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
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
4.
5.
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
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)
16.
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)
19.
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.
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
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
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
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
34.
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
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