new consensus on ncpf
DESCRIPTION
Non cirrhotic portal fibrosisTRANSCRIPT
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