This document discusses portal hypertension (PHT), including its definition, classification, etiology, pathophysiology, clinical features, diagnosis and management. PHT is defined as a pathological increase in portal pressure above 5 mmHg. It can be classified based on the location of obstruction as pre-sinusoidal, sinusoidal, or post-sinusoidal. The etiology includes pre-hepatic causes such as portal vein thrombosis, intra-hepatic causes like cirrhosis, and post-hepatic causes like Budd-Chiari syndrome. Clinical features include upper GI bleeding, splenomegaly, ascites, and varices. Diagnosis involves clinical evaluation, endoscopy, imaging like ultrasound, and treatment depends

1. DR MAHTAB
MBBS DCH DNB
GMSH 16 CHD

2.  MAJOR CAUSE OF MORBIDITY AND MORTALITY
IN CHILDREN WITH LIVER DISEASE

3. DEFINTION
 NORMAL PORTAL PRESSURE - 7MMHG
 > 10-12MMHG CALLED - PORTAL HYPERTENSION
 OTHER DEF:
INTRA SPLENIC PRESSURE >17MMHG
WEDGE HEPATIC PRESSURE >4MMHG
PHT IS PATHOLOGIC INCRESASE INCREASE IN
PORTAL PRESSURE IN WHICH THE PRESSURE
GRADIENT BETWEEN THE PORTAL VEIN AND IVC
IS INCREASED ABOVE 5MMHG

4.  PORTAL PRESSURE GRADIENT
>6-10 SUB CLINICAL HTN
.>10 VARICES
>12 VARICEAL BLEED,ASCITES

5. ANATOMY OF PORTAL SYSTEM

6. Classification of PHT
 Pre Sinusoidal:Extrahepatic(1)
Intrahepatic(2)
 Sinusoidal(3)
 Post sinusoidal:intrahepatic(4)
extrahepatic(5)
smalathi 6
1
2
3
4
5

7. ETIOLOGY
 CAUSE CAN BE DIVIDED INTO
1. PRE HEPATIC
2. INTRA HEPATIC
3. POST HEPATIC
EXTRA HEPATIC IS IMPORTANT CAUSE OF PHT IN
NEONATES

8. PRE HEPATIC :
 CAUSED BY OBSTRUCTION TO BLOOD FLOW AT
LEVEL OF PORTAL VEIN . IT IS IMPORTANT CAUSE OF
EHPHT IN OUR COUNTRY
 CHILD RECURRENT UPPER GI BLEED HAVE “GOOD
LIVER BAD VEINS”
 DEVELOPMENTAL DEFECT : PORTAL VEIN
AGENESIS,ATRESIA,STENOSIS
 PORTAL VEIN THROMBOSIS (UMBILICAL
INFECTION:OMPHALITIS,INTRA ABDOMINAL
INFECTION:ACUTE APPENDICITIS,PRIMARY
PERITONITIS,SYSTEMIC INFECTION,MAY BE DUE TO
DEHYDRATION.

9.  INTRA LUMINAL TRAUMA :PORTAL VEIN DAMAGE BY
CATHETERIZATION BY EXCHANGE TRANSFUSION
,INFUSION BY SODIUM BICARBONATE
 HYPERCOAGULABLE STATE (DEF OF FACTOR
FIVE:LEIDEN MUTATION,PROTEIN CAND S DEF,ANTI
THROMBIN THREE DEF,
 OTHER MAY PATICULARLY IN ADULT IS OCP
,PREGNENCY,PNH,BLUNT ABDOMEN TRAUMA,POST
OPERATIVE EG POSTSPLENECTOMY,
 50% CHILDREN WITH EHPHT ETIOLOGY IS
UNKNOWN

10. HEPATIC
INTRA HEPATIC PRESINUSOIDAL PHT
 SCHISTOSOMIASIS
 CONGENITAL HEPATIC FIBROSIS
 CHRONIC ACTIVE HEPATITIS
 CHRONIC MYELOID LEUKEMIA
 LYMPHOMA
 SARCOIDOSIS
 LEUKEMIC INFILTRATION
 TOXIN; ARSENIC ,VINYL
CHLORIDE,HYPERVITAMINOSIS A,METHOTREXATE
 TROPICAL SPLENOMEGALY SYNDROME
 PRIMARY BILIARY CIRRHOSIS

11. INTRAHEPATIC SINUSOIDAL PHT
 CIRROHOSIS DUE TO HEPATITIS B AND C
 METABOLIC DISORDER LIKE WILSON
DISEASE,CHOLESTATIC DISORDER LIKE BILIARY
ATRESIA,NEONATAL HEPATITIS
 DRUG LIKE INH ,METHOTRXATE

