recurrent pyogenic cholangitis
TRANSCRIPT
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RECURRENT PYOGENIC
CHOLANGITIS
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Definition• First described by Digbi in 1930 in in patients from Hong Kong
• Defined by – Cook as triad of • Recurrent bacterial cholangitis• Intrahepatic pigmented stones• Biliary strictures
• Alias – • Oriental cholangio hepatitis• Hong Kong Disease• Biliary obstruction syndrome of Chinese• Hepatolithiasis
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Epidemiology• Exclusively seen in Southeast Asia
• Equal frequency in male and female
• Commonly 3rd and 4th decade
• More common in rural than in urban population
• Not many studies from India – Khuroo – 5/1104 who underwent USG for biliary disease had RPC
Gut 1989
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Etiology• Exact etiology not known
• Clusters of RPC are seen in areas where biliary parasitosis is common i.e. flukes and round worm
• Three main treamtodes• Clonorchis sinesis• Opisthorchis viverrini• Fasciola hepatica
• These infestations – not sine qua non, seen in 20-45% of RPCsHuang M H, J Gastro Hepato, 2005
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Clonorchis and opisthorchis
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Ascariasis• Nearly 1/4 of the world has round worm infection
• Indian data ascariasis is the commonest cause of RPC
• Study by Khuroo, 30 pts of RPC were studied, 22 had evidence of ascariasis
IJHBPD 2015• Stones were studied using infra-Red spectrophotometry
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Etiology• Bacterial agents - Transient portal bacteremia introduces
bacteria in the biliary tract – common organism – Ecoli, Klebsiella, Pseudomonas, Proteus and rarely anerobes
Oriental cholangitis, Carmona, Am J Surg 1984
• Source of bacteria could be lower intestine or due to biliary injury caused by parasites
• But bacteria as a cause or result is uncertain
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• Bacterial infection lead to formation of pigment stones
• Bacterial glucoronidases – unconjugates the bilirubin from glucoronides making it insoluble in the bile combines with calcium and precipitate as stone, leads to cycle
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Etiology• Host factors –• Dietary factors – diet low in fats and protein
• Low fat reduces level of cholecystokinin in the diet reducing GB contraction – bile stasis and stone formation
• Low protein diet reduces inhibitors of bacterial glucoronidases inhibitor levels in bile
• Sphincter oddi dysfunction is seen in atleast ½ of those RPC, may be a/w papillitis – cause or effect relation not explored
Khuroo, Hepatology, 1993
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Pathogenesis Pyogenic cholangit
is
Pigment stone
formation
Ductal obstructi
on Distention
Bile stone
formation
Ductal injury
Parasite
infestation
Gastroenteriti
sMalnutrition
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Pathogenesis• De novo stone formation occurs in the intrahepatic bile ducts
as contrast to common gallstones formed in GB
• Left hepatic duct is the commonest site of stone formation – especially the left lateral segmental duct
Cosenza, Am J Sur 1999• LHD – possible – more acute angle as compared to RHD
stasis and stricture predisposed
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Pathology• Classic finding intra and extrahepatic strictures
• Inflammatory infiltrate with periductal fibrosis and abscess frequently seen in wall of involved bile ducts
• Scarring of liver with multiple adhesions or deep subcapsular abscess
• Obstruction of CBD/CHD may lead to secondary biliary cirrhosis
• Atrophy of affected lobe, nidus for cholangiocarcinoma
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Clinical presentation• Typical presentation recurrent cholangitis
• Charcot’s triad seen in 44%• Pain without cholangitis 32%• Pancreatitis 17%
Sperling RM, Dig Dis Sci 1997
• Prior history of cholangitis seen in majority but 30% may be diagnosed on first presentation
• Hepatomegaly – 20%, GB palpable in 10%
• Lab studies compatible with biliary obstruction
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Imaging studies• USG – first line investigation• Findings – • Dilated biliary tree, intrahepatic calculi(90%)• Also can detect intrahepatic abscess
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• CT – Better than USG
• Non contrast film better for stones
• Contrast subtle duct dilatation
• Findings – IHBR, calculi, pneumobilia(due to reflux from ampulla, gas forming agents – Klebsiella/clostridium) , cholangitis, biliomas or abscess and cholangiocarcinoma
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MRCP• Better for non calcified calculi
• Short duct stricture(<1cm) are better visualised than CT
• MRCP shows • 100 % of surgically proven dilatations• 96% focalstrictures• 98% calculi• Vs direct cholangiography(44-47%
Park , Radiology 2001
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Imaging in complications• Abscess – seen in 20%, most common in right lobe, rim
enhancement on CT differentiates from bilioma
• Bilioma – Intrahepatic bile lakes, +/- communication with biliary tree, hypodense on CT
• Portal vein thrombosis- as complication of cirrhois/ d/t adjacent periportal inflammation
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Other investigations• Acute cholangitis – CBC, RT and LFT, cultures,
• Clonorchis and opisthorcis evaluation by stool for eggs
• Eggs present only after 4 weeks of infection
• Duodenal or biliary fluids also may show eggs intact worms
• Peripheral eosinophilia and raised IgE levels.
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Management • Acute complications
• Initiate fluids and antibiotics after cultures – blood and bile
• ERCP if drainage is planned or required• Cholangiography – arrowhead sign• Missing duct sign• Decreased arborizing pattern• Stenting followed by ductal clearance
• Surgery if ERCP fails – CBD exploration, T- tube drainage and cholecystectomy
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Prevention of long of complication• Optimal approaches ???, Combination usually followed
• Stone clearance – • ERCP• Percutaneous• Surgical – choledocholithotomy • Choledochoscope – via T-tube, hepaticojejunostomy, transpapillary• Overall success rate of stone removal with these techniques – 88%
Cheng WJ Surg 2000, Gott Am Surg PE 1996• Despite successful clearance recurrence seen in 30% more with
intrahepatic strictures• Resultant – surveillance 3-6 mon USG
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Long term recurrence• UDCA benefit in recurrence not known, despite data many
people use since it increase bile flow, ↓ viscosity ↓ risk of stone formation
• Hepatic resection – segment which are main source of complications – if localized may be resected
• Lesser rates of secondary biliary cirrhosis, cholangioca, mortality and better quality of life
Vetrone, J Am Coll Surg 2006
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• Bilio-enteric anastomosis – controversial
• Choledocho-duodenostomy, choledocho-jejunostomy, spinchteroplasty – usually C/I since adequate drainage may not be achieved.
• The rate cholangitis was higher in patients with H-J(30.6%) compared with hepatectomies(3.4%) alone
Kusano, Am Surg 2001
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• Combined approach – • 136 pts, lithotripsy during intraop choledochoscopy, • 54 – bilateral hepatectomy, unilateral in other substet• Stone clearance 82% b/l and 66% in u/l• Hospital mortality 5.6% and 0% • Complications rate same 46%• Supports unilateral hepatectomy is feasible approach in pts. with b/l
hepatolithiasisYang T Ann Surg
2010
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• Treatment with anti helminithic drugs
• Praziquantel 75mg/kg in 3 divided doses for 1 day
• Universally effective for opisthorchis and Clonorchis
• S/e headache and vomiting
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Prognosis and complications• Korean series cumulative recurrence rates of cholangitis• 25% at 3 years• 37% at 5 years• Overall – 45% over mean study period 56 month
• Recurrent stones more likely a/w cholangitis than residual stones
Hwang J Clin Gastro - 2004• Choalngiocarcinoma risk 3-9%
Kubo WJ surg 1996• Secondary biliary cirrhosis may require liver transplant.
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