portal hypertension

1. PORTAL HYPERTENSION IN
CHILDREN
Dr Harshavardhan

2. CONTENTS
• INTRODUCTION
• ANATOMY
• ETIOLOGY/CLASSIFICATION
• CLINICAL MANIFESTATION
• DIAGNOSIS
• TREATMENT
• SUMMARY

3. Introduction
• The normal portal venous pressure is about 5-
10mmhg.
• Portal hypertension is defined when portal
venous pressure is 10-12mmhg.
• portal venous pressure builds up when their is
an obstruction to portal venous flow

4. Introduction cont..
• Portosystemic collaterals start developing with
porovenous pressure of 10 mmhg.
• But variceal bleeding occur when portovenous
pressure exceeds 12 mmhg

5. ANATOMY
• The portal system includes all veins that carry
blood from the abdominal part of the
alimentary tract, the spleen, pancreas and
gallbladder

6. ANATOMY
• The portal vein is formed
by confluence of the
splenic,superior
mesentric vein posterior
to head of pancrease.
• The coronary vein
draining the gastric
venous bed insert in to
portal vein at or distal to
the splenomesentric
confluence

7. ANATOMY
• Inferior mesentric vein
drain into splenic vein
anywhere along its
lenght

8. Collateral circulation
• When the portal circulation is obstructed,
whether it be within or outside the liver
• A remarkable collateral circulation Develops
to carry portal blood into the systemic veins

9. Collateral circulation
• There are four main groups of collaterals
formed during intrahepatic obstruction:
Group 1-
• (a)At cardia of stomach: left gastric
vein, posterior gastric and short gastric veins
of the portal system anastomose with the
intercostal, diaphragmo - oesophageal and
azygos minor veins of the caval system.

10. Collateral circulation
• (b) At anus: superior haemorrhoidal vein of
the portal system anastomoses with the
middle and inferior haemorrhoidal veins of
the caval system.
Group II: paraumblical veins form collaterals
with abdominal wall veins
Group III: where the abdominal organs are in
contact with retroperitoneal tissues or
adherent to the abdominalwall.

11. Collateral circulation
• Group IV:splenic vein forms collaterals with
left renal vein via diaphragmatic, pancreatic,
left adrenal or gastric veins.

12. Sites of portal systemic collateral circulation

13. Classification of portal hypertension
PRESINUSOIDAL
SINUSOIDAL
POST
SINUSOIDAL
EXTRAHEPATIC
INTRAHEPATIC
EXTRAHEPATIC
INTRAHEPATIC
PORTAL VEIN
THROMBOSIS,SPLENI
C VEIN THROMBOSIS
SCHISTOSOMIASIS,PRIM
ARY BILIARY
SCLEROSIS,CHRONIC
ACTIVE
HEPATITIS,CONG.HEPATI
C FIBROSIS,SARCOIDOSIS
CIRRHOSIS,VIT A
INTOXICATION,CYTOTOX
IC DRUGS
HEPATIC VEIN
THROMBOSIS
VENOOCCLUSIVE
DISEASE

15. classification of portal hypertension
TYPE DESCRIPTION
1. HEPATOCELLULAR
INTRINSIC LIVER DISEASE WITH
INCREASED LIVER FIBROSIS
2. VASCULAR
PREHEHEPATIC EXTRAHEPATIC PVT,CAVERNOUS
TRANSFORMATION OF PORTAL
VEIN,EXTRINSIC COMPRESSION OF
PORTAL VEIN
POSTHEPATIC BUDD-CHIARI SYNDROME,STENOSIS OF
HEPATIC VEIN ORIFICE
HIGHFLOW ARTERIO VENOUS COMMUNICATION
INTRAHEPATIC OR EXTRA HEPATIC

16. Hemodynamics in portal hypertension.

17. Clinical presentation
GASTROINTESTINAL HEMORRHAGE:
• Bleeding from the GI tract is one of the most
common, dramatic,and ominous signs of portal
hypertension.
• Bleeding most commonly occurs from varices in the
distal esophagus and gastric cardia. Rectal bleeding is
less common
• Variceal hemorrhage may take the form of
hematemesis,hematochezia, melena, or chronic
anemia.

18. Clinical presentation
Hypersplenism:
• Silent splenomegaly is often the first
sign of a serious underlying disorder of
portal hypertension.
• Hypersplenism occurs particularly in
children with extrahepatic portal vein
thrombosis and no other stigmata of
liver disease.
• patients may present with severe
thrombocytopenia and leukopenia from
splenic sequestration of platelets and
white blood cells

19. Clinical presentation
Encephalopathy:
• Encephalopathy occurs in patients with advanced liver
disease with jaundice and low levels of liver-dependent
coagulation factors or low albumin levels.
• Learning disabilities,behavioral abnormalities are
manifestations of encephalopathy in children ,
• In contrast to the traditional signs of disorientation, memory
loss, and drowsiness commonly seen in adults
• Children may also have accompanying hyperammonemia.

