portal hypertension in children.. dr.malathi sathiyasekaran
DESCRIPTION
Portal Hypertension in Children by Dr.Malathi Sathiyasekaran, Pediatric Gastroenterologist, Chennai.TRANSCRIPT
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smalathi 1
Malathi Sathiyasekaran
Pediatric Gastroenterologist.
Chennai
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smalathi 2
S.GL.G
Portal venous System
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smalathi 3
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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smalathi 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 5 mm of Hg.
PPG > 10 mmHg(varices) PPG > 12mmHg(variceal bleed,ascites) PPG > 6 to 10 mmHg(subclinical PHT)
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smalathi 5
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
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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
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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
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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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