New consensus on ncpf

Non cirrhotic portal fibrosis

1. New consensus on non-cirrhotic portal fibrosis ( ncpf ) GUIDE: DR.ATUL SHENDE CANDIDATE:DR.SARATH MENON.R DIVISION OF GASTROENTEROLOGY MGM MEDICAL COLLEGE,INDORE

2. INTRODUCTION NON-CIRRHOTIC PORTAL HYPERTENSION NCPF CONCEPT & TERMINOLOGY NCPF vs EHPVO vs CIRRHOSIS CLINICAL PROFILE DIAGNOSIS MANAGEMENT PROGNOSIS

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

5. terminology Non –cirrhotic portal fibrosis by ICMR in 1969 Idiopathic portal hypertension in Japan Hepato portal sclerosis in West

6. Ncpf Indian subcontinent Low socio-economic status Age gp - 25-35 yrs No sex prediliction

7. 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

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

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

10. Natural history Bleeding rate from varices high Mortality is low due to preserved liver functions. Transient ascites after bleed

11. 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

12. 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

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

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

15. hemodynamics Wedge hepatic venous pressure is normal (WHVP) Hepatic venous pressure gradient is normal ( WHFP- FHVP)

16. 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

17. 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.

18. Differential diagnosis EHPVO Idiopathic portal hypertension( Japan ) Incomplete septal cirrhosis Childs A compensated cirrhosis

19. 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&thickened Spleno -portal axis Dilated & patent Spleno -portal axis

20. differentials Incomplete septal cirrhosis Compensated cirrhosis diagnosed - LIVER BIOPSY

21. 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

22. complications Varices Portal biliopathy Portal colopathy Portal gastropathy

23. 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

24. 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

25. Portal colopathy Enlarged hemorrhoids Rectal varices endoscopy- diffuse vascular ectasia

26. 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

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

28. 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

29. Secondary propylaxis (re-bleeding) Endoscopic therapy Shunt surgery

30. Management of special situations Hypersplenism - splenectomy in symptomatic done with shunt sx . Portal biliopathy – cholangitis & choledocholithiasis - - biliary stenting,sphincterectomy , stone extraction.

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

32. 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