This document provides an overview of extra-hepatic portal vein obstruction (EHPVO). It discusses the prevalence, etiology, clinical features, diagnosis, management, and prognosis of EHPVO. Key points include that EHPVO is characterized by obstruction of the portal vein outside the liver, it is more common in India than Western countries, causes include thrombosis and hypercoagulable states, clinical features include variceal bleeding and splenomegaly, management involves preventing complications through anticoagulation and procedures like TIPS, and prognosis depends on the underlying cause and presence of complications.

1. Extra-Hepatic Portal
Vein Obstruction
Dr Manoj Kumar Kurmana
Resident MU5
Under guidance of
Dr Maniram Kumhar (Head of Dept ,Unit & Sr Prof)
Dr Munesh Kumar ( Associate Prof)
Dr Ravindra Tiwari ( Asst. Prof)
Dr Harsh Tak (Asst. Prof)

2. Table of contents
01
04
02
05
03
Introduction
Prevalence
Etiology
Clinical Features
Acute PVT & Portal Cavernoma
06
07
08
09
Diagnosis
Management
Prognosis
References

3. Introduction
◤ Portal Hypertension : It is characterized by elevated hepatic
venous pressure gradient (HVPG) exceeding 5 mmHg.
HVPG = Wedged Hepatic Venous Pressure – Free Hepatic Venous
Pressure
◤ Pathophysiology :
Pressure = Flow x Resistance
 Increased intrahepatic resistance due to cirrhosis,
regenerative nodules, and microthrombi.
 Increased splanchnic blood flow due to vasodilation within
the splanchnic vascular bed.
◤ Clinically Significant Portal Hypertension: HVPG ≥10 mmHg, risk
of decompensation with variceal bleeding, ascites, or hepatic
encephalopathy.

4. Introduction

5. Introduction
◤ Most Common Cause of Portal Hypertension :
Cirrhosis (>60% of cases), portal vein
obstruction, and coagulation disorders.
◤ Complications: Gastroesophageal varices,
ascites, hypersplenism.
◤ Diagnosis : Thrombocytopenia, enlarged
spleen, ascites, and esophageal varices.
USG, contrast-enhanced imaging &
endoscopy for varices.

6. Introduction
◤ EHPVO(Extra-Hepatic Portal Vein Obstruction) is a medical condition characterized by the
blockage of the portal vein, typically outside the liver. This condition significantly impacts
portal blood flow and can lead to a range of clinical issues
◤ EHPVO is defined as "a vascular disorder of liver, characterized by obstruction of the
extra-hepatic PV with or without involvement of intra-hepatic PV radicles or splenic or
Superior Mesentric Vein"
◤ Types of EHPVO:-
 Primary EHPVO: This is the most common form. It can lead to acute portal vein
thrombosis and portal cavernoma.
 Secondary EHPVO: Caused by factors like malignant invasion, compression(bland),
or encasement of the portal vein.

7. Prevalence
◤ How common is EHPVO?
 Data Estimates from Sweden Studies show varying prevalence estimates,
indicating that EHPVO often develops at a late stage of underlying diseases. An
autopsy study in Sweden estimated the prevalence of EHPVO to be as high as 1.0%.
 In another Swedish study based on hospital discharge diagnoses, however, the
prevalence was much lower (3.7 per 100,000 population).The difference between
these 2 estimates suggests that EHPVO commonly develops at a late stage of
many diseases.
 Chronic liver disease and abdominal malignancy are each found in about one third
of patients.
 Regional Variations: The occurrence of EHPVO can differ in various regions.

8. Prevalence
◤ EHPVO is common in India and is responsible for 6% to 40% of all patients with
portal hypertension in various series
 This proportion is <10% in the West.
 Males are predominantly affected
 In children, EHPVO accounts for up to 70% of cases of portal
hypertension.

9. Aetiopathogenesis of EHPVO
◤ Etiology in Different Age Groups:
 Children: Commonly associated with thrombosis of the portal vein.
 Contributing factors include infections like neonatal umbilical sepsis,
sepsis, severe dehydration, and abdominal surgery.
 Hypercoagulable states may also play a role, though rarely.

