Recurrent Pyogenic (Oriental) Cholangitis

KK Madhavan

Oriental cholangiohepatitis –Early reports

( Mage et al : Annals of Surgery , Aug 1965)

1957 - 1963 Chinese Occidental

Common duct exploration 30 (80%) 76 ( 20%)

Common duct stones 23 ( 60%) 46 ( 12%)

Stoneless cholecystitis 12 (30%) 3 (0.8%)

Chlonorchis Sinensis 3 0

Oriental cholangiohepatitis –Early reports

Ong GB et al – A study of recurrent Pyogenic cholangitis .Arch Surg 84:409 ; 1962

Walter W – Cholangiohepatitis . Editorial . JAMA , 178 : 166 ; 1961

Hoerr SO – Stoneless gall bladder but stonesin the common bile duct

Arch Surg 85 : 321 ; 1962

Nomenclature

Recurrent pyogenic cholangitis ( PRC)

Oriental Cholangitis

Hong Kong disease

Intrahepatic pigment stone disease

Biliary obstruction of the Chinese

RPC – areas of involvement

Right Duct alone ( 20%)

Left Duct alone(40%)

Both ducts involved(40%)

GB disease only in 20% of cases of RPC

Complications of RPCAttacks of cholangitis and sepsis

Biliary cirrhosis / Liver failure

Portal hypertension / PVT

Liver abscess

Pancreatitis

Cholangiocarcinoma

Cholangiocarcinoma in RPC

Incidence – 2 – 13 % of patients with intrahepatic stones

? Co – incidence ? Association

DD – Inflammatory pseudotumour ( common in RPC)

Role of tumour markers ( CEA , Ca 125 , Ca 19-9 )

Role of PET scan – not defined

Indicators of onset of CC in RPC

Increase in jaundice without cholangitis ( ef: PSC)

Sudden unexplained weight loss

Marked rise in alkaline phosphatase

Incompletely cleared stones at previous operations

Rising tumour markers ( Ca 125 / CEA)

A mass on CT / MRI ( DD from inflammatory pseudotumours)

“ Nothing is perfect in the management of

OCH . While surgical and radiological

techniques are jointly benefecial , the

ultimate panacea of OCH treatment may be

medical control of biliary lithogenic factors “

van Sonnenberg et al . AJR 1986 ; 146 : 327 - 31

Epidemiology• intrabiliary pigment stone formation, stricturing of

the biliary tree and biliary obstruction with recurrent bouts of cholangitis

• almost exclusively in people who live or who have lived in southeast Asia

• peak prevalence in the third and fourth decades of life

• Su et al.1 reported the relative incidence to be 20to 30% of all cases of gallstone disease (China and Taiwan)

Su CH, Lui WY, Peng FK. Relative prevalence of gallstonedisease in Taiwan. A nationwide cooperative study. Dig DisSci 1992;37:764–768.

Pathogenesis• bile stasis and stone formation proximal to biliary

strictures recurrent cholangitis• stone formation occurs de novo within the

intrahepatic bile ducts = hepatolithiasis• Transient portal bacteremia intiates cycle of

infection and 2° stone formation• stones = calcium bilirubinate or brown pigment

in contrast to the cholesterol stones more commonly seen in patients with other gallstone-related diseases.

• bile ducts are markedly abnormal– extrahepatic and intrahepatic ductal dilatation– focal areas of stricturing in the intrahepatic

biliary tree• biliary wall is fibrotic with inflammatory cell

infiltration. • bile is purulent and filled with debris,

composed of bile pigment, desquamated epithelial cells, bacteria, and pus

• left hepatic duct, especially the left lateral segmental duct, is usually affected in the early course of the disease– reason unknown. – left hepatic ducts come off at a more acute

angle compared with the right hepatic ducts, thus predisposing to stasis and stricture formation.

• hypertrophy of the papilla of the sphincter of Oddi – repeated passage of stones

• enlargement and scarring of the liver with multiple capsular adhesions or deep subcapsular abscesses.

• liver atrophy - multiple episodes of infection and inflammation, leaving segments containing nothing more than fibrous tissue and dilated ducts

CTAP

CTAP

CTAP

Etioliogy

• infections can be demonstrated in 20 to 45% of patients with RPC– Parasitic infection: liver trematodes/flukes

Clonorchis sinensis, Opisthorchis species, and Fasciola hepatica

– helminthic human infections: Ascaris lumbricoides, an intestinal roundworm

– Bacterial: E Coli• Stasis

Clinical Manifestation

• 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.

