This document discusses recurrent pyogenic cholangitis (RPC), a condition characterized by recurrent bacterial cholangitis, intrahepatic pigmented stones, and biliary strictures. It is seen predominantly in Southeast Asia. Parasitic infections and bacterial infections both contribute to the formation of stones within the bile ducts. Patients typically present with recurrent episodes of cholangitis. Imaging studies can identify stones and bile duct abnormalities. Treatment involves stone removal, antibiotics, and sometimes surgical interventions like duct clearance or liver resection to prevent long-term complications like cirrhosis or cancer. Recurrence rates remain high even after treatment.

1. RECURRENT
PYOGENIC
CHOLANGITIS

2. 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

3. 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

4. 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 RPCs
Huang M H, J Gastro Hepato, 2005

5. Clonorchis and opisthorchis

8. 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

9. 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

10. • 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

11. 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

12. Pathogenesis
Pyogenic
cholangitis
Pigment
stone
formation
Ductal
obstruction
Distention
Bile stone
formation
Ductal injuryParasite infestation Gastroenteritis Malnutrition

13. 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

14. 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

15. 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

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

17. • 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

18. 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

19. 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

20. 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.

21. 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

23. 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

24. 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

25. • 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

26. • 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
hepatolithiasis
Yang T Ann Surg 2010

27. • 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

28. 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.