20. Clinical presentation
Bleeding from Nongut Sites:
• Severe thrombocytopenia can lead to hematuria,
menorrhagia in adolescent girls, epistaxis, and
hematochezia.
• In cases of advanced liver disease, hemorrhagic
complications in the lungs may cause severe respiratory
compromise.

21. Clinical presentation
Ascites:
• Children with ascites may have advanced
liver disease with synthetic failure.
• Ascites may be accompanied by a low
serum albumin level ,decreased plasma
oncotic pressure.Dilated lymphatics in the
abdomen from increased hydrostatic
pressure in all portal tributaries.
• There will be an increase in renal tubular
absorption of sodium and water in
patients with decompensated cirrhosis

22. Clinical presentation
• Signs of liver failure:
• may be apparent in
cirrhotics like palmar
erythema,
gynecomastia, spider
naevi and loss of axillary
and pubic hair.

23. Diagnosis
• The diagnosis of the underlying cause of portal
hypertension depends on the synthesis of the clinical
information gathered from the parents and the child and the
results of imaging tests and laboratory investigations.

24. History and general examination
History:
• Relevant to cirrhosis or chronic hepatitis .
• Gastrointestinal bleeding: number, dates, amounts,
• Results of previous endoscopies
• Patient history: blood transfusion, hepatitis B,
hepatitis C, intra - abdominal, neonatal or other
sepsis, myeloproliferative disorder

25. History and general examination
Examination
• Signs of hepatocellular failure
• Abdominal wall veins:
site
direction of blood fl ow
• Splenomegaly
• Liver size and consistency
• Ascites
• Oedema of legs
• Rectal examination

26. Laboratory investigations
a. Hematology especially to look for any evidence of
hypersplenism.
b. Liver function tests to differentiate cirrhotic from
non cirrhotic portal hypertension and to classify
them as per Child Pugh classification
c. Coagulation profile
d. Viral markers

27. Upper GI Endoscopy
• Is used in establishing the cause of
GI bleeding and to confirm the
presence of varices in the esophagus
and stomach.
• Varices are small ( ≤ 5 mm diameter)
or large ( > 5 mm diameter) when
assessed with full insufflation.
• The larger the varix the more likely it
is to bleed.

28. Endoscopy cont...
• Dilated subepithelial
veins may appear as
raised cherry - red spots.
• The haemocystic spot is
approximately 4 mm in
diameter.
• All these signs are
associated with a higher
risk of variceal bleeding.

29. Endoscopy cont...
• Portal hypertensive
gastropathy seen in
endoscopy as a mosaic
- like pattern with small
polygonal areas,
surrounded by a whitish
-yellow depressed
border.

30. Ultrasound
• The first imaging study in any
child who presents with
hematemesis should be
abdominal ultrasonography.
• Cavernous transformation of the
portal vein and portal vein
thrombosis are best diagnosed
by ultrasonography

31. Ultrasound
• Liver parenchymal abnormalities such as
nodularity, inhomogeneity, or the presence of
cysts can be seen.
• Information about the size of the spleen can
be obtained.
• To know post operative patency of porto
systemic shunt.

32. Ultrasound
• Duplex Doppler has been used to measure
portalblood flow.
• In cirrhosis, the portal vein velocity tends to fall
and when less than 16 cm/s ,portal hypertension
is likely.
• A complete evaluation of the intra-abdominal
vasculature including the hepatic veins, the
patency of the splenic and superior mesenteric
veins, and the inferior vena cava is possible using
duplex usg

33. Computed tomography (CT) and magnetic
resonance (MR) angiography
• Are excellent diagnostic tools and have
supplanted conventional digital angiography
for most purposes.
• Both modalities provide excellent information
about all the intra-abdominal vessels and
detailed information about the liver anatomy
including the bile ducts.

34. Computed tomography (CT) and
magnetic resonance (MR) angiography
• CT-angiography has several advantages;
• It can be done more quickly and
• Is less prone to image degradation from
motion artifact than in MR angiography

35. Computed tomography (CT) and
magnetic resonance (MR) angiography
Contrast - enhanced CT scan in a patient
with cirrhosis and a large retroperitoneal
retrosplenic collateral circulation (arrow).
l, liver; s, spleen.