– Repeated attacks progressive damage to the bile ducts and liver parenchyma formation of liver abscesses or cirrhosis.

Complications

• sepsis and abscess formation at distant sites - lungs / brain

• rupture of obstructed pus-filled bile ducts into the peritoneum

• formation of a fistula into the gastrointestinal tract or abdominal wall

• Portal vein thrombosis and hemobilia

• Dx - imaging of the liver and biliary system in patients with a clinically compatible history

Imaging• US HBS:

– ductal dilation and stones can be seen in 85 to 90of % patients

– Hepatic abscesses• 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

• Cholangiography– MRCP/ERCP/PTC

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.

• Anteroposterior ERCP image shows:– abrupt tapering of the left

lateral segmental duct (arrows) without depiction of calculus and the peripheral duct,

– results in an arrowhead appearance and missing duct

– fails to show any intrahepatic ductal abnormality in the right lobe.

Park MS et al, Recurrent Pyogenic Cholangitis: Comparison between MR Cholangiography and Direct CholangiographyRadiology Sept 2001

• Anteroposterior percutaneous transhepatic cholangiogram:– severe stricture

(arrow), with dilated ducts, multiple filling defects, and abrupt tapering in the right anterior segment

Park MS et al, Recurrent Pyogenic Cholangitis: Comparison between MR Cholangiography and Direct CholangiographyRadiology Sept 2001

Management

• Principles:– treatment of acute complications – long-term prevention of complications

• Hepatolithiasis:– Complicated vs uncomplicated

• the absence or presence of biliary strictures, infections, septic manifestations, and intrahepatic abscess.

Complicated vs Uncomplicated Hepatolithiasis

• Uncomplicated/unfit for sx• percutaneoustranshepatic choledochoscopic or

endoscopic retrograde cholangiopancreatographic (ERCP) routes are used for stone extraction or stricture dilation

• Complicated / failed non-sx Rx• surgical procedures which include:

– CBD/common hepatic duct (CHD) exploration with extraction of stones,

– with or without unilateral/bilateral partial hepatectomy.

Acute Complications

• Cholangitis: – fluid resuscitation, antibiotics, and biliary drainage:

• may be more difficult to achieve drainage in patients with RCP 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

• Surgery often involves cholecystectomy with common bile duct exploration and T-tube drainage.

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%

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 lithotripsy

Endoscopic Rx:

• Presence of strictures, peripheral stone impaction, ductal angulation challenges of endoscopic extraction

• Choice of puncture path depends on • Location of stones• No. of stone-bearing ducts and segments• Tract is dilated with biliary drainage catheter

inserted 4 to 6/52 for maturation of tract tract dilation

• All strictures were biopsied

• Intrahepatic stricture – major determinant for the recurrence of stones / symptoms

• Hepatic resection – localised disease to one liver segment/lobe

Surgery for RPC

• 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.

Hepatectomy for bilateral primary hepatolithiasis: a cohort study.Yang T; Lau WY; Lai EC; Yang LQ; Zhang J; Yang GS; Lu JH; Wu MCAnn Surg. 2010 Jan;251(1):84-90.

• no large controlled trials comparing hepatic resection with other management strategies. – retrospective reports of patients followed in major centers in Asia

over many years. • 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[52,62,64].

• 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

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

• Biliary-enteric anastomosis: side to side anastomosis btw hepatic duct confluence and jejunal Roux-en-Y

• Ligaclips – mark anastomosis for radiologic identification

IJ Beckingham et al. Subparietal hepaticjejunal access loop for long term management of intrahepatic stones.BJS 1998;85. 1360-1363

Jejunum

Roux en Y JJ

Anastomosis of CHD and JJ

SMA: RHA and GDA

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

Surgical treatment of hepatolithiasis: long-term results.Jan YY; Chen MF; Wang CS; Jeng LB; Hwang TL; Chen SCSurgery 1996 Sep;120(3):509-14.BACKGROUND: Hepatolithiasis is a common disease in East Asia and is prevalent in

Taiwan. Surgical and nonsurgical procedures for management of hepatolithiasis have been discussed, but long-term follow-up results of surgical treatment of hepatolithiasis are rarely reported.

