new consensus on ncpf
DESCRIPTION
Non cirrhotic portal fibrosisTRANSCRIPT
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NEW CONSENSUS ON NON-CIRRHOTIC PORTAL FIBROSIS (NCPF)GUIDE: DR.ATUL SHENDE
CANDIDATE:DR.SARATH MENON.R
DIVISION OF GASTROENTEROLOGY
MGM MEDICAL COLLEGE,INDORE
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INTRODUCTION NON-CIRRHOTIC PORTAL HYPERTENSION
NCPF
CONCEPT & TERMINOLOGY
NCPF vs EHPVO vs CIRRHOSIS
CLINICAL PROFILE
DIAGNOSIS
MANAGEMENT
PROGNOSIS
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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
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NCPF - DEFINITION
Disease of uncertain etiology Portal fibrosis & invlv. small and med.portal
veins Portal hypertension,splenomegaly,variceal
bleed. Liver functions & stucture- normal
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TERMINOLOGY
Non –cirrhotic portal fibrosis by ICMR in 1969
Idiopathic portal hypertension in Japan
Hepato portal sclerosis in West
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NCPF
Indian subcontinent
Low socio-economic status
Age gp- 25-35 yrs
No sex prediliction
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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
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infections/other agents
chronic/ mild in Later age
c/c antigenenemia/endotoxemia
phlebosclerosis
pre-sinusoidal fibrosis
pre-sinusoidal resistance
PORTAL HYPERTENSION
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CLINICAL PROFILE
Age – 2nd and 3rd decades M=F Hemetemesis & malaena (well-tolerated) Feeling of lump Esophagial varices Gastric varices Portal gastropathy Transient ascites
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NATURAL HISTORY
Bleeding rate from varices high Mortality is low due to preserved liver
functions. Transient ascites after bleed
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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
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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
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IMAGING
Usg- porto splenic axis dilated & patent - occ.thrombus in intrahepatic branch - echogenic boundary of PV (wall
thickness)
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ENDOSCOPY
Esophagial varices – 80-95% Varices are large at time of diagnosis Gastric varices Portal hypertensive gastropathy- rare Anorectal varices common
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HEMODYNAMICS
Wedge hepatic venous pressure is normal (WHVP)
Hepatic venous pressure gradient is normal ( WHFP- FHVP)
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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
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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.
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DIFFERENTIAL DIAGNOSIS
EHPVO
Idiopathic portal hypertension( Japan)
Incomplete septal cirrhosis
Childs A compensated cirrhosis
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parameter EHPVO NCPF Cirrhosis
Median age 10 yr 28 yr 40 yr
Ascites Absent/transientafter 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,regeneration
Usg Portal/splenic vein block & cavernoma
dilated & patent&thickenedSpleno-portal axis
Dilated & patentSpleno-portal axis
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DIFFERENTIALS
Incomplete septal cirrhosis Compensated cirrhosis diagnosed - LIVER BIOPSY
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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
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COMPLICATIONS
Varices
Portal biliopathy
Portal colopathy
Portal gastropathy
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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
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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
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PORTAL COLOPATHY
Enlarged hemorrhoids
Rectal varices
endoscopy- diffuse vascular ectasia
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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
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SCREENING
All patients with moderative- massive splenomegaly with NCPF should have a screening endoscopy
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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
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SECONDARY PROPYLAXIS (RE-BLEEDING)
Endoscopic therapy
Shunt surgery
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MANAGEMENT OF SPECIAL SITUATIONS
Hypersplenism- splenectomy in symptomatic done with shunt sx.
Portal biliopathy – cholangitis & choledocholithiasis- - biliary
stenting,sphincterectomy, stone extraction.
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PROGNOSIS
Excellent Mortality from acute bleed is lower After successful eradication of
esophagicgastro varices- 2- 5 yr survival is 100%
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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
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