portal hypertension in paediatrics

1. By Dr Liza Bulsara
Under Guidance of :-
Dr. Sunil Mhaske sir
(Professor & Head)
In Presence of :-
Dr. Kothari sir (Associate Professor)
And all residents.

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S.GL.G
Portal venous System

3. Portal Hypertension
Normal range of portal venous pressure is 5 to 10 mm
of Hg or 10-15 cm saline above the pressure present in
the IVC.
Portal hypertension is defined as elevation of this
pressure gradient to values above 10 to 12 mm Hg.
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4. Portal Hypertension
Definition:PHT is a pathologic increase in portal
pressure in which the pressure gradient between
the portal vein and the IVC (Portal pressure
gradient or PPG)is increased above the upper
limit of 10 mm of Hg.
PPG > 10 mmHg(varices)
PPG > 12mmHg(variceal bleed,ascites)
PPG > 6 to 10 mmHg(subclinical PHT)
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5. Features Normal
(mm hg)
portal
hypertensio
n
Portal venous
pressure
7-10 >12
Intrasplenic
pressure
<17 >17
Wedge hepatic
pressure
<6 >6
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6. Classification of PHT
Pre Sinusoidal:Extrahepatic(1)
Intrahepatic(2)
Sinusoidal(3)
Post sinusoidal:intrahepatic(4)
extrahepatic(5)
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1
2
3
4
5

7. Pathophysiology of PH
Cirrhosis results in scarring (perisinusoidal
deposition of collagen)
Scarring narrows and compresses hepatic
sinusoids (fibrosis)
Progressive increase in resistance to portal
venous blood flow results in PH
Portal vein thrombosis, or hepatic venous
obstruction also cause PH by increasing the
resistance to portal blood flow

8. Pathophysiology of PH
As pressure increases, blood flow decreases and
the pressure in the portal system is transmitted to
its branches
Results in dilation of venous tributaries
Increased blood flow through collaterals and
subsequently increased venous return cause an
increase in cardiac output and total blood volume
and a decrease in systemic vascular resistance
With progression of disease, blood pressure
usually falls

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10. Portal Vein CollateralsCoronary vein and short gastric veins -> veins of the
lesser curve of the stomach and the esophagus, leading
to the formation of varices
Inferior mesenteric vein -> rectal branches which, when
distended, form hemorrhoids
Umbilical vein ->epigastric venous system around the
umbilicus (caput medusae)
Retroperitoneal collaterals ->gastrointestinal veins
through the bare areas of the liver
Posterior abdominal wall:veins of posterior abdominal
wall,subdiaphragmatic veins,retroperitoneal veins.
Splenorenal collaterals
Splenoperitoneal collaterals.

11. Etiology of PH
 Causes of PH can be divided into
1. Pre-hepatic
2. Intra-hepatic
3. Post-hepatic

12. Pre-hepatic PH
Caused by obstruction to blood flow at the level of
portal vein
Examples: congenital atresia, extrinsic compression,
s, portal, superior mesenteric, or splenic vein
thrombosis,cirrohsis.

13. Intrahepatic:
Infections:cirrohsis due to hepatitis B,C.
Metabolic disorders: wilson’s diseases.
Congenitial hepatic fibrosis
Chronic active hepatitis
Chronic myeloid luekemia
Lymphoma
Sarcoidosis
Luekemic infiltration
Toxins:arsenic,vinyl chloride,hyperviayminosis A.
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14. Post-hepatic
Caused by obstruction to blood flow at the level of
hepatic vein
Examples: Budd-Chiari syndrome, chronic heart
failure, constrictive pericarditis, vena cava webs

15. Budd-Chiari Syndrome
Caused by hepatic venous obstruction
At the level of the inferior vena cava, the hepatic
veins, or the central veins within the liver itself
result of congenital webs , acute or chronic
thrombosis and malignancy

16. Budd-Chiari Syndrome
Acute symptoms include hepatomegaly, RUQ
abdominal pain, nausea, vomiting, ascites
Chronic form present with the sequelae of
cirrhosis and portal hypertension, including
variceal bleeding, ascites, spontaneous bacterial
peritonitis, fatigue, and encephalopathy
Diagnosis is most often made by US evaluation of
the liver and its vasculature
Cross-sectional imaging using contrast-enhanced
CT or MRI

17. Budd-Chiari Syndrome
Gold standard for the diagnosis has been
angiography
Management has traditionally been surgical
intervention (surgical decompression with a side-
to-side portosystemic shunt)
Minimally invasive treatment using TIPS may be
first-line therapy now
Response rates to medical therapy are poor