10. Aetiopathogenesis of EHPVO
◤ Adults: Acute portal vein thrombosis is the most frequent presentation
 Other causes include liver diseases (cirrhosis, hepatocellular carcinoma)
and pancreatic diseases (chronic pancreatitis, trauma, tumors).
 Hypercoagulable states, Myeloproliferative disorders, inflammatory
bowel, disease, dehydration, OCP use, pregnancy although less
common, are often associated with EHPVO.

11. Aetiopathogenesis of EHPVO
◤ Portal Biliopathy:
 An uncommon but notable complication characterized by bile duct
abnormalities due to collaterals compressing the bile duct.
 Can lead to symptoms like biliary colic, obstructive jaundice, or
cholangitis.
 Diagnosable by cholangiography but rarely symptomatic.
 Role of Prothrombotic Conditions:
 Occult prothrombotic disorders are frequently detected in adults with
EHPVO.
 Examples include JAK2 mutation, antithrombin III, protein C, and protein
S deficiencies, prothrombin gene mutation, factor V Leiden mutation, and
more.

12. Clinical Features of EHPVO
◤ Clinical Features in Children:
 Most Common Presentation: Recurrent upper gastrointestinal (GI)
bleeding from esophageal varices (hematemesis and melena).
 Tolerated Bleeding: Bleeding episodes are usually well-tolerated.
 Splenomegaly: Almost universal in pediatric EHPVO patients.
 Asymptomatic Hypersplenism: Often asymptomatic, although some may
present with symptomatic anemia or thrombocytopenia.
 Growth Retardation: Approximately 50% of children with EHPVO
experience growth retardation
 Transient Ascites: Occurs in some children following upper GI bleeding.

13. ◤ Clinical Features in Adults:
 Acute EHPVO: Presents as acute abdominal pain, fever, ascites, and intestinal
ischemia.
 Chronic EHPVO: Characterized by recurrent upper GI bleeding from
esophageal or gastric varices.
 Uncommon Presentations: Some patients may experience transient
transudative ascites after upper GI bleeding, symptomatic hypersplenism, or
portal biliopathy (rarely symptomatic).
Clinical Features of EHPVO

14.  Variceal Bleeding: May occur from ectopic varices in the duodenum or
anorectal region.
 Liver Dysfunction: Some patients may develop liver dysfunction during long-
term follow-up.
 Hepatitis Risk: Patients at risk of acquiring hepatitis B and hepatitis C through
blood transfusions for recurrent GI bleeding.
Clinical Features of EHPVO

15. Acute Portal Vein Thrombosis in EHPVO
◤ Acute PVT is characterized by the presence of a thrombus, shown on imaging as solid
material in the lumen of the portal vein, in the absence of a cavernoma. The
subsequent transformation of an acutely thrombosed portal vein into a portal
cavernoma has frequently been referred to as chronic PVT.

16. Acute Portal Vein Thrombosis in EHPVO
◤ Risk Factors for PVT
 Hyper-coagulable States: These include various conditions like antiphospholipid
syndrome, factor V Leiden mutation, and more
 Infections: Conditions like appendicitis, cholangitis, and liver abscess can lead to
PVT.
 Inflammatory Diseases: Inflammatory conditions like IBD or pancreatitis may also
contribute.

17. Acute Portal Vein Thrombosis in EHPVO
 Impaired Portal Vein Flow: Conditions like cirrhosis, cholangiocarcinoma, and
nodular regenerative hyperplasia can obstruct blood flow in the portal vein.
 Miscellaneous Factors: Central obesity, bladder cancer, and living at high
altitudes are among other possible risk factors.
 Pathogenesis of Acute PVT
 Acute PVT can be caused by inflammatory foci, injuries to the portal veins, or
stasis of blood in the portal venous bed.

18. Acute Portal Vein Thrombosis in EHPVO
◤ Clinical Features
 Patients with acute PVT often experience severe abdominal pain,
sometimes with a fever.
 Symptoms and physical examination may vary based on the stage and
complications of PVT.