36. Venography
• Is an invasive modality that has few indications as a
diagnostic tool in children with portal hypertension.
• cases of unusual vascular malformations such as
arteriovenous communications in the abdomen or
liver that may best be delineated by venography.

37. Venography
Splenic venography:
• used to outline splenic
and portal veins
• The collateral circulation
is particularly well
visualized.
• Splenic venography has
now been replaced by
less invasive procedures.
The gastro - oesophageal collateral
circulation can be seen and the
intrahepatic portal vascular tree is
distorted( ‘ tree in winter ’ appearance).

38. Liver biopsy
• Liver biopsy especially to ascertain the
etiology of cirrhosis.
• For this patient should not have ascites and
• should have corrected coagulation
parameters.

39. Portal pressure measurement
• Measurements are
taken in the wedged
hepatic venous pressure
(WHVP) and free
hepatic venous pressure
(FHVP).
• The hepatic venous
pressure gradient
(HVPG) is the difference
between WHVP and
FHVP.

40. Portal pressure measurement
• The normal HVPG is 5 – 6 mmHg and values of 10
mmHg or more represent clinically significant portal
hypertension.
• HVPG is related to survival and also to prognosis in
patients with bleeding oesophageal varices

41. Portal pressure measurement cont..
• The procedure may be used to monitor
therapy, for instance the effect of beta -
blockers such as propranolol, with optimal
target reduction of HVPG by 20% from
baselineor to less than 12 mmHg, which
results in a reduced risk of bleeding

42. Management of a cute variceal
bleeding
Management of acute variceal bleeding include:
• 1.General measures
• 2..vasoactive drugs
• 3. Sengstaken – Blakemore tube and self - expanding
oesophageal stent
• 4. Endoscopic banding ligation and injection of
varices.
• 5. Emergency transjugular intrahepatic stent shunt

43. General measures
• Patients admitted with acute variceal hemorrhage
require intense resuscitation with blood and
crystalloids, replacement of coagulation factor
deficiencies with fresh frozen plasma.
• If ascites is very tense, intra - abdominal pressure
may be reduced by a cautious paracentesis and
intravenous albumin replacement and the use of
spironolactone.

44. vasoactive drugs
• Vasoactive drugs lower portal venous pressure
and should be started even before diagnostic
and therapeutic endoscopy.
Vasopressin and terlipressin:
• lowers portal venous pressure by constriction
of splanchnic arterioles.

45. vasoactive drugs cont..
• can cause coronary vasoconstriction and an
electrocardiogram should be taken before they are
given.
• S/E :Abdominal colicky discomfort and evacuation of
the bowels together with facial pallor are usual
during the infusion
• Myocardial ,intestinal ischaemia,rarely infarction are
complications
• Dose:2 mg intravenously every 6 h for 48 h. It may be
continued for a further 3 days at 1 mg every 4 – 6 h.

46. vasoactive drugs cont..
• Octreotide and vapreotide are synthetic analogues of
somatostatin.
• Octreotide is started as a continuous infusion at 1 to
2 mg/kg/hr, up to a maximum of 100 mg/hr, and
continued for as long as symptoms of bleeding
persist.

47. vasoactive drugs cont..
• The long-term medical management of children with
portal hypertension includes the use of nonselective
beta blockers such as propranolol or nadolol,
• Reduces splanchnic blood flow and wedge hepatic
vein pressure.
• Their use may decrease the incidence of recurrent
bleeding by as much as 50% and lessen the need for
liver transplantation in patients with liver disease.

48. Sengstaken – Blakemore tube
• The use of oesophageal
tamponade has decreased
markedly with the use of
vasoactive drugs,
oesophageal sclerotherapy
and TIPS.
• The gastric balloon is infl
ated with 250 mL of air,
oesophageal tube is then
infl ated to a pressure of 40
mmHg,

49. Sengstaken – Blakemore tube
• The sengstaken black
more tube is kept in
place until emergency
therapeutic endoscopy
or tips can be
performed.
• The tube should not be
placed continously for
more than 8 hours.

50. Sengstaken – Blakemore tube
Complications include:
• obstruction to upper airways.
• Ulceration of the lower oesophagus complicates
prolonged or repeated use.
• Oesophagel rupture can occur, usually when the
gastric balloon is wrongly inflated in the oesophagus

51. Endoscopic sclerothrapy
• Sclerosing solutions
include sodium
morrhuate,
ethanolamine, sodium
tetradecyl sulfate, and
polidocanol are used .
• Generally, two to three
injections at 1 mL per
injection are required for
each varix, up to a
maximum of 10 to 15 mL
per session

52. Endoscopic sclerothrapy
• Acute complications include chest pain, esophageal
ulceration, and mediastinitis
• chronic ones include esophageal strictures from
fibrosis after multiple injection sessions.

