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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
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JHSGR 17/5/2008 The Hong Kong Disease –Management Updates Dr. YF Yeung Department of Surgery Prince of Wales Hospital
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 • 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
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
Surgical ECBD Liver Resection Drainage Procedure Endoscopic Percutaneous Transhepatic Cholangioscopic Lithotripsy (PTCL) “mother-baby” endoscope system Definitive Management
Hepatectomy • Indications • Stones localized in unilateral lobe • Bile duct stricture • Atrophy of affected segments/ lobe • Suspected cholangiocarcinoma • Failed / recurrent disease after non-operative treatment
Drainage Procedure • Principle • 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
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 World J Gastroenterololgy 2006
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
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