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