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    New Consensus On Non Cirrhotic Portal Fibrosis

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    ramesh5889
    This document discusses non-cirrhotic portal fibrosis (NCPF), a condition seen predominantly in the Indian subcontinent characterized by portal hypertension and splenomegaly without cirrhosis. It defines NCPF and differentiates it from other causes of portal hypertension. The document covers the clinical presentation, diagnostic criteria, management of complications like variceal bleeding, and prognosis of patients with NCPF. It emphasizes that NCPF has a multifactorial etiology and can be successfully managed with a good long-term survival if complications are addressed properly.

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    New Consensus On Non Cirrhotic Portal Fibrosis

    Uploaded by

    ramesh5889
    186 views41 pages
    This document discusses non-cirrhotic portal fibrosis (NCPF), a condition seen predominantly in the Indian subcontinent characterized by portal hypertension and splenomegaly without cirrhosis. It defines NCPF and differentiates it from other causes of portal hypertension. The document covers the clinical presentation, diagnostic criteria, management of complications like variceal bleeding, and prognosis of patients with NCPF. It emphasizes that NCPF has a multifactorial etiology and can be successfully managed with a good long-term survival if complications are addressed properly.

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    66374645 New Consensus on Non Cirrhotic Portal Fibrosis

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    This document discusses non-cirrhotic portal fibrosis (NCPF), a condition seen predominantly in the Indian subcontinent characterized by portal hypertension and splenomegaly without cirrhosis. It defines NCPF and differentiates it from other causes of portal hypertension. The document covers the clinical presentation, diagnostic criteria, management of complications like variceal bleeding, and prognosis of patients with NCPF. It emphasizes that NCPF has a multifactorial etiology and can be successfully managed with a good long-term survival if complications are addressed properly.

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    186 views41 pages

    New Consensus On Non Cirrhotic Portal Fibrosis

    Uploaded by

    ramesh5889
    This document discusses non-cirrhotic portal fibrosis (NCPF), a condition seen predominantly in the Indian subcontinent characterized by portal hypertension and splenomegaly without cirrhosis. It defines NCPF and differentiates it from other causes of portal hypertension. The document covers the clinical presentation, diagnostic criteria, management of complications like variceal bleeding, and prognosis of patients with NCPF. It emphasizes that NCPF has a multifactorial etiology and can be successfully managed with a good long-term survival if complications are addressed properly.

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    of 41

    NEW CONSENSUS ON NON-CIRRHOTIC PORTAL FIBROSIS (NCPF)

    GUIDE: DR.ATUL SHENDE CANDIDATE:DR.SARATH MENON.R DIVISION OF GASTROENTEROLOGY MGM MEDICAL COLLEGE,INDORE

    INTRODUCTION

    NON-CIRRHOTIC PORTAL HYPERTENSION NCPF CONCEPT & TERMINOLOGY

    NCPF vs EHPVO vs CIRRHOSIS


    CLINICAL PROFILE DIAGNOSIS MANAGEMENT PROGNOSIS

    NON-CIRRHOTIC PORTAL HYPERTENSION

    Increase in portal pressure due to pre-sinusoidal (intrahepatic) 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

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

    TERMINOLOGY

    Non cirrhotic portal fibrosis by ICMR in 1969 Idiopathic portal hypertension in Japan

    Hepato portal sclerosis in West

    NCPF

    Indian subcontinent Low socio-economic status

    Age gp- 25-35 yrs


    No sex prediliction

    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

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

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

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

    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

    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

    IMAGING

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

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

    HEMODYNAMICS

    Wedge hepatic venous pressure is normal (WHVP)

    Hepatic venous pressure gradient is normal ( WHFP- FHVP)

    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

    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.

    DIFFERENTIAL DIAGNOSIS

    EHPVO Idiopathic portal hypertension( Japan)

    Incomplete septal cirrhosis


    Childs A compensated cirrhosis

    parameter Median age

    EHPVO 10 yr

    NCPF 28 yr

    Cirrhosis 40 yr

    Ascites

    Absent/transientaft Absent/transient er bleed after bleed


    nil nil nil nil

    + to +++

    Encephalopathy Jaundice/signs of liver failure

    ++ ++

    Liver function test


    Liver Gross

    normal

    normal

    deranged

    normal

    normal

    Shrunken,nodular

    microscopic

    normal

    Normal/portal fibrosis dilated & patent&thickened Spleno-portal axis

    Necrosis,regenerat ion Dilated & patent Spleno-portal axis

    Usg

    Portal/splenic vein block & cavernoma

    DIFFERENTIALS Incomplete septal cirrhosis Compensated cirrhosis diagnosed - LIVER BIOPSY

    NCPF VS IPH
    NCPF Age (years) M: F Hemetemesis/ malena 25-35 1:1 94 % IPH 43-56 1:3 40%

    Spenomegaly Autoimmune features


    Wedge hepatic venous pressure Geography

    Dispropationate & massive rare


    normal Indian subcontinent

    moderate common
    Mildly raised Japan

    COMPLICATIONS

    Varices Portal biliopathy

    Portal colopathy
    Portal gastropathy

    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

    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

    PORTAL COLOPATHY

    Enlarged hemorrhoids Rectal varices

    endoscopy- diffuse vascular ectasia

    MANAGEMENT OF ACUTE BLEEDING


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

    SCREENING

    All patients with moderative- massive splenomegaly with NCPF should have a screening endoscopy

    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

    SECONDARY PROPYLAXIS (RE-BLEEDING)

    Endoscopic therapy Shunt surgery

    MANAGEMENT OF SPECIAL SITUATIONS

    Hypersplenism- splenectomy in symptomatic done with shunt sx. Portal biliopathy cholangitis & choledocholithiasis- biliary stenting,sphincterectomy, stone extraction.

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

    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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