METHODS: We conducted a retrospective study of case records of patients with hepatolithiasis who underwent surgical or nonsurgical percutaneous transhepatic cholangioscopy treatment. Of 614 patients with hepatolithiasis seen between January 1984 and December 1988, 427 underwent follow-up after surgical (380) or percutaneous transhepatic cholangioscopy (47) treatment for 4 to 10 years and constituted the basis of this study.

RESULTS: Long-term results of 427 patients with hepatolithiasis after surgical and nonsurgical treatment within 4 to 10 years of follow-up were recurrent stone rate 29.6% (105 of 355), repeated operation 18.7% (80 of 427), secondary biliary cirrhosis 6.8% (29 of 427), late development of cholangiocarcinoma 2.8% (12 of 427), and mortality rate 10.3% (44 of 427). The patients with hepatectomy had a better quality of life (symptom-free) with a lower recurrent stone rate (9.5%), lower mortality rate (2.1%), and lower incidence of secondary biliary cirrhosis (2.1%) and cholangiocarcinoma (0%) than did the nonhepatectomy group (p<0.01). The patients without residual stones after choledochoscopy had a better quality of life than did the residual stone group (p<0.01).

CONCLUSIONS: Long-term follow-up study of hepatolithiasis after surgical treatment revealed a high recurrent stone rate (29.6%) that required repeated surgery and a high mortality rate (10.3%) resulting from repeated cholangitis, secondary biliary cirrhosis, and late development of cholangiocarcinoma. Patients who received hepatectomy or without residual stones after choledochoscopy had a good prognosis and quality of life.

Department of Surgery, Chang Gung Memorial Hospital, Linkou Medical Center, Chang Gung Medical College, Taipei, Taiwan.

Long-term results of hepaticojejunostomy for hepatolithiasis.Kusano T; Isa TT; Muto Y; Otsubo M; Yasaka T; Furukawa MAm Surg 2001 May;67(5):442-6.

The results of a hepaticojejunostomy as a biliary-enteric bypass for benign disease are usually excellent. On the other hand, hepatolithiasis features a high rate of residual and recurrent stones with cholangitis after surgery.

This study aims to evaluate the long-term results of a hepaticojejunostomy (HJ) for hepatolithiasis regarding both the degree of the occurrence of postoperative cholangitis and the outcome.

The clinical records of 159 patients with hepatolithiasis who underwent surgical treatment over a 23-year period were also retrospectively reviewed. Ninety-four of 159 patients underwent a hepatecetomy and 65 patients were subjected to liver-preserving surgery by means of intra- and postoperative endoscopic lithotripsy. In addition 72 patients underwent a hepaticojejunostomy.

The rate of residual or recurrent stones was 31.4 % after complete stone removal. Twenty-two (30.6%) of the 72 patients developed some kind of cholangitis. This rate was significantly higher than that (three of 87 patients) of the non-biliary-enteric anastomosis group regarding the occurrence of biliary complications. We conclude that the use of a hepaticojejunostomy for patients with possible residual stones or intrahepatic bile duct lesions remains controversial.

First Department of Surgery, Faculty of Medicine, University of the Ryukyus, Okinawa, Japan

Prognosis

• most common causes of death in patients with RCP are:– Sepsis– liver failure– complications from biliary cirrhosis - portal

hypertension. • increased risk for cholangiocarcinoma.

– annual incidence of 5% with hepatolithiasis seen during a three-year period

A reappraisal of cholangiocarcinoma in patient with hepatolithiasis.Chen MF; Jan YY; Wang CS; Hwang TL; Jeng LB; Chen SC; Chen TJCancer 1993 Apr 15;71(8):2461-5.

Surveillance for recurrence

• serial US HBS every 3-6/12• recurrent symptoms undergo an ERCP +/-

therapeutic intervention

Literature Review: Hepatectomy for Hepatolithiasis

• Surgery 2004• Aim: to evaluate the perioperative and

long-term results of hepatectomy for hepatolithiasis.

• Method: 103 consecutive patients with hepatolithiasis who underwent hepatectomy from 1989 to 2001

Hepatectomy• seems to be the most definitive approach for

hepatolithiasis, – can remove the stones and the biliary stricture

simultaneously, thus reducing the risk of recurrentintrahepatic stones.