19. Acute Portal Vein Thrombosis in EHPVO
◤ Diagnosis and Natural History
 Imaging, like Doppler US or contrast-enhanced CT, helps in diagnosing
acute PVT.
 Its difficult to Dx on USG as the thrombus may be hypoechoic & With time,
it becomes more echogenic and easier to identify but where as Colour
Doppler will be able to demonstrate absent flow & Enhancement of walls
on CT & Complete or Partial non opacification of vein indicate PVT.
 Early anticoagulation treatment is associated with better outcomes in
many cases.

20. Portal Cavernoma in EHPVO
◤ Portal cavernoma is a condition characterized by the disappearance of the
normal portal vein, replaced by a network of portoportal collaterals.
◤ The distinction between chronic portal vein thrombosis (PVT) and portal
cavernoma is based on the presence of documented acute PVT in the former.

21. Portal Cavernoma in EHPVO
◤ Causes of Portal Cavernoma
 The causes of portal cavernoma are similar to those of acute PVT,
although there is a lower proportion of recognized local factors at the
stage of portal cavernoma.
◤ Liver Structure and Function
 In the presence of a portal cavernoma, liver structure and function
typically remain normal, even though atrophy of peripheral segments and
hypertrophy of central segments may occur.

22. Portal Cavernoma in EHPVO
◤ Symptoms and Clinical Findings
 Patients with portal cavernoma often remain asymptomatic until
complications related to portal hypertension arise.
 GI bleeding related to portal hypertension is the most common
complication.

23. Portal Cavernoma in EHPVO
 Diagnosis
 Imaging modalities, such as ultrasound (US), CT, and MRI, are useful in diagnosing
portal cavernoma where Cavernous transformation appears as numerous tortuous
vessels occupying the portal vein bed is seen on USG & numerous vascular
structures in the region of the portal vein, which enhance during the portal venous
phase, and not during the arterial phase on MULTIPHASE CT.
 GI bleeding is a common complication.
 Recurrent venous thrombosis and biliary complications may also occur.

24. Portal Cavernoma in EHPVO
◤ Therapeutic Approaches
 Anticoagulation therapy is sometimes recommended to prevent
recurrent thrombosis.
 Management of portal hypertension complications is crucial.
 Nonselective β-adrenergic blockers and endoscopic variceal ligation can
be used to prevent bleeding.
 In selected cases, surgical or radiological interventions like TIPS or meso-
Rex shunt (mesenterico-left portal vein shunt)can be considered.

25. Portal Cavernoma in EHPVO
◤ Prognosis
 Mortality in patients with a portal cavernoma is often related to the
underlying condition, and not typically due to the complications of portal
hypertension

26. Complications
◤ Complications of EHPVO
 Portal Hypertension: The most common complication, leading to the
development of varices and related issues.
 Gastrointestinal Bleeding: Varices, particularly esophageal varices, can
rupture and result in life-threatening bleeding.
 Portal Vein Thrombosis: Thrombosis within the portal venous system can
further exacerbate portal hypertension and affect liver function.
 Ascites: Accumulation of fluid in the abdominal cavity is often associated
with advanced portal hypertension and liver disease.
 Splenomegaly: Enlargement of the spleen is common and can lead to
hypersplenism with reduced platelet counts.

27. Management
◤ Management of EHPVO
Effective management of EHPVO is crucial to improve the patient’s quality of life and
address the underlying cause.
◤ Treatment Goals
 The primary goals of EHPVO management are to prevent and manage
complications, improve the patient’s quality of life, and address the underlying
cause

28. Management
 Anticoagulation Therapy
 In cases of acute EHPVO, anticoagulation therapy may be initiated to prevent
further thrombus extension and reduce the risk of complications.
 Anticoagulation helps to prevent clot formation and allows for the recanalization
of the portal vein.
 Endoscopic Interventions
 Endoscopic procedures are used to manage varices, particularly esophageal
varices.
 Techniques like variceal band ligation and sclerotherapy can reduce the risk of
variceal bleeding.

