Presentation by DR. MISHAL on the topic of NON CIRRHOTIC PORTAL HYPERTENSION. Its a grey area but very important topic particularly for FCPS residents .

2. NoncirrhoticPortal Hypertension
Dr. Syeda Mishal Saleem
PGT Internal Medicine
POF Hospital, Wah Cantt

3. • Portal hypertension is a clinical manifestation which is defined as the presence of a Hepatic
venous pressure gradient > 5 mmHg.
• The main cause of PHTN is Liver Cirrhosis. There are, however, a variety of disorders which
PHTN without Liver Cirrhosis and falls under the category of Non-Cirrhotic portal hypertension.
• The diseases leading to NCPH are primarily vascular in nature and classified anatomically based
site of involvement as:
INTRODUCTION:
PreHepatic
Hepatic
PostHepatic

4. Hepatic
vein
Sinusoids
Portal
vein
Liver
Splenic vein
Coronary vein
SMV
IMV

5. Causes of NCPH
PreHepatic Hepatic PostHepatic
Presinusoidal Sinusoidal Postsinusoidal
Extrahepatic
portal vein
obstruction
(EVPHO)
Idiopathic
Non cirrhotic portal
hypertension
Amylodosis Sinusoidal obstruction
syndrome/ veno-
occlusivedisease
Inferior vena cava
Obstruction
Splenic vein
thrombosis
Portosinusoidal Vascular
disease
Fibrosis due to Metabolic
causes (Nonalcohol
associated fatty liver disease)
Granulomatous phlebitis
(Sarcoidosis, Myobacterium
avium) Constrictive pericarditis
Splanchnic
arteriovenous
fistulas
Granulomatous liver lesions
(Schistosomiasis)
Fibrosis due to inflammatory
causes( viral hepatitis, CMV)
Phlebosclerosis of hepatic
veins (due to early Alcohol
associated liver disease,
chronic radiation injury,
hypervitaminosis A)
Restrictive Cardiomyopathy
Devlopment disorders (Adult
polycystic liver disease, congenital
hepatic fibrosis,Arteriovenous
fistulas)
Drugs and toxins:
Amiodarone, methoteraxate,
vinyl chloride , copper)
Primary vascular malignancies
(epitheloid
hemangioendothelioma,
angisarcoma)
Billiary Disorders ( Billiary
cholangitis, Autoimmune
cholangiopathy, PSC , Toxic billiary
injury from vinyl chloride )
Defenestration of sinusoidal
lining in early alchol
associated liver disease.
Hepatic venous obstruction /
budd chiari syndrome
Neoplastic occlusion of portal vein (
due to Lymphoma, epithelial
malignancies, CLL)
Infiltrative ( Mastocytosis,
Gaucher disease)
Lipogranuloma

6. .
 INCPH diagnosis is based on clinical criteria and the formal exclusion of any other causes of portal
hypertension. Diagnosis require following criteria :
. Presence of portal hypertension in the abscence of cirrhosis on liver
histology.
Exclusion of conditions that may cause cirrhosis or non cirrhotic portal
hypetension e.g : congenital hepatic fibrosis, sarcoidosis, schistosomiasis
• --
Imaging showing patent portal and heptic veins
At least one
clinical sign of
portal
hypertension
Spleenomeg--
-aly
Esophageal
or Gastric
Varices
Ascites
Portovenou-
-s
collaterals
Exclusion of Chronic liver disease like Chronic Hepatitis B or C virus infection, NASH,
Alchol associated steatohepatitis, Autoimmune hepatitis
Diagnosis:
Idiopathic non cirrhotic portal hypertension is most common in Asia
Idiopathic non cirrhotic portal hypertension

7. Porto-sinusoidal vascular Disease:
It is a broad spectrum term that includes following groups:
 Patients with histologic features of INCPH on liver biopsy (e.g: oblitrative venopathy,
nodular regenerative hyperplasia, incomplete septal cirrhosis) but without clinical
manifestation of portal hypertension (e.g: esophageal or gastric varices)
The etiology of PSVD can be classified in five categories: )
1) Immunological
disorders (i.e.
association with
common variable
immunodeficiency
syndrome,
connective tissue
diseases, Crohn’s
disease, etc.
2) Chronic infections
3) Exposure to
medications or toxins
(e.g. azathioprine,
long term use of
didanosine,
combination of
didanosine and
stavudine, 6-
thioguanine, arsenic),
4) genetic
predisposition (i.e.
familial aggregation
and association with
Turner disease)
5) Prothrombotic
conditions (e.g.
inherited
thrombophilias
myeloproliferative
neoplasm
antiphospholipid
syndrome).

