recurrent pyogenic cholangitis

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.