NCPF is a condition characterized by portal fibrosis and involvement of small and medium portal veins, leading to portal hypertension and splenomegaly despite normal liver function and structure. It commonly affects individuals in the Indian subcontinent aged 25-35 years from low socioeconomic backgrounds. Diagnosis involves evidence of portal hypertension and varices with normal liver function tests and histology showing obliteration of small portal veins without cirrhosis or injury. Management focuses on treatment and prevention of variceal bleeding through endoscopic therapies and beta blockers, with an otherwise excellent prognosis.

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