Non cirrhotic portal fibrosis

Common among Young men(25 - 40 year old), Differential Diagnosis: EHPVO, Cirrhosis

1. Non Cirrhotic Portal Fibrosis Dr. Pratik Edwards

2. Anatomy and Physiology The portal vein is formed by the union of the superior mesenteric vein and the splenic vein just posterior to the head of the pancreas at about the level of the second lumbar vertebra E nters the liver at the porta hepatis in two main branches, one to each lobe; it is without valves in its larger channels Portal blood flow is about 1000–1200ml/min C ontributes 40ml/min or 72% of the total oxygen supply to the liver Normal Portal pressure is about 7 – 10 mmHg Normal portal vein diameter is 1 cm

3. Non-cirrhotic portal fibrosis S yndrome of obscure etiology ‘ O bliterative portovenopathy ’ leading to PHT, M assive splenomegaly well-tolerated episodes of variceal bleeding In young adults, L ow socioeconomic backgrounds N ormal hepatic functions

4. Lesion in NCPF It is generally vascular . Location of lesion: 1 . Branches of Portal vein 2. Perisinusoidal area of Liver

5. Epidemlology Reported worlwide , but maximum cases from india Male predominance of 2:1 to 4:1 Age : Mainly a disease of young men (25 – 35 years) ‏ Low socioeconomic background

6. Etiology Poorly understood Several Hypothesis are proposed: 1.Malntrition 2.Exposure to toxics and metals 3.Recuurent intestinal in f ections`

7. 1. Infective hypothesis Abdominal Infection Portal pyaemia Pylephlebitis Thrombosis Sclerosis Obliteration of small and medium sized portal radicle

8. 2. Exposure to trace metals and chemicals Chronic exporure to : Arsenic, 8 vinyl chloride monomer, Coppersulfate Methotrexate hypervitaminosis A 6-mercaptopurine azathiopine corticosteroids

9. 3. Immumological and Immunogenetic hypoothesis Reduction in cytotoxic/ suppressor T - Lymphocytes Decreased T4/T8 lymphocyte ratio Reduction in cell mediated immunity

10. Pathology Liver may be normal or markedly nodular. Nodularity is confined to sub - capsular zone. Portal venous system shows prominent and dilated branches with marked sclerosis the wall. Significant perivascular fibrosis along the portal vein and its branches. Gross pathology

11. Pathology Patchy and segmental sub endothelial thickening of the large and medium sized intrahepatic branches of portal vein. Emergence of new aberrant portal channels is characteristic of NCPF Microscopy:

12. Clinical features Gastrointestinal bleed (one or more well tolerated episodes). Feeling of lump in left upper quadrant of abdomen (due to enlargement of spleen). Left upper quadrant pain (due to perislenitis and splenic infarction) ‏ Symptoms

13. Clinical features Splenomegaly (Size of spleen ranges from 4 – 15cms below left coastal margin) ‏ Liver enlargement (Palpable upto 2cms below coastal margin) ‏ Rarely – Jaundice, Ascites and Liver failure. Signs

14. Laboratory features Anaemia (either microcytic hypochromic/ normocytic normochromic) ‏ Leukopenia Thrombocytopenia Bone marrow is hypercellular Mild compensated DIC(due to endotoxaemia or portosystemic collaterals) ‏ LFT – normal/ mildly deranged.

15. Radiology Portal and splenic veins are dilated and multiple collateral present at the splenic hilum . There is marked thickening of portal vein wall specially of intraheptic branches. There may be spontaneous lieno renal shunt. Ultrasonography

16. Radiology Dilatation of portal and splenic vein along with various collaterals. Spleno porto venography Endoscopy Esophagogastric varices Anorectal varices

17. Differential diagnosis: 1. EHPVO (Extra hepatic portal vein obstruction) ‏ 2. Compensated cirrhosis in the young 3. Tropical splenomegaly syndrome 4. Splenomegaly of myeloid syndrome 5. Kala azar 6. Felty’s syndrome

18. Management It involves control of acute bleeding ( variceal bleeding) and prevention of rebleeding by medical and surgical means. Medical management: 1. Emergency treatment 2. Primary Prophylaxis

19. 1. Emergency Mangement Initial resuscitation with replacement of blood volume loss is done with blood transfusion and IV fluids Specific traement of bleeding lesion done by: a. Pharmacotherapy b. Endoscopictherapy c. Ballon tamponade

20. a. Pharmacotherapy : Drugs used are Somatostatin , octreotide terlipressin vasopressin : 0.1 – 0.5 u/min b. Endoscopic therapy : i . Endoscopic variceal ligation: Banding device attached to the tip of the endoscope is used to ligate varix using rubber bands . One to three bands are applied to each varix ii. Endoscopic sclerotherapy : sclerosants used are 1.5% sodium tetradecyl sulphate, cyanoacrylate , 5% ethanolamine oleate , absolute alcohol, 3% phenol in water and 5 % phenol in oil c. Balloon tamponade : Used only in massive bleeding as temporary measure.

21. 2. Primary Prophylaxis Used in patients with increased chances of bleeding such as patients with large varices, red wale markings on varices and severe liver failure. It includes: a. b – blockers b. Vasodilators c. Combined therapy

22. a. b – Blockers: Noncardioselective b blockers like propanolol and nadolol are used. These drugs act by reducing portal and collateral blood flow. Doses : - Propranolol : 20 mg every 12 hours until there is 25% reduction in resting heart rate Nadolol : 10 mg every 12 hours b . Vasodilators: Isosorbide mononitrate reduce portal pressure gradient. c . Combined therapy: Both b – blockers and Isosrbide mononitrate are used.

23. Surgical Management Indication for Surgery: 1. Failure of endoscopic therapy to control acute bleeding. 2. Recurrent bleeding. 3. Symptomatic hypersplenism. 4. Repeated splenic infection. 5. Patient who desires for one time treatment.

24. Surgery options are: 1. Porto – systemic shunt : a . Total shunt: Portal blood is completely diverted to systemic circulation. Eg : -Central splenorenal shunt with splenectomy , - Mesocaval shunt, - Portocaval shunt. b . Selective shunts: Decompresses the gastrosplenic - portal zone with maintained portal perfusion of the liver through hepato petal collateral. Eg :- -Distal spleno renal shunt (Warren shunt), - Distal spleno caval shunt, - Gastroepiploic to left renal shunt, -Left gastric vein to left renal vein shunt.

25. 2. Devascularisation procedure: Indications: - failed shunt, - patient who have no shuntable vein - in emergency when shunt can not be performed . Eg : Sugiura deevascularization

26. The prognosis of patients with NCPF is good 5 years survival in patients in whom variceal bleeding can be controlled has been reported to be approximately 95–100%. It is not merely absence of cirrhosis, but also of hepatic venous outflow obstruction, such as veno -occlusive disease and Budd– Chiari syndrome.

27. THANK YOU