53. Endoscopic banding
• Banding was found to
be a more effective,
more rapid, and safer
method of reducing the
chance of bleeding from
varices
• The incidence of
complications and of
long-term rebleeding
was lower with
banding.

54. Endoscopic banding
• Obliteration of varices was accomplished in almost
100% of children after only two sessions.
• Esophageal banding in concert with pharmacologic
control has become the procedure of choice in the
early therapy of bleeding esophageal varices

55. GLUE INJECTION
• Injection of cyanoacrylate glue is particularly
indicated for bleeding gastric varices in the
fundus as it is more effective than ligation or
sclerotherapy.

56. TRANSJUGULAR INTRAHEPATIC PORTOSYSTEMIC
SHUNTS(TIPS)
• The main indication for
TIPS is variceal
hemorrhage recalcitrant
to more conservative
therapy with endoscopy
or octreotide
• usually reserved for
patients with advanced
liver disease and serves
as a bridge to
transplantation.

57. TRANSJUGULAR INTRAHEPATIC
PORTOSYSTEMIC SHUNTS(TIPS)
• Other indications include refractory ascites, hepatic
venous outflow obstruction in both transplant and
nontransplant patients, and hepatorenal syndrome.
• The TIPS method has been used in children with
cystic fibrosis, biliary atresia,and congenital hepatic
fibrosis.

58. TRANSJUGULAR INTRAHEPATIC
PORTOSYSTEMIC SHUNTS(TIPS)
• Its primary limitation is the high rate of shunt
thrombosis.
• Vigilance is required to monitor shunt patency
and to declot the shunt when it is thrombosed
• The use of PTFE stents has greatly reduced the
rate of occlusion compared to bare metal
stents

59. Surgical procedures
SHUNTING NON SHUNTING
SELECTIVE
NON
SELECTIVE
1.DISTAL
SPLENORENAL
SHUNT(DSRS)
2.REX SHUNT
1.PORTOCAV
AL SHUNT
2.MESOCAVA
L SHUNT
1.SPLENECTOMY
2.HASSAB OPERATION
3.TERMINAL
ESOPHAGO-PROXIMAL
GASTRECTOMY
4.ESOPHAGEAL
TRANSECTION

60. Porto-systemic shunting
• The aim is to divert blood flow from portal
system to systemic circulation by
anastomosing the portal vein or its tributaries
i.e. splenic vein or superior mesenteric vein to
renal vein or IVC in order to reduce pressure in
the varices.

61. SHUNTING
NON SELECTIVE SHUNTING:
• 1.Portocaval shunting: The portal vein is
joined to the inferior vena cava either end to
side, with ligation of the portal vein, or side to
side.

62. NON SELECTIVE SHUNTING:
2.mesocaval shunting:
• shunt is made between
the superior mesenteric
vein and the inferior
vena cava using a
Dacron graft

63. Selective shunts
1. Selective ‘ distal ’
splenorenal shunt :
• Veins feeding the
oesophagogastric
collaterals are divided
• while allowing drainage
of portal blood through
short gastric – splenic
veins through a
splenorenal shunt to the
inferior vena cava

64. Selective shunts
2.rex shunting:
• Done in long standing
extrahepatic portal
hypertension with
portal vein thrombosis.

65. Complications of shunting
• Shunt thrombosis
• Anastomotic stenosis
• Ascites
• Increase incidence of hepatic encephalopathy

66. LONGTERM SURVIVAL OF PORTAL – SYSTEMIC
SHUNTS
• Operative mortality varies between 15 and 90%
depending upon the liver function. In Child’s A it may
be as low as 15% but in Child’s C, it may be as high as
90%.
• The average survival of cirrhotic patients after shunt
surgery, however, is only 5 years and a liver
transplantation is the only definitive mode of
treatment in these patients

67. Hepatic t ransplantation
• Liver transplant must be considered for variceal
bleeding occurring with end -stage liver disease.
• if there have been at least two episodes of bleeding
from varices despite optimal therapy.
• Splenorenal and mesocaval shunts and TIPS are not
contraindications, but migrated or misplaced TIPS
can cause complications

68. SUMMARY
• In patients with portal hypertensions the number of
treatment options increased
• These options have greatly decreased the need for
emergency surgery in children with portal
hypertension
• As majority of patients in tropics are of EHPVO, long
term survival of these patients is significantly better
after a shunt surgery than non operated patients

69. SUMMARY
• However, in a cirrhotic with poor liver function
surgery has high mortality, results in high incidence
of encephalopathy and liver failure. In these patients
TIPS, if available, is a good option.

71. Thank you