The Hong Kong Disease The Hong Kong Disease ––

Management UpdatesManagement Updates

Dr. YF YeungDr. YF Yeung

Department of SurgeryDepartment of Surgery

Prince of Wales HospitalPrince of Wales Hospital

JHSGR 17/5/2008

The Hong Kong DiseaseThe Hong Kong Disease

SARS

The Hong Kong DiseaseThe Hong Kong Disease

Recurrent Pyogenic Cholangitis Recurrent Pyogenic Cholangitis (RPC)(RPC) Oriental cholangitisOriental cholangitis Oriental cholangiohepatitisOriental cholangiohepatitis Intrahepatic pigmented Intrahepatic pigmented

calculus diseasecalculus disease

Recurrent Pyogenic Recurrent Pyogenic CholangitisCholangitis

Cook in 1954Cook in 1954 Repeated primary biliary infectionRepeated primary biliary infection Pus-forming bacteriaPus-forming bacteria Multiple stones and strictures in the biliary Multiple stones and strictures in the biliary

treetree

Pathogenesis not well understood Pathogenesis not well understood nowadaysnowadays

Calcium bilirubinate stones within extra- Calcium bilirubinate stones within extra- and intraheptic biliary ductsand intraheptic biliary ducts

AetiologyAetiology

? Oriental diet? Oriental diet Low saturated fat: biliary stasisLow saturated fat: biliary stasis Low protein diet: increased formation of calcium Low protein diet: increased formation of calcium

bilirubinate stonesbilirubinate stones

? Poor environmental hygiene? Poor environmental hygiene Recurrent enteric infection and portal Recurrent enteric infection and portal

bacteraemiabacteraemia

? Clonorchis sinensis and Ascaris ? Clonorchis sinensis and Ascaris lumbricoidslumbricoids

EpidemiologyEpidemiology Predominantly lower socio-economic class Predominantly lower socio-economic class

and rural areasand rural areas Male = FemaleMale = Female Peak age incidence: 3rd to 4th decadesPeak age incidence: 3rd to 4th decades Overall incidence is decreasing in East AsiaOverall incidence is decreasing in East Asia

HK experienceHK experience 1950-1952: 30 patients / year1950-1952: 30 patients / year 1984-1989: 22.8 patients / year1984-1989: 22.8 patients / year

Lo et al. Lo et al. HKMJ 1997HKMJ 1997

Increasing incidence in the West due to Increasing incidence in the West due to Asian immigrantsAsian immigrants

Imaging Imaging FeaturesFeatures

ERCPERCP Truncated tree Truncated tree

signsign

Ductal ectasiaDuctal ectasia

Abrupt taperingAbrupt tapering

Arrow head Arrow head appearanceappearance

Imaging FeaturesImaging Features

Percutaneous Percutaneous Transhepatic Transhepatic CholangiographCholangiographyy

Severe strictureSevere stricture

Dilated ductsDilated ducts

Multiple filling Multiple filling defectsdefects

Imaging Imaging FeaturesFeatures

MRCP

•Dilated ducts

•Strictures

•Filling defects

Imaging Imaging FeaturesFeatures CTCT

HepatolithiasisHepatolithiasis

Parenchymal Parenchymal atrophyatrophy

Obliterated portal Obliterated portal veinvein

Management - Management - MultidisciplinaryMultidisciplinary

Acute episodeAcute episode Control of biliary sepsisControl of biliary sepsis Drainage +/- extraction of stonesDrainage +/- extraction of stones

ERCPERCP PTCPTC

Definitive treatmentDefinitive treatment Correction of anatomic abnormalities/ Correction of anatomic abnormalities/

sources of chronic infectionssources of chronic infections

Definitive ManagementDefinitive Management

Surgical

ECBD

Liver Resection

Drainage Procedure

Endoscopic

Percutaneous Transhepatic Cholangioscopic Lithotripsy (PTCL)

“mother-baby” endoscope system

HepatectomyHepatectomy

IndicationsIndications Stones localized in unilateral lobeStones localized in unilateral lobe Bile duct stricture Bile duct stricture Atrophy of affected segments/ lobeAtrophy of affected segments/ lobe Suspected cholangiocarcinomaSuspected cholangiocarcinoma Failed / recurrent disease after non-Failed / recurrent disease after non-

operative treatmentoperative treatment

HepatectomyHepatectomySeriesSeries nn MortalityMortality MorbidityMorbidity Stone Stone

ClearanceClearance

CholangioCholangio-CA-CA

FU FU (mth(mth

))

Recur-Recur-rencerence

Chen

2004

103 2% 28% 98% 10% 56 7.8%

Cheung

2005

52 3.8% 33.3% 98% 3.8% 58 13.3%

(5 yrs)

Uchiyama 2007

38 0% 23.7% 100% 7.9% 108 13.9%

(5 yrs)

Lee

2007

123 1.6% 33.3% 92.7% 2.4% 40.3 5.7%

Drainage ProcedureDrainage Procedure

PrinciplePrinciple Eliminate biliary stasisEliminate biliary stasis Newly formed stones can pass unimpeded into Newly formed stones can pass unimpeded into

the bowelthe bowel

IndicationsIndications Extrahepatic ductal stonesExtrahepatic ductal stones Extrahepatic biliary strictureExtrahepatic biliary stricture Grossly dilated common duct with problem of Grossly dilated common duct with problem of

bile stasisbile stasis

Drainage ProcedureDrainage Procedure Choledochoduodenostomy (CD)Choledochoduodenostomy (CD)

