 85% OF PATIENTS
51. BILIARY STOMA
• MUCUS AND BILE LEAKAGE– CUTANEOUS IRRITATION,
EXCORIATION (INDEPENDENT OF AFFERENT LOOP LENGTH)
• EARLY CLOSURE COMPLICATIONS – 17%
• –PERSISTENT WOUND INFX
• – FISTULA FORMATION
• –PARASTOMAL HERNIA
• – RE-OPENING OF STOMA FOR FURTHER RX AFTER 2 YEARS
52. INTRODUCTION
Fazl Q. Parray based on their institutional experience
developed the following grading system and management
strategy for patients with OCH. According to them, the basic
modality of management continues to be ERCP; however, the
following surgeries are only carried on the patients after failed
ERCP trial or advanced grades of OCH.
53. Grade Disease Proposed Surgical Management
1 Disease limited to the extra hepatic ducts
with stones/worms, No Liver parenchymal disease, no
strictures, and CBD size less than 1.5 cm.
CBD exploration with T Tube
drainage
2 Disease limited to the extra hepatic ducts with
stones/worms, No liver parenchymal disease, with strictures
of CBD, or CBD size more than 1.5 cm.
CBD exploration with hepaticojejunostomy with
or without access loop
3 Disease involving intrahepatic ducts right or left, with
stones/worms with dilatable strictures and no liver
parenchymal disease.
CBD exploration with clearance of intrahepatic stones and
dilatation of strictures with hepaticojejunostomy with an
access loop.
4 Disease involving intrahepatic ducts right or left, with
stones/worms with severe non-dilatable strictures or
parenchymal disease on same side.
Liver resections, left lateral sectorectomy, right or left
hepatectomy.
5 Disease involving both intrahepatic ducts, with
stones/worms with severe non-dilatable strictures or
parenchymal disease on both side.
Hepatectomy on more effected side with clearance of
contralateral ducts and dilatation of the strictures and
hepaticojejunostomy with access loop, otherwise liver
transplant.
