The Hong Kong Disease – Management Updates JHSGR 17/5/2008 The Hong Kong Disease – Management Updates Dr. YF Yeung Department of Surgery Prince of Wales Hospital

The Hong Kong Disease SARS

The Hong Kong Disease Recurrent Pyogenic Cholangitis (RPC) Oriental cholangitis Oriental cholangiohepatitis Intrahepatic pigmented calculus disease

Recurrent Pyogenic Cholangitis Cook in 1954 Repeated primary biliary infection Pus-forming bacteria Multiple stones and strictures in the biliary tree Pathogenesis not well understood nowadays Calcium bilirubinate stones within extra- and intraheptic biliary ducts

Aetiology ? Oriental diet ? Poor environmental hygiene Low saturated fat: biliary stasis Low protein diet: increased formation of calcium bilirubinate stones ? Poor environmental hygiene Recurrent enteric infection and portal bacteraemia ? Clonorchis sinensis and Ascaris lumbricoids

Epidemiology Predominantly lower socio-economic class and rural areas Male = Female Peak age incidence: 3rd to 4th decades Overall incidence is decreasing in East Asia HK experience 1950-1952: 30 patients / year 1984-1989: 22.8 patients / year Lo et al. HKMJ 1997 Increasing incidence in the West due to Asian immigrants

Why intervention important? Complications of RPC Recurrent cholangitis Liver abscess Parenchyma atrophy Biliary cirrhosis Cholangiocarcinoma

Imaging Features ERCP Truncated tree sign Ductal ectasia Abrupt tapering Arrow head appearance

Imaging Features Percutaneous Transhepatic Cholangiography Severe stricture Dilated ducts Multiple filling defects

Imaging Features MRCP Dilated ducts Strictures Filling defects

Imaging Features CT Hepatolithiasis Parenchymal atrophy Obliterated portal vein

Management - Multidisciplinary Acute episode Control of biliary sepsis Drainage +/- extraction of stones ERCP PTC Definitive treatment Correction of anatomic abnormalities/ sources of chronic infections

Definitive Management Surgical ECBD Liver Resection Drainage Procedure Endoscopic Percutaneous Transhepatic Cholangioscopic Lithotripsy (PTCL) “mother-baby” endoscope system

Hepatectomy Indications Stones localized in unilateral lobe Bile duct stricture Atrophy of affected segments/ lobe Suspected cholangiocarcinoma Failed / recurrent disease after non-operative treatment

Hepatectomy Series n Mortality Morbidity Stone Clearance Cholangio-CA FU (mth) Recur-rence Chen 2004 103 2% 28% 98% 10% 56 7.8% Cheung 2005 52 3.8% 33.3% 58 13.3% (5 yrs) Uchiyama 2007 38 0% 23.7% 100% 7.9% 108 13.9% Lee 2007 123 1.6% 92.7% 2.4% 40.3 5.7%

Drainage Procedure Principle Indications Eliminate biliary stasis Newly formed stones can pass unimpeded into the bowel Indications Extrahepatic ductal stones Extrahepatic biliary stricture Grossly dilated common duct with problem of bile stasis

Drainage Procedure Choledochoduodenostomy (CD) Sump syndrome Ascending cholangitis High risk of stasis Hepaticojejunostomy (HJ) Hinder post-operative choledochoscopic removal of residual stones Hepaticocutaneous jejunostomy with a stoma for easy access Possible complications: fistula, infection, parastomal hernia, early stoma closure Sphincteroplasty Parilla P et al. BJS 1991 Rat P et al. Hepatogastroenterology 1993 Huang et al. Am J Gastroenterol 2003

Is Drainage Procedure a MUST after hepatectomy?

Intra-op bleeding OT time Residual stone Post-op cholangitis World J Gastroenterololgy 2006 Intra-op bleeding OT time Residual stone Post-op cholangitis Liver resection(76) 500ml 282min 18.4% 22% HJ ECBD (47) 300ml 226min 23.4% 27% 314 patients Liver resection (85) 189min 21.2% 8.2% T-tube ECBD (106) 150ml 166min 34% 35.7%

Concluded indications for HJ World J Gastroenterololgy 2006 Median FU 7.6 years (2-12) Concluded indications for HJ Hepatolithiasis complicated with extrahepatic ducts or its second branches stricture Hepatolithiasis with congenital bile duct dilatation in which the dilated bile duct should be resected Dysfunction of the papilla of Vater

Percutaneous Transhepatic Cholangioscopic Lithotripsy (PTCL) Indications Stones distributed in multiple segments Previous biliary surgery Poor surgical risk Refuse surgery

PTCL Causes of incomplete stone clearance Biliary stricture Bile duct angulation Muddy stones with sludge Peripheral stone distribution Biliary stricture is the major determinant for recurrence

PTCL Series N Mortality Morbidity Stone clearance Recurrence Mean FU (mths) Huang 2003 245 0.8 1.6% 85.3% 63.2% 209 Cheung 79 7.6% 76.8% 30% 37.3 Chen 2005 74 3% 82% 59% 121

J Am Coll Surg 1999 Morbidity Mortality Stone clearance 5 yr recurrence 10 yr recurrence Hepatec -tomy(26) 38.5% 3.8% 96.2% 5.6% 16.0% 54 patients PTCL (28) 21.4% 3.6% 96.4% 31.5% 54.3%

Our Experience on Hepatectomy for RPC Series N Mortality Morbidity Stone Clearance Cholangio-CA FU (mth) Recur rence PWH 66 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% 58 13.3% (5 yrs) Uchiyama 2007 38 0% 23.7% 100% 7.9% 108 13.9% Lee 2007 123 1.6% 92.7% 2.4% 40.3 5.7%

Conclusion RPC is not “dead” in Hong Kong Health care burden in HK for the recurrent nature of the disease Management should be of multidisciplinary approach and tailored to individual patient Hepatectomy is safe and effective