Non Cirrhotic Portal Fibrosis (NCPF) is a syndrome of obscure etiology characterized by obliterative portovenopathy leading to portal hypertension and massive splenomegaly in young adults from low socioeconomic backgrounds. The exact cause is unknown but is hypothesized to be related to malnutrition, recurrent infections, exposure to toxins or metals. Pathology shows sclerosis of portal vein branches. Clinical features include gastrointestinal bleeding, splenomegaly, and abdominal pain. Management involves controlling acute bleeding episodes and preventing rebleeding through medications or surgery like portosystemic shunts. Prognosis is generally good if bleeding can be controlled.

1. Non Cirrhotic Portal Fibrosis
Dr. Pratik Edwards

3. 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
• Enters 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
• Contributes 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

4. Non-cirrhotic portal fibrosis
• Syndrome of obscure etiology
• ‘Obliterative portovenopathy’ leading to PHT,
• Massive splenomegaly
• well-tolerated episodes of variceal bleeding
• In young adults,
• Low socioeconomic backgrounds
• Normal hepatic functions

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

6. 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

7. Etiology
• Poorly understood
• Several Hypothesis are proposed:
1.Malntrition
2.Exposure to toxics and metals
3.Recuurent intestinal infections`

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

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

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

12. 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

13. 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:

14. 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

15. 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

16. 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.

17. 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

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

19. 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

21. 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

22. 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

23. 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.

24. 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

25. 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.

26. 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.

27. 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.

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

29. • 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.