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Malathi Sathiyasekaran

Pediatric Gastroenterologist.

Chennai

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S.GL.G

Portal venous System

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Portal Hypertension

Normal range of portal venous pressure is 5 to 10 mm of Hg 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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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 5 mm of Hg.

PPG > 10 mmHg(varices) PPG > 12mmHg(variceal bleed,ascites) PPG > 6 to 10 mmHg(subclinical PHT)

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Classification of PHT

Pre Sinusoidal:Extrahepatic(1)

Intrahepatic(2)

Sinusoidal(3)

Post sinusoidal:intrahepatic(4)

extrahepatic(5)

1

234

5

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Presinusoidal:Extrahepatic

Intrahepatic

Portal,splenic vein thrombosis,cong.malformations

CHF,NCPF

Sinusoidal cirrhosis

PostsinusoidalIntrahepatic.Extrahepatic

VOD,Classical BCS

IVC obstruction

Causes of PHT

Clinical Evaluation

• Splenomegaly • Abdominal veins •Ascites

• Varices

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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

II Evaluate: Physical signs of chronic liver disease

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III Evaluate: Presence of PSE•Neuropsychological tests - NCT

•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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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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Age & PHT

4%3%

70%

20%

IndiaChildren Adults

West - Children

64%

17%

7%12%

EHPVO

Cirrhosis

NCPF

BCS

CHF

10%

72%

18%

Anand A C et al.Yachha et al

Goncalves M E

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3

54

6

102

Extra Hepatic PresinusoidalSinusoidal Post SinusoPost Hepatic

Age & PHT

ICH & HC Chennai, 1999 - 2001ICH & HC Chennai, 1999 - 2001

Total Number 165

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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 10.44P.M, 9.12A.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))Binay K De IJ Gastro

1997.

• 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

Anterior Abdominal 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 CollateralsSplenomegaly

Patent dilated PV splenomegaly collaterals

Liver coarse echoes Collaterals dilated PVascitesSplenomegaly

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

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EHPVO

Most common cause of PHT in children in India.

Usual presentation is UGIB(>80%) Asymptomatic splenomegaly(<10%) Pain LHC. Age of presentation :4-7 yrs. Triggered by respiratory infection

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Causes of EHPVO

Portal thrombosis:Infections

Umbilical sepsis(10-22%)

Neonatal Sepsis

Intra abd infections

NEC

Acute appendicitis

Peritonitis

Recurrent gut infection.

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Causes of EHPVO

• Hypercoagulable states

Protein C def

Protein S def

Anti thrombin III def

• Congenital

• Trauma

• Invasion by tumours

• Idiopathic

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Natural history of EHPVO

Recurrent well tolerated major GI bleeds.

Occasionally minor bleeds presenting as occult blood loss.

Frequency of bleed decreases as child grows older especially after puberty.

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Complications of EHPVO

Growth retardation. Delay in sexual development. Ano rectal varices. Hypersplenism. Portal Biliopathy ?chronic liver disease

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Diagnosis of PHT

Clinical

Upper GI Endoscopy

Ultrasound/Doppler

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ENDOSCOPY

Site,Grade

Predictors of bleed

Portal hypertensive

gastropathy

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Ultrasound&Doppler

Liver size and echogenicity. Portal vein-visualization,size

cavernomatous malformation,phasic

variations with respiration. Ascites Collaterals Splenomegaly

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Ultrasound and Doppler

Liver echotexture: Cirrhosis: coarse EHPVO:Normal Portal vein: Cirrhosis:Portal Vein dilated>10mm EHPVO:portal vein not visualised,replaced

by a fibrous cord,cavernomatous malformation.

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Other features on US

Variation of splenic and SMV diameter with respiration:Normally increases but not in PHT

Thickness of lesser omentum:Ratio of omental thickness to diameter of aorta >1.7 in PHT

Direction of portal flow:Normal is hepato petal in severe cirrhosis it may be hepato fugal.

Presence of collaterals:Gastric,lieno renal.(left renal vein may appear wider)

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Measurement of Variceal Pressure

• Direct puncture of varix

• Indirect – pressure gauge or manometric capsule mounted on endoscope

High Variceal Pressure–increased risk of Hghe

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Hepatic Venous Pressure Gradient

HVPG

WHVP – FHVP = HVPG

• HVPG 5-7mmHg

• WHVP – Pressure distal to inflated balloon in hepatic venous radicle

• FHVP – Pressure after deflation

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Management of variceal bleed

Pharmacotherapy

Endosopy-EST,EVL.

Surgery

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Management of EHPVO

Without bleed

With Bleed Acute massive

Following bleed.

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EHPVO :without bleed.

Medical:Primary prophylaxis:Non selective B blockers 1 mg/kg/day.

Endoscopic:EVL if there are predictors of bleed(Grade II or IV varices,daughter varices,CR spots,hemocystic spots)No EST

Surgical:No role unless child presents with massive splenomegaly and hyper splenism

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EHPVO with bleed

Medical:Resuscitation,vasoactives,vasodilators.

Octreotide and somatostatin recommended. Endoscopic:Endoscopic sclerotherapy or

Endoscopic variceal ligation. Surgical:Devas procedure.Suguira,

Sadasivam,ModifiedTanner

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EHPVO following bleed.

Medical:Continue b blockers.

Endoscopic:regular EST till varices are sclerosed

Surgery:Shunt procedures

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Indications for surgery in EHPVO

Devascularisation and decompression surgeries

Failure of EST. Hypersplenism. Child living in remote areas or with rare

blood groups. ?Growth retardation.

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Reasons for not advocating early surgery in EHPVO

Natural history of the disease.

Veins may be too thin for good anastomosis.

Chance of thrombosis at operated site.

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TIPSS

Trans jugular Intra hepatic Porto Systemic Shunt

Helps in resistant ascites ,massive bleeds.

Bridge before liver Tx.

HVPG,Liver Bx can also be done at the same time.

Cannot be done in EHPVO.

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