Non Cirrhotic Portal Fibrosis

Dr. Pratik Edwards

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

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

Lesion in NCPF

•It is generally vascular.

•Location of lesion: 1. Branches of Portal vein 2. Perisinusoidal area of Liver

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

Etiology

• Poorly understood

• Several Hypothesis are proposed: 1.Malntrition2.Exposure to toxics and metals 3.Recuurent intestinal infections`

1. Infective hypothesisAbdominal Infection

Portal pyaemia

Pylephlebitis

Thrombosis

Sclerosis

Obliteration of small and medium sized portal radicle

2. Exposure to trace metals and chemicalsChronic exporure to :

Arsenic, 8 vinyl chloride monomer, Coppersulfate Methotrexate hypervitaminosis A 6-mercaptopurine azathiopine corticosteroids

3. Immumological and Immunogenetic hypoothesis

• Reduction in cytotoxic/ suppressor T - Lymphocytes

• Decreased T4/T8 lymphocyte ratio

• Reduction in cell mediated immunity

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

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:

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

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

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.

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

Radiology

• Dilatation of portal and splenic vein along with various collaterals.

Spleno porto venography

Endoscopy

Esophagogastric varices

Anorectal varices

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

ManagementIt involves control of acute bleeding (variceal bleeding) and prevention of rebleeding by medical and surgical means.

Medical management:

1. Emergency treatment

2. Primary Prophylaxis

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

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.

2. Primary ProphylaxisUsed 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

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.

Surgical ManagementIndication 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.

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.

2. Devascularisation procedure:

Indications: -failed shunt, -patient who have no shuntable vein -in emergency when shunt can not be performed.

Eg: Sugiura deevascularization

• 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.

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