8.  Schistosomiasis is one of the most common causeof NCPH worldwie
 Chronic hepatic schistsomiasis is characterized by :
, .
esophageal
varices
hepatomegaly
spleenomegaly
with
hypersplenism
• schistsomal eggs in stool + liver biopsy - granulomas or
ovas
Diagnosis:
Schistosomiasis

9. • 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
Mesenteric Vein’’ .
• Recent thrombus in the PV along with the presence of cavernoma, however, it excludes
Liver Cirrhosis and other liver diseases like NCPF, as well as HCC.
• Isolated occlusion of the splenic vein or superior mesenteric vein does not constitute
EHPVO
• It is a major cause of PHTN (54%) and UGIB in children (68–84%) from the developing
world, however it occurs in adults too.
Extrahepatic Portal Vein Obstruction

10. Clinical Features
Variceal
bleed:
Esophageal
Varices 84%
Gastric
varices 7%
Portal
Hypertensiv
e
gastropathy
7%
Ectopic
varices- 2%
Duodenal,
anorectal, GB
bed, biliary tree
• Variceal hemorrhages episodes are
often recurrent, related to febrile
illnesses, use of NSAID, and ↑ in
frequency and severity with age till
adolescence.
• In contrast to cirrhosis, these bleeding
episodes are well tolerated in view of
preserved liver synthetic functions.
• Moderate splenomegaly
• Portal biliopathy - 60-100 %
(Asymptomatic)
• Symptomatic Portal bilipathy (pain,
jaundice, fever)- develop in 5-14%
of cases on long-term follow-up
• Growth retardation

11. • Prognosis:
• Prognsis of EHPVO after control of variceal bleed is good,
with long term (>10 years) survival nearly 100%
• Natural history is complicated by development of portal biliopathy
& ectopic varices

12. NCPF (Non-cirrhotic portal Fibrosis)
• NCPF 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.
• The liver functions primarily remain normal, in most cases

13. NCPF (Non-cirrhotic portal Fibrosis)
Diagnostic criteria: (APASL)
• Patent spleno-portal axis and hepatic veins on ultrasound Doppler
• Presence of moderate to massive splenomegaly
• Evidence of portal hypertension, varices, and/or collaterals
• Test results indicating normal or near-normal liver functions
• Icreased or Near-normal HVPG
•Liver histology-no evidence of cirrhosis or parenchymal injury Other features:
• Absence of signs of chronic liver disease
• NOTE: 2/3rd of patients present with G I Bleed

14. PROGNOSIS:
• Uncontrolled variceal bleeding is a common cause of death
• Long term survival after eradication of esophagogastric varices or after
a shunt surgery is nearly 80-100%
• Liver functions usually remain well preserved, but with course of time in
20–33% of cases, liver slowly undergoes parenchymal atrophy with
subsequent decompensation

15. Clinical Features of NCPH:
Abdominal pain due to acute portal vein thrombosis
Severe GI bleed due to hepatic decompensation,
Intestinal infarction may occur when mesenteric venous
thrombosis also occurs with portal vein thrombosis
Spleenomegaly with hyperspleenism
Ascites (25%)
Enlargement of porto-systemic collaterals :Esophagus, Stomach, Rectum,
Retroperitoneum, Biliary (Portal biliopathy), Periumbilical
Hepatic encephalopahy (rare)

16. Laboratory Findings:
LFTS
• Usually normal
• Elevations of alkaline
phosphatase and
GGT are seen with
development of portal
biliopathy, while
hypoalbuminemia
may be seen during
bleed episodes
particularly in the
setting of growth
failure
Pancytopenia
• Due to
Hyperspleenism
Hypercoagulable state :
• Factor V leiden Mutation
• Deficiency of protein C
• Deficiency of protein S
• Antiphopholipid Syndrome
• JAK 2 tyrosine kinase mutations--
myeloproliferative neoplasm
• Elevated factor VIII levels
• Hyperhomocystenemia
• Mutation of TT677 of
methylenetetrahydrofolate
• Mutation in gene that codes for
thrombin- activatable fibrinolysis
inhibitor.
• Hypercoagulopathy secondary to
portosystemic shunting and
reduced blood flow

