Non cirrhotic portal fibrosis

1. NEW CONSENSUS ON
NON-CIRRHOTIC PORTAL FIBROSIS
(NCPF)
GUIDE: DR.ATUL SHENDE
CANDIDATE:DR.SARATH MENON.R
DIVISION OF GASTROENTEROLOGY
MGM MEDICAL COLLEGE,INDORE

2. INTRODUCTION
 NON-CIRRHOTIC PORTAL HYPERTENSION
 NCPF
 CONCEPT & TERMINOLOGY
 NCPF vs EHPVO vs CIRRHOSIS
 CLINICAL PROFILE
 DIAGNOSIS
 MANAGEMENT
 PROGNOSIS

3. NON-CIRRHOTIC PORTAL HYPERTENSION
 Increase in portal pressure due to pre-sinusoidal (intra-
hepatic) or pre hepatic lesions
 Absence of cirrhosis
 Absence of hepatic venous outflow obstn.
 Vascular lesions
 WHVP(wedge hepatic venous pressure) is normal
 NCPF & EHPVO- 2 main causes

6. NCPF - DEFINITION
 Disease of uncertain etiology
 Portal fibrosis & invlv. small and med.portal veins
 Portal hypertension,splenomegaly,variceal bleed.
 Liver functions & stucture- normal

7. TERMINOLOGY
 Non –cirrhotic portal fibrosis by ICMR in 1969
 Idiopathic portal hypertension in Japan
 Hepato portal sclerosis in West

8. NCPF
 Indian subcontinent
 Low socio-economic status
 Age gp- 25-35 yrs
 No sex prediliction

9. ETIOLOGY
 Infections – bacterial inf. From gut.
- umblical sepsis,diarrhoea in
infancy & early childhood.
 chronic arsenicosis
 Auto- immune disorders
 Vinyl chloride
 Pro-thrombotic state (west)
 Exact etiology is still unknown

10. infections/other agents
chronic/ mild in Later age
c/c antigenenemia/endotoxemia
phlebosclerosis
pre-sinusoidal fibrosis
pre-sinusoidal resistance
PORTAL HYPERTENSION

11. CLINICAL PROFILE
 Age – 2nd and 3rd decades
 M=F
 Hemetemesis & malaena (well-tolerated)
 Feeling of lump
 Esophagial varices
 Gastric varices
 Portal gastropathy
 Transient ascites

12. NATURAL HISTORY
 Bleeding rate from varices high
 Mortality is low due to preserved liver functions.
 Transient ascites after bleed

13. HISTOPATHOLOGY
 Liver size & structure normal
 Obliterative portovenopathy
-patchy & segmental subendothelial
thickening of med & small portal vein
- obliteration of small portal veins & emerg.
new abberant portal channels

14. INVESTIGATIONS
 LFT- normal or near normal
 Pancytopenia due to hypersplenism
 Bone marrow –hypercellular
 Coagulation profile and PLC- mild derranged
 Needle biopsy-
- absence of regenerative nodules
- small portal vein obliteration
- portal tract fibrosis
- perivenular fibrosis
- lack of hepatocellular injury

15. IMAGING
 Usg- porto splenic axis dilated & patent
- occ.thrombus in intrahepatic branch
- echogenic boundary of PV (wall thickness)

17. ENDOSCOPY
 Esophagial varices – 80-95%
 Varices are large at time of diagnosis
 Gastric varices
 Portal hypertensive gastropathy- rare
 Anorectal varices common

18. HEMODYNAMICS
 Wedge hepatic venous pressure is normal
(WHVP)
 Hepatic venous pressure gradient is normal
( WHFP- FHVP)

20. DIAGNOSTIC FEATURES
 Presence of mod- massive splenomegaly
 Evidence of portal hypertension,varices and /or
collaterals
 Patent speno-portal axis & hepatic veins on
ultrasound color doppler
 Normal or near normal liver functions
 Wedge hepatic venous pressure gradient- normal
 Liver histology- no cirrhosis & parenchymal
injury

21. OTHER FEATURES
 Absence of signs of CLD
 No decompensation except transient ascites
 Absence of serum markers of hep B &C
 No known etiology of liver disease
 USG – DILATED & THICKENED portal vein
with peripheral pruning & hyperechoic
areas.

