RECURRENT PYOGENIC

CHOLANGITIS

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

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

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

Clonorchis and opisthorchis

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

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

• 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

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

Pathogenesis Pyogenic cholangit

is

Pigment stone

formation

Ductal obstructi

on Distention

Bile stone

formation

Ductal injury

Parasite

infestation

Gastroenteriti

sMalnutrition

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

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

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

Imaging studies• USG – first line investigation• Findings – • Dilated biliary tree, intrahepatic calculi(90%)• Also can detect intrahepatic abscess

• 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

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

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

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.

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

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

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

• 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

• 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

• 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

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.