Sump syndromeSump syndrome Ascending cholangitisAscending cholangitis High risk of stasisHigh risk of stasis

Hepaticojejunostomy (HJ)Hepaticojejunostomy (HJ) Hinder post-operative choledochoscopic Hinder post-operative choledochoscopic

removal of residual stones removal of residual stones Hepaticocutaneous jejunostomy with a stoma Hepaticocutaneous jejunostomy with a stoma

for easy accessfor easy access Possible complications: Possible complications: fistula, infection, fistula, infection,

parastomal hernia, early stoma closureparastomal hernia, early stoma closure

SphincteroplastySphincteroplasty

Parilla P et al. BJS 1991Rat P et al. Hepatogastroenterology 1993

Huang et al. Am J Gastroenterol 2003

Is Drainage Procedure Is Drainage Procedure a MUST after a MUST after hepatectomy?hepatectomy?

World J Gastroenterololgy 2006

Intra-op Intra-op bleedingbleeding

OT timeOT time Residual Residual stonestone

Post-op Post-op cholangitischolangitis

Liver Liver resection(76)resection(76)

500ml500ml 282min282min 18.4%18.4% 22%22%

HJHJ

ECBD (47)ECBD (47) 300ml300ml 226min226min 23.4%23.4% 27%27%

314 314 patientspatients

Liver Liver resection (85)resection (85)

300ml300ml 189min189min 21.2%21.2% 8.2%8.2%

T-tubeT-tube

ECBD (106)ECBD (106) 150ml150ml 166min166min 34%34% 35.7%35.7%

Median FU 7.6 years (2-12)Median FU 7.6 years (2-12)

Concluded indications for HJConcluded indications for HJ Hepatolithiasis complicated with extrahepatic ducts or Hepatolithiasis complicated with extrahepatic ducts or

its second branches strictureits second branches stricture

Hepatolithiasis with congenital bile duct dilatation in Hepatolithiasis with congenital bile duct dilatation in which the dilated bile duct should be resectedwhich the dilated bile duct should be resected

Dysfunction of the papilla of VaterDysfunction of the papilla of Vater

World J Gastroenterololgy 2006World J Gastroenterololgy 2006

Percutaneous Transhepatic Percutaneous Transhepatic Cholangioscopic Lithotripsy Cholangioscopic Lithotripsy

(PTCL)(PTCL) IndicationsIndications

Stones distributed in multiple segmentsStones distributed in multiple segments

Previous biliary surgeryPrevious biliary surgery

Poor surgical riskPoor surgical risk

Refuse surgeryRefuse surgery

PTCLPTCL Causes of incomplete stone Causes of incomplete stone

clearanceclearance Biliary strictureBiliary stricture Bile duct angulationBile duct angulation Muddy stones with sludgeMuddy stones with sludge Peripheral stone distributionPeripheral stone distribution

Biliary stricture is the major Biliary stricture is the major determinant for recurrencedeterminant for recurrence

PTCLPTCL

SeriesSeries NN MortalityMortality MorbidityMorbidity Stone Stone clearanceclearance

RecurrenceRecurrence Mean FU Mean FU (mths)(mths)

Huang

2003

245 0.8 1.6% 85.3% 63.2% 209

Cheung

2003

79 0 7.6% 76.8% 30% 37.3

Chen

2005

74 0 3% 82% 59% 121

J Am Coll Surg 1999

MorbidityMorbidity MortalityMortality Stone Stone clearanceclearance

5 yr 5 yr recurrencerecurrence

10 yr 10 yr recurrencerecurrence

HepatecHepatec

-tomy(26)-tomy(26)

38.5%38.5% 3.8%3.8% 96.2%96.2% 5.6%5.6% 16.0%16.0%

54 54 patientspatients

PTCL PTCL (28)(28)

21.4%21.4% 3.6%3.6% 96.4%96.4% 31.5%31.5% 54.3%54.3%

Our Experience on Our Experience on Hepatectomy for RPCHepatectomy for RPC

SeriesSeries NN MortalityMortality MorbidityMorbidity Stone Stone

ClearanceClearance

CholangioCholangio-CA-CA

FU FU (mth(mth

))

RecurRecur

rencerence

PWH 66 0 36.4% 93.9% 6.1% 42.7 12.9%Chen 2004

103

2% 28% 98% 10% 56 7.8%

Cheung 2005

52 3.8% 33.3% 98% 3.8% 58 13.3% (5 yrs)

Uchiyama 2007

38 0% 23.7% 100% 7.9% 108 13.9% (5 yrs)

Lee2007

123

1.6% 33.3% 92.7% 2.4% 40.3 5.7%

ConclusionConclusion

RPC is not “dead” in Hong KongRPC is not “dead” in Hong Kong

Health care burden in HK for the recurrent Health care burden in HK for the recurrent nature of the diseasenature of the disease

Management should be of multidisciplinary Management should be of multidisciplinary approach and tailored to individual patientapproach and tailored to individual patient

Hepatectomy is safe and effectiveHepatectomy is safe and effective