22. DIFFERENTIAL DIAGNOSIS
 EHPVO
 Idiopathic portal hypertension( Japan)
 Incomplete septal cirrhosis
 Childs A compensated cirrhosis

23. parameter EHPVO NCPF Cirrhosis
Median age 10 yr 28 yr 40 yr
Ascites Absent/transientaft
er bleed
Absent/transient
after bleed
+ to +++
Encephalopathy nil nil ++
Jaundice/signs of
liver failure
nil nil ++
Liver function test normal normal deranged
Liver –Gross normal normal Shrunken,nodular
microscopic normal Normal/portal
fibrosis
Necrosis,regenerat
ion
Usg Portal/splenic vein
block &
cavernoma
dilated &
patent&thickened
Spleno-portal axis
Dilated & patent
Spleno-portal axis

24. DIFFERENTIALS
 Incomplete septal cirrhosis
 Compensated cirrhosis
diagnosed - LIVER BIOPSY

25. NCPF VS IPH
NCPF IPH
Age (years) 25-35 43-56
M: F 1:1 1:3
Hemetemesis/ malena 94 % 40%
Spenomegaly Dispropationate &
massive
moderate
Autoimmune features rare common
Wedge hepatic venous
pressure
normal Mildly raised
Geography Indian subcontinent Japan

26. COMPLICATIONS
 Varices
 Portal biliopathy
 Portal colopathy
 Portal gastropathy

28. PORTAL BILIOPATHY
 Term introduced in 1992.
 Abnormalities of extra & intra hepatic bile ducts
with portal hypertension
- identation by paracholedochal collaterals
- localized strictures,angulation of duct
- displc. Duct,focal narrowing,dilations
 left hepatic duct (mc)
 Symptoms- abd.pain,jaundice,fever
 complication- cholangitis,choledocholithiasis

29. PORTAL HYPERTENSIVE GASTROPATHY
 Rare in NCPF
 Gastric mucosal & sub mucosal vascular ectasia
 Potential for acute & c/c bleeding
 endoscopy- mosaic or snake skin pattern mucosa

31. PORTAL COLOPATHY
 Enlarged hemorrhoids
 Rectal varices
 endoscopy- diffuse vascular ectasia

33. MANAGEMENT OF ACUTE BLEEDING
 General management (icu ) - I v fluids, NGT,
- blood transfusions
 Pharmocological therapy-
- octreotide,vasopressin
- efficacy in NCPF is not known
 Endoscopic therapy-
sclerotherapy & band ligation
80- 90% efficacy
band ligation (preffered)
 Combination therapy- more effective in acute bleed
- prevent rebleed

35. SCREENING
 All patients with moderative- massive
splenomegaly with NCPF should have a
screening endoscopy

36. PRIMARY PROPHYLAXIS
 Beta blockers
 Endoscopic therapy
 Combination of both- more effective
 Shunt sx – if large esophageal varices with
symptomatic splenomegaly,
thrombocytopenia <20,000,
repeated splenic infarcts
 Gastric varices-
- cyanoacrylate glue injection

37. SECONDARY PROPYLAXIS (RE-BLEEDING)
 Endoscopic therapy
 Shunt surgery

38. MANAGEMENT OF SPECIAL SITUATIONS
 Hypersplenism- splenectomy in symptomatic
done with shunt sx.
 Portal biliopathy –
cholangitis & choledocholithiasis-
- biliary stenting,sphincterectomy,
stone extraction.

39. PROGNOSIS
 Excellent
 Mortality from acute bleed is lower
 After successful eradication of esophagicgastro
varices- 2- 5 yr survival is 100%

40. CONCLUSION
 Common cause of PHT in indian subcontinent
 Socially disadvantaged people
 Multifactorial etiogenesis
 Splenomegaly with complications of PTH &
well preserved liver function
 Diagnosis- clinical,imaging,histology
 Proper management,life expectancy is normal
 Since 1990, there is decline in occurence