17. Imaging:
Color Doppler USG
Contrast enhanced
CT
Initial
Diagostic
Test Magnetic
Resonance
Angiography
(MRA)
Confirmatory
Test
 EUS may be helpful in some casese.
 In patients with jaundice, Magnetic Resonance Cholangiography may
demonstrate compression of bile duct by a large portal cavernoma
(portal bilipathy), a finding that is more common in patients with
Hypercoagulable state.
 In patients with Pylephlebitis, CT may demonstrate intra-abdominal
source of infection, thrombosis, or gas in the portl venous system or a
hepatic abscess.

18. Esophageal or
Gastric vices
Endoscopy
> Non cirrhotic
portal fibrosis
>Schistosomia-
-sis
>Nodular
regenerative
hyperplasis
> Sinusoidal
dilatation
Liver biopsy
Distinguish
noncirrhotic portal
hypertension from
cirrhosis
Elastography
OTHER STUDIES:

19. TREATMENT:
Endoscopic band ligation
Beta blockers (non selective) to reduce portal pressure
Portosystemic Shunting (TIPS) , is reserved for faliures of
endoscopic therapies
Liver transplantation for progressive liver dysfunction
Anticoagulation or thrombolytic theray

20. Role of Anticoagulation:
 Anticoagulation, particularly with low molecular weight Heparin or
unfractioned Heprain or thrombolytic therapy may be indicated for
for isolated acute portal vein thrombosis,which leads to atleast
PARTIAL RECANALIZATION in upto 75% of cases when started
within 6 months of thrombosis.
 It can also be possibly used for acute Splenic vein thrombosis.
 Oral anticagulat is continued long term if a hypercogulable disorder
is identified or if an acute portal vein thrombosis extends into the
mesenteric veins.

21. CASE 1:
A 55 years old man, hypertensive and diabetic, came in ER with complaints of 2-3 episodes of
severe vomitings with blood and pain abdomen for 2 days. He had repeated visits in
gastroenterology clinic for repeated GI infections.No other significant history.
On examination there is massive spleenomegaly.
His BCP is showing pancytopenia, LFTS are normal.
USG- Abdomen shows thick ehogenic portal veins and spleenomegaly. Liver size is normal.
1. What is your diagnosis?
2. What other test you are going to do in emergency managment??
3. What is your treatment plan??
1. Non Cirrhotic Portal hypertension (idiopthic)
2. Endocopy -- varices
3. Endoscopic band ligation, followed by CT Abdomen/MRA
and then proceed accordingly

22. CASE 2:
A 47 years old man, resident of Phillipines, referred by GP to Gatroenterology clinic for complaint of 2
episodes of vomiting with blood since morning, abnormal LFTS and abnormal Liver ultrasound. He was noted
to have abnormal Lfts during his annual checkup 1 year back. His anomalous results persisted, and
diagnosed as fatty liver on basis of laboratory results at that time..
6 years back he was suspected to had Tuberculosis, so he was put on ATT.
• Now, his labs revealed slightly elevated basophils (1.2%) , AST 66U/L, GGT145U/L.
• Hepatitis pannels are negative for Hep B and C. Iron studies , stool and urine culture are normal for acute
infections.
• USG- Abdomen showed an extremely hterogenous parenchyma, which appeared to be interstitial fibrosis
throughout the liver with increased septal lines, suggestive of underlying liver fibrosis. And multiple high
echogenic nodules. Also high echogenic portal veins. CT abdomen showed dilated callliber of PV, multiple
calcified nodules with normal liver margins.
1. What is your suspected diagnosis??
2. What other diagnostic tests you are going to perform for final diagnosis??
1. Chronic Schistosomiasis leading to NCPH
2. Liver Biopsy

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24. • uptodate, non cirrhotic portal hypertension, 2021
• cmdt, non cirrhotic portal hypertension, 2021
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