29. ◤ TIPS Procedure
 In patients with severe portal hypertension and recurrent bleeding
despite other measures, a Transjugular Intrahepatic Portosystemic Shunt
(TIPS) procedure may be considered.
 TIPS involves creating a shunt to redirect blood flow and reduce portal
pressure.
 Surgical Shunts
 In some cases, surgical shunts such as the meso-Rex bypass may be an
option to manage portal hypertension when other interventions are not
suitable.
 These procedures aim to redirect blood flow within the portal system.

30.  Liver Transplantation
 Liver transplantation is considered in severe cases with liver failure or
intractable complications that do not respond to other treatments.
 It serves as a definitive treatment, providing a new, functioning liver

31. ◤ Long-Term Follow-Up
 Patients with EHPVO require long-term follow-up to monitor their
condition, assess the effectiveness of treatments, and make necessary
adjustments.
 The goal is to prevent complications and optimize quality of
life.Collaborative Care
 EHPVO management often involves a multidisciplinary approach,
requiring collaboration between hepatologists, gastroenterologists,
interventional radiologists, and surgeons.
 Individualized treatment plans are developed based on the patient’s
specific condition and needs.

32. Prognosis
◤ Prognosis of EHPVO
 The prognosis of extrahepatic portal vein obstruction (EHPVO) can vary
depending on several factors.
 Factors influencing prognosis include the underlying cause, age at
diagnosis, the extent of portal vein involvement, and the presence of
complications.
 Patients diagnosed at a younger age may have better long-term outcomes.

33. NCPF
◤ Non Cirrhotic Portal Fibrosis (NCPF) is a distinct condition characterized by
'obliterative portovenopathy' leading to Portal hypertension,
massive splenomegaly and well-tolerated episodes of variceal bleeding, one of
the important causes of noncirrhotic portal hypertension which is also Known as
Idiopathic Portal Hypertension (IPH) in some Asian region where as Different terms
are used worldwide, including hepatoportal sclerosis and noncirrhotic
intrahepatic portal hypertension.
◤ NCPF/IPH is a disease of uncertain etiology characterized by periportal fibrosis
and involvement of small and medium branches of the portal vein, resulting in the
development of portal hypertension where liver functions and structure primarily
remain normal (APASL)

34. NCPF
◤ Uncertain Etiology : Noncirrhotic Portal Fibrosis (NCPF) lacks a clear cause,
making its pathogenesis intricate.
◤ Portal Venous Injury:
○ NCPF is linked to portal venous injury, especially in the presinusoidal region.
○ Intestinal infections can trigger intramural thrombi and stellate cell
activation, leading to perisinusoidal fibrosis.
◤ Xenobiotic Exposure:Suspected factors like arsenic exposure, often from
contaminated water, play a role where Arsenic may induce hepatic oxidative
stress and proinflammatory cytokines, but its involvement is debated.
◤ Immunologic Abnormalities: NCPF frequently involves immunologic irregularities,
including T-lymphocyte and adhesion molecule issues.
◤ Other Factors : Potential associations include infections, autoimmune disorders,
prothrombotic states, and exposure to various chemicals or medications.
◤ The majority of NCPF cases have an unknown cause.

35. NCPF vs EHPVO vs Cirrhosis

36. References
◤ Filipe Gaio Nery, Dominique Charles Valla.,Vascular Diseases of the Liver In M. Feldman, L. S. Friedman, &
L. J. Brandt (Eds.), Sleisenger and Fordtran’s Gastrointestinal and Liver Disease: Pathophysiology,
Diagnosis, Management (12th ed.,pg 1324-1326)
◤ Y P Munjal, Surendra K Sharm et.al API Textbook of Medicine, Ninth Edition
◤ Ghai OP,Piyush Gupta, Paul VK et.al.,Ghai Essential Pediatrics 8th Ed.
◤ Longo, D. L., Fauci, A. S., Kasper, D. L., Hauser, S. L., Jameson, J. L., & Loscalzo, J. (2018).
Harrison’s Principles of Internal Medicine (21st ed.). McGraw-Hill Education.

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