18. Portal Vein Thrombosis
Most common cause in children (fewer than 10%
of adult pts.)
Normal liver function and not as susceptible to
the development of complications, such as
encephalopathy
Diagnosis by sonography, CT and MRI
Often, the initial manifestation of portal vein
thrombosis is variceal bleeding in a noncirrhotic
patient with normal liver function

19. Portal Vein Thrombosis -
Causes
Umbilical vein infection (the most common cause
in children)
Coagulopathies (protein C and antithrombin III
deficiency),
Hepatic malignancy, myeloproliferative disorders
Inflammatory bowel disease
pancreatitis
trauma
Most cases in adults are idiopathic

20. Portal Vein Thrombosis
Therapeutic options are esophageal variceal ligation
and sclerotherapy
Distal splenorenal shunt
Rex shunt in patients whose intrahepatic portal vein
is patent (most commonly children)

21. Splenic Vein Thrombosis
Most often caused by disorders of the pancreas
(acute and chronic pancreatitis, trauma,
pancreatic malignancy, and pseudocysts)
Related to the location of the splenic vein
Gastric varices are present in 80% of patients
Occurs in the setting of normal liver function
Readily cured with splenectomy (variceal
hemorrhage), although observation for
asymptomatic patients is acceptable.

22. Clinical Evaluation
• Splenomegaly • Abdominal veins •Ascites
• Varices

23. Ascites:
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24. Pot belly & smiling umbilicus:
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25. Distended engorged veins:
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• Splenomegaly
• Ascites
• PS Collaterals – abd. veins &
varices
• Hyper dynamic circulation
• Porto Systemic Encephalopathy
I Evaluation of PHT: type and
consequences of PHT

27. II Evaluate: Physical signs of chronic
liver disease

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III Evaluate: Presence of PSE
•Neuropsychological tests
•Asterixis
•Foetor Hepaticus

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• An enlarged spleen is the single most
important diagnostic sign of PHT
•Does not correlate with height of portal
pressure, size of varices or age of pt.
•Correlates with type of PHT (*
NCPF
12cm, * *
EHPVO 6cm)
•Spleen may not be palpable soon after a
bleed
Splenomegaly

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Dilated Abdominal Veins
• Presence supports the diagnosis of PHT
(Cirrhosis, BCS)
• Absence does not exclude PHT (EHPVO)
• Periumbilical veins indicate intrahep PHT,
(murmur – Cruvellier Baumgarten)
• Back veins – indicates HVOO (Classical
BCS/IVC)

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Jean cruhveiller & paul caumen von baumgarten:

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Dilated Veins

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Ascites
• Presence of ascites supports diag of PHT
• Present in sinusoidal/post-sinusoidal
• Sudden accumulation of ascites – HVOO
• “Frog belly” – IVC obstruction
• Ascites in EHPVO (0-36%),
NCPF (5-10%) transient

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• Consistency more significant than size
• Size correlates poorly with height of pp.
• Normal, soft or small liver EHPVO
• Firm, nodular ,↓ vertical span or
enlarged,L .lobe palpable- cirrhosis
• Left lobe liver enlarged - CHF
• Firm liver – NCPF (10-15% nodular)
Liver Size & Consistency

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Presentation:GI Bleed
• GI Bleed usually is the first
presentation in EHPVO/NCPF.
• Bleeds well tolerated in presinusoidal
PHT.
• Bleeds occur night / morning (Peaks at 10
P.M, 9A.M).
• Mortality following variceal bleed in
cirrhosis 20% to 30%.

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Porto Systemic Hepatic
Encephalopathy
• Minimal Encephalopathy (>50%)
• Recurrent
• Persistent
• Acute
All 4 forms seen in cirrhosis. In NCPF /
EHPVO, this may follow GI bleed but
majority recover

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• Hypersplenism
• Thrombocytopenia - NCPF > EHPVO >
Cirrhosis
• Anemia
• Anemia could also be secondary to GI
Bleed
Hematological changes

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Clinical Features
• Growth Retardation – Resistance to the action
of growth hormone (EHPVO)
• Portopulmonary hypertension – non-
embolic pulmonary vasoconstriction in the
presence of PHT. (4% of cirrhosis, 9% of NCPF))
.
• Hepatorenal syndrome – renal insufficiency
in patients with severe liver failure in the absence
of any other cause of renal pathology (cirrhosis).

