portal hypertension in children, liver disease in children, hematmasis in children, per oral bleeding, hepatospleenomegaly

1. Dr. Virendra Kumar Gupta
Assistant Professor
Department Of Pediatric Gastroentero-hepatology & Liver
Transplantation
NIMS Medical College & Hospital , Jaipur

2. S.GL.G
Portal venous System

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.

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)

5. Classification of
PHT
 Pre Sinusoidal:Extrahepatic(1)
Intrahepatic(2)
 Sinusoidal(3)
 Post sinusoidal:intrahepatic(4)
extrahepatic(5)
1
2
3
4
5

6. Presinusoidal:
Extrahepatic
Intrahepatic
Portal,splenic vein
thrombosis,cong.malformations
CHF,NCPF
Sinusoidal cirrhosis
Postsinusoidal
Intrahepatic.
Extrahepatic
VOD,Classical BCS
IVC obstruction
Causes of PHT

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

8. • Splenomegaly
• Ascites
• PS Collaterals – abd. veins &
varices
• Hyper dynamic circulation
• Porto Systemic Encephalopathy
I Evaluation of PHT: type and
consequences of PHT

9. II Evaluate: Physical signs of chronic
liver disease

10. III Evaluate: Presence of PSE
•Neuropsychological tests - NCT
•Asterixis
•Foetor Hepaticus

11. • 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

12. 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)
{HVOO- Hepatic Venous outflow Obstruction}

13. Dilated Veins

14. 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

15. • 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

16. Age & PHT
4%
3%
70%
20%
India
Children
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

17. 3
54
6
102
Extra Hepatic Presinusoidal
Sinusoidal Post Sinuso
Post Hepatic
Age & PHT
ICH & HC Chennai, 1999 - 2001
Total Number 165

18. 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%.

19. 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

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

21. 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).

22. 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

23. 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

24. 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

25. 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

26. Causes of EHPVO
 Portal thrombosis:Infections
Umbilical sepsis(10-22%)
Neonatal Sepsis
Intra abd infections
NEC
Acute appendicitis
Peritonitis
Recurrent gut infection.

27. Causes of EHPVO
• Hypercoagulable states
Protein C def
Protein S def
Anti thrombin III def
• Congenital
• Trauma
• Invasion by tumours
• Idiopathic

28. 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.

29. Complications of EHPVO
 Growth retardation.
 Delay in sexual development.
 Ano rectal varices.
 Hypersplenism.
 Portal Biliopathy
 ?chronic liver disease

30. Diagnosis of PHT
 Clinical
 Upper GI Endoscopy
 Ultrasound/Doppler

31. ENDOSCOPY
 Site,Grade
 Predictors of bleed
 Portal hypertensive
gastropathy

32. Ultrasound&Doppler
 Liver size and echogenicity.
 Portal vein-visualization,size
cavernomatous malformation,phasic
variations with respiration.
 Ascites
 Collaterals
 Splenomegaly

33. 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.

34. 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)

35. Measurement of Variceal Pressure
• Direct puncture of varix
• Indirect – pressure gauge or
manometric capsule mounted on
endoscope
High Variceal Pressure–increased risk of Hghe

36. 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

37. Management of variceal bleed
 Pharmacotherapy
 Endosopy-
EST,EVL.
 Surgery

38. Management of EHPVO
 Without bleed
 With Bleed
Acute massive
 Following bleed.

39. 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

40. EHPVO with bleed
 Medical:Resuscitation,vasoactives,vasodila
tors.
Octreotide and somatostatin recommended.
 Endoscopic:Endoscopic sclerotherapy or
Endoscopic variceal ligation.
 Surgical:Devas procedure.Suguira,
Sadasivam,ModifiedTanner

41. EHPVO following bleed.
 Medical:Continue b blockers.
 Endoscopic:regular EST till varices are
sclerosed
 Surgery:Shunt procedures

42. 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.

43. 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.

44. 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.