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 (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

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 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

DIFFERENTIALS

Incomplete septal cirrhosis Compensated cirrhosis diagnosed - LIVER BIOPSY

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

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 therapy- sclerotherapy & 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