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Clinical Features
•Hepato pulmonary syndrome – triad
of PHT, intrapulmonary vascular dilatation and
arterial hypoxemia (PaO2 < 70mm of Hg) In the
absence of primary cardio pulmonary disease.
(17.5% cirrhotics, 13.3% NCPF, 10% EHPVO)
Anand A C IJ Gastro 2001.
•Foetor Hepaticus – results from porto
systemic shunting of blood, allows mercaptans
to pass directly to the lungs.
•Portal Biliopathy

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Evaluation of various forms of portal
hypertension
Parameter EHPVO NCPF Cirrhosis HVOO
Mean age
(years)
Children
& occ.
adults
18-25 All ages All ages
GI Bleed ++ Well
tolerated
++ Well
tolerated
+ + / -
Ascites 5% - 10% 5% - 10% + + + + +
Pedal oedema - - ++ +++
Encephalopathy - - + + / -
Spleen + + + ++ + +
Liver Normal or
Small
volume
Firm Decreased
vol / firm /
nodular
Enlarged /
firm /
nodular

41. Anterior
Abdomin
al Veins
- / few veins on
lumbar region
+ / - ++ + + + Back
vein
T. Protein
A/G ratio
Normal Normal T.P decreased
Glob increased
T P decreased
Glob increased
(Chronic)
US PV thrombosis
Cavernoma
Collaterals
Splenomegaly
Patent dilated PV
splenomegaly
collaterals
Liver coarse
echoes
Collaterals
dilated PV
ascites
Splenomegaly
Liver enlarged
Hepatic vein
thrombosis or
IVC obstruction
Liver
biopsy
Normal Normal / Peri
Portal fibrosis
Necrosis,
nodules
fibrosis
Centrilobular
necrosis,
fibrosis
Reversed
lobulation
Features EHPVO NCPF CIRRHOSIS HVOO

42. Diagnosis of PHT
Clinical
Upper GI Endoscopy
Ultrasound/Doppler
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43. Complications of PH
GI bleeding due to gastric and esophageal varices
Ascites
Hepatic encephalopathy

44. Varices
Most life threatening complication is bleeding from
esophageal varices
Distal 5 cm of esophagus
Usually the portal vein-hepatic vein pressure gradient
>12 mm Hg
Bleeding occurs in 25-35% of pts. With varices and
risk is highest in 1st yr.

45. Prevention of Varices
Primary prophylaxis: prevent 1st episode of bleeding
Secondary prophylaxis: prevent recurrent episodes of
bleeding
Include control of underlying cause of cirrhosis and
pharmacological, surgical interventions to lower
portal pressure

46. Prevention of Varices
Beta blockade: Beta blockade (Nadolol, Propranolol)
Nitrates:Organic nitrates
Surgery: No longer performed*
Endoscopy: Sclerotherapy (no longer used*
) and
variceal ligation
* Refers to primary prophylaxis

47. Treatment of Varices
 Initial Management:
1. Airway control
2. Hemodynamic monitoring
3. Placement of large bore IV lines
4. Full lab investigation (Hct, Coags, LFTs,)
5. Administration of blood products
6. ICU monitoring

48. Pharmacologic Treatment of
Varices
Decreases the rate of bleeding
Enhances the endoscopic ability to visualize the
site of bleeding
Vasopressin - potent splanchnic vasoconstrictor;
decreases portal venous blood flow and pressure
Somatostatin: decrease splanchnic blood flow
indirectly; fewer side effects
Octreotide: Initial drug of choice for acute
variceal bleeding

49. Endoscopic Therapy for Varices
Endoscopic Sclerotherapy: complications occur in 10-
30% and include fever, retrosternal chest pain,
dysphagia, perforation
Endoscopic variceal ligation: becoming the initial
intervention of choice; success rates range from 80-
100%

50. Balloon Tamponade
Sengstaken-Blakemore tube
Minnesota tube
Alternative therapy for pts. who fail pharmacologic or
endoscopic therapy
Complications: aspiration, perforation, necrosis
Limited to 24 hrs; works in 70-80%

51. TIPS
Transjugular intrahepatic portasystemic shunt
1st line treatment for bleeding esophageal
varices when earlier-mentioned methods fail
Success rates 90-100%
Significant complication is hepatic
encephalopathy

52. Surgical Intervention
Liver transplantation: only definitive procedure for
PH caused by cirrhosis
Shunts
Totally diverting (end-side portacaval)
Partially diverting (side-side portacaval)
Selective (distal splenorenal shunt)
Devascularization