66374645 New Consensus on Non Cirrhotic Portal Fibrosis

7/31/2019 66374645 New Consensus on Non Cirrhotic Portal Fibrosis

1/41

NEW CONSENSUS ON NON -CIRRHOTIC PORTAL FIBROSIS

(NCPF )GUIDE: DR.ATUL SHENDECANDIDATE:DR.SARATH MENON.RDIVISION OF GASTROENTEROLOGY

MGM MEDICAL COLLEGE,INDORE


2/41

INTRODUCTIONNON-CIRRHOTIC PORTAL HYPERTENSION

NCPF

CONCEPT & TERMINOLOGY

NCPF vs EHPVO vs CIRRHOSIS

CLINICAL PROFILE

DIAGNOSIS

MANAGEMENT

PROGNOSIS


3/41

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


4/41


5/41


6/41

NCPF - DEFINITION

Disease of uncertain etiologyPortal fibrosis & invlv. small and med.portal veinsPortal hypertension,splenomegaly,variceal bleed.

Liver functions & stucture- normal


7/41

TERMINOLOGY

Non cirrhotic portal fibrosis by ICMR in 1969

Idiopathic portal hypertension in Japan

Hepato portal sclerosis in West


8/41

NCPF

Indian subcontinent

Low socio-economic status

Age gp- 25-35 yrs

No sex prediliction


9/41

ETIOLOGY

Infections bacterial inf. From gut.- umblical sepsis,diarrhoea in

infancy & early childhood.

chronic arsenicosisAuto- immune disordersVinyl chloridePro-thrombotic state (west)

Exact etiology is still unknown


10/41

infections/other agents

chronic/ mild in Later age

c/c antigenenemia/endotoxemia

phlebosclerosis

pre-sinusoidal fibrosis

pre-sinusoidal resistance

PORTAL HYPERTENSION


11/41

CLINICAL PROFILE

Age 2nd and 3 rd decadesM=FHemetemesis & malaena (well-tolerated)

Feeling of lumpEsophagial varicesGastric varicesPortal gastropathyTransient ascites


12/41


13/41

HISTOPATHOLOGY

Liver size & structure normalObliterative portovenopathy

-patchy & segmental subendothelial

thickening of med & small portal vein- obliteration of small portal veins & emerg.

new abberant portal channels


14/41

INVESTIGATIONS

LFT- normal or near normalPancytopenia due to hypersplenismBone marrow hypercellular

Coagulation profile and PLC- mild derrangedNeedle biopsy-

- absence of regenerative nodules- small portal vein obliteration

- portal tract fibrosis- perivenular fibrosis- lack of hepatocellular injury


15/41

IMAGING

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


16/41


17/41

ENDOSCOPY

Esophagial varices 80-95%Varices are large at time of diagnosisGastric varices

Portal hypertensive gastropathy- rareAnorectal varices common


18/41

HEMODYNAMICS

Wedge hepatic venous pressure is normal(WHVP)

Hepatic venous pressure gradient is normal( WHFP- FHVP)


19/41


20/41

DIAGNOSTIC FEATURES

Presence of mod- massive splenomegalyEvidence of portal hypertension,varices and /orcollaterals

Patent speno-portal axis & hepatic veins onultrasound color dopplerNormal or near normal liver functionsWedge hepatic venous pressure gradient- normal

Liver histology- no cirrhosis & parenchymalinjury


21/41

OTHER FEATURES

Absence of signs of CLDNo decompensation except transient ascitesAbsence of serum markers of hep B &C

No known etiology of liver diseaseUSG DILATED & THICKENED portal vein

with peripheral pruning & hyperechoicareas.


22/41

DIFFERENTIAL DIAGNOSIS

EHPVO

Idiopathic portal hypertension( Japan)

Incomplete septal cirrhosis

Childs A compensated cirrhosis

t EHPVO NCPF Ci h i


23/41

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 ofliver failure

nil nil ++

Liver function test normal normal deranged

Liver Gross normal normal Shrunken,nodular

microscopic normal Normal/portalfibrosis

Necrosis,regeneration

Usg Portal/splenic veinblock &

cavernoma

dilated &patent&thickened

Spleno-portal axis

Dilated & patentSpleno-portal axis


24/41

DIFFERENTIALS

Incomplete septal cirrhosisCompensated cirrhosis

diagnosed - LIVER BIOPSY


25/41

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 venouspressure

normal Mildly raised

Geography Indian subcontinent Japan


26/41

COMPLICATIONS

Varices

Portal biliopathy

Portal colopathy

Portal gastropathy


27/41


28/41

P ORTAL BILIOPATHY

Term introduced in 1992.Abnormalities of extra & intra hepatic bile ductswith portal hypertension

- identation by paracholedochal collaterals- localized strictures,angulation of duct- displc. Duct,focal narrowing,dilationsleft hepatic duct (mc)

Symptoms- abd.pain,jaundice,fevercomplication- cholangitis,choledocholithiasis


29/41

P ORTAL HYPERTENSIVE GASTROPATHY

Rare in NCPFGastric mucosal & sub mucosal vascular ectasiaPotential for acute & c/c bleeding

endoscopy- mosaic or snake skin pattern mucosa


30/41


31/41

P ORTAL COLOPATHY

Enlarged hemorrhoids

Rectal varices

endoscopy- diffuse vascular ectasia


32/41


33/41

MANAGEMENT OF ACUTE BLEEDING General management (icu ) - I v fluids, NGT,

- blood transfusionsPharmocological therapy-

- octreotide,vasopressin- efficacy in NCPF is not known

Endoscopic therapy-sclerotherapy & band ligation80- 90% efficacyband ligation (preffered)

Combination therapy- more effective in acute bleed- prevent rebleed


34/41


35/41

SCREENING

All patients with moderative- massivesplenomegaly with NCPF should have ascreening endoscopy


36/41

P RIMARY PROPHYLAXIS

Beta blockersEndoscopic therapyCombination of both- more effective

Shunt sx

if large esophageal varices withsymptomatic splenomegaly,thrombocytopenia


37/41


38/41

MANAGEMENT OF SPECIAL SITUATIONS

Hypersplenism- splenectomy in symptomaticdone with shunt sx.

Portal biliopathy

cholangitis & choledocholithiasis-

- biliary stenting,sphincterectomy,stone extraction.


39/41

PROGNOSIS

ExcellentMortality from acute bleed is lowerAfter successful eradication of esophagicgastro

varices- 2- 5 yr survival is 100%


40/41

CONCLUSION

Common cause of PHT in indian subcontinentSocially disadvantaged peopleMultifactorial etiogenesis

Splenomegaly with complications of PTH &well preserved liver function

Diagnosis- clinical,imaging,histologyProper management,life expectancy is normal

Since 1990, there is decline in occurence


41/41

Documents

66374645 New Consensus on Non Cirrhotic Portal Fibrosis