• Indications for partial hepatectomy were as follows: – bile duct stricture associated with stones – atrophy of the affected liver segments or lobe – Presence of liver abscess; – cholangiocarcinoma found or suspected clinically

• stone recurrence rate of 9% after a median follow up of 56months is rather low compared with that of nonsurgical approaches or hepaticojejunostomy alone, which are generally associated with a longterm stone recurrence rate of 30% to 54%

• most studies demonstrated a high recurrence rate after percutaneous lithotripsy, especially in patients with biliary stricture

• Immediate outcomes:– stone clearance rate , operative morbidity ,mortality.

• Long-term results:– Stone recurrence rate, survival

• Results:– immediate stone clearance rate was 90%– final stone clearance rate was 98% after subsequent

choledochoscopic lithotripsy by cutaneous stoma or T-tube route

– operative morbidity 28%– hospital mortality rates 2%

• Multivariate analysis:– Right hepatectomy ( P = .006) and

preoperative hyperbilirubinemia ( P = .038) were predictive of postoperative complications.

• Cholangiocarcinoma was the only significant prognostic factor of long-term survival by Cox regression analysis

Approach to Bilobar Disease

• hepatic resection of the more severely affected side combined with hepaticocutaneous-jejunostomy for removal of stones in the other side is an effective approach.

• careful evaluation of the hilar bifurcation with choledochoscopy

Conclusions • hepatic resection is a safe and effective treatment for

intrahepatic stones in association with intrahepatic bile duct stricture, liver abscess, or cholangiocarcinoma.

• addition of hepaticocutaneous-jejunostomy is useful for removal of residual stones or recurrent stones and – is particularly indicated in patients with bilateral stone disease

after hepatic resection in one side. • Hepatic resection is associated with a low incidence of

long-term stone recurrence or recurrent cholangitis. • coexisting cholangiocarcinoma remains a common

cause of late death in patients with hepatolithiasis even after hepatic resection

• World J Surgery 2007• Aim: evaluate immediate and long-term

results of hepatectomy as treatment for hepatolithiasis.

• Method: 123 consecutive patients who underwent hepatectomy for hepatolithiasis at our institution from 2000 to 2005 were analyzed retrospectively.

Results

• immediate stone clearance rate after hepatectomy and intraoperative choledochoscopy was 92.7% – Of the 9 patients with residual stones, 5 had stones in

both lobes of the liver preoperatively– 4/9 patients were treatedsuccessfully with postoperative cholangioscopy from

the T-tube tract or ERCP. • final stone clearance rate was 96% (118 out of

123).

• surgical morbidity was 33.3% (41 out of 123) with wound infection (21 out of 123, 17.1%) the most common complication

• During a median follow-up of 40.3 months (range 6–52), 7 patients (5.7%) had recurrent stones

• All 7 had recurrent stones in the right lobe, even though the original stones were distributed in both lobes of the liver.

Conclusions

• Hepatic resection is a safe and effective treatment for hepatolithiasis.

• associated with a high immediate stone clearance rate and low long-term stone

• recurrence rate. • associated cholangiocarcinoma and

recurrent stone-induced cholangitis remain common problems that adversely affect long-term survival

• J Gastrointestinal Surgery 2007• Aim: to analyze the rate of residual stones

and complications of invasive and noninvasive treatments and procedures, as well as the long-term outcome including stone recurrence

• Method: retrospective analysis 2,660 patients from January 1971 to June 2006, with cholelithiasis

Conclusion

• hepatectomy should be considered when the stones are located in strictured bile ducts, especially within the unilateral lobe.

• recommend that hepatectomy should be considered when stones are localized in the unilateral lobe after PTCSL– because PTCSL is considered to be a difficult

treatment as a radical therapy for primary hepatolithiasis.

• Annals of Surgery 2010• Aims: to evaluate the perioperative and long-

term outcomes of partial hepatectomy for complicated bilateral hepatolithiasis, based on our algorithm of management of hepatolithiasis.

• Method: retrospective study of 136 patients (January 2000 to December 2006) with complicated hepatolithiasis who underwent partial hepatectomy in the Eastern Hepatobiliary Surgery Hospital (Shanghai, China) were studied.

• indications for partial hepatectomy• intrahepatic biliary strictures which failed other forms of

treatment,• atrophy of the affected liver segment(s) or hemi-liver, • presence of liver abscess, • suspected cholangiocarcinoma, • segmental bile ducts filled with stones, which were

inaccessible to treatment by other approaches.

• Partial hepatectomy was only performed for patients with recurrent, troublesome localized, and severe disease.

Thank You

?Questions?