12. INTRA HEPATIC POST SINUSOIDAL
PHT
 VENO-OCCLUSIVE DISEASE NONTHROMBOTIC
OCCLUSION BY CONNECTIVE TISSUE AND COLLAGEN
OF THE TERMINAL HEPATIC VENOUS
RADICLES,WITHOUT ASSOCIATED ABNORMALITIES
OF HEPATIC VEIN OR INFERIOR VENA CAVA
ETIOLOGY;TOXIN PYRROLIZIDINE
ALKALOID,AFLATOXIN
HERBS;SENECIO,CROTALARIA,CORDIA ALBA
DRUGS; HYPERVITAMINOSIS A,VINCRISTINE,6-
MERCAPTOPURINE,CYCLOPHOSPHAMIDE,BUSULFAN

13. POST HEPATIC
 CAUSED BY OBSTRUCTION TO BLOOD FLOW AT
LEVEL OF HEPATIC VEIN
EXAMPLE :BUDD CHIARI SYNDROME,CHRONIC
HEART FAILURE,CONSTRICTIVE
PERICARDITIS,VENA CAVA WEB

15. PATHOPHYSIOLOGY
 PRIMARY HEMODYNAMIC ABNORMALITIES IS
INCREASED RESISTENCE TO PORTAL BLOOD
FLOW

16. 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

17. 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

18. smalathi 18

19. 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.

20. CLINICAL FEATURE
 UPPER GI BLEEDING AND SPLENOMEGALY ARE
PATHOGNOMIC FEATURE
 OTHER SYMPTOM DEPEND UPON SITE OF
INVOLVEMENT

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

22. Pre hepatic
PHT
1.Upper GIT bleeding
2.pallor
3.Splenomegaly
4.JAUNDICE, (rare)
5.HEPATOMEGALY AND ASCITES (rare)
PRESINUSOIDAL SINUSOIDAL
1.upper GIT bleeding
2.splenomegaly
3.jaundice
4.hepatomegaly
5.ascites
6.hepatic encephalopathy
PORTAL HYPERTENSION
post sinusoidal
post hepatic
abdominal pain with vomiting
massive ascites
tender hepatomegaly
upper GIT bleeding
jaundice
splenomegaly
upper GIT BLEED
the combination of GIT bleed and splenomegaly is pathognomonic of PTH.
left lobe of liver enlarged in intr hepatic PTH and caudate lobe enlarged in
post hepatic PTH

23. Clinical Presentation of GI bleeding
 Hematemesis Vomiting of fresh or old blood
Proximal to Treitz ligament
Bright red blood = significant bleeding
Coffee ground emesis = no active
bleeding
 Melena Passage of black & foul-smelling stools
Usually upper source – may be right
colon
 Hematochezia Passage of bright red blood from rectum
If brisk & significant → UGI source

24. CLINICAL FEATURE OF EHPHT
 UPPER GI BLEED: USUALLY <10YR,MAY BE MASSIVE
BUT HEPATIC ENCEPHALOPATHY DOES NOT
DEVELOP LIVER FUNCTION IS WELL MAINTAIN
 SPLENOMEGALY :MAY PRESENT WITH AN ASYMP
TOMATIC LUMP IN LT HYPOCHONDRIUM
 PALLOR MAY BE DUE TO BLEEDING OR DUE TO
SPLENOMEGALY

25. CLINICAL FEATURE OF INTAHEPATIC
PHT
 UPPER GI BLEED:INITIAL PRESENTATION IN 2/3
PT,CAN AS EARLY AS INFANCY,FROM
OESOPHAGEAL VARICES USUALLY BUT MAY FROM
GASTRIC,DUODENAL,COLONIC VARICES
 HEPATOMEGALY MAY PRESENT IN EARLY
STAGE,FIRM IN
CONSISTENCY,DISPROPORTIONATE ENLARGE
LEFT LOBE ,MAY SHRINK OR SPAN DECREASE
LATER ON

26.  SPLENOMEGALY 2ND MOST FREQUENT
SIGN,MORE COMMON IN MACRONODULAR
THAN MICRO NODULAR CIRRHOSIS .SOME TIME
SPLEEN BECOME IMPALPABLE AFTER MASSIVE
BLEED IT IS DIAGNOSTIC CONFUSION IT IS
CALLED AS SMITH HOWARD SYNDROME,SPLEEN
ARE FIRM TO HARD IN CONSISTENCY
 ASCITES FREQUENT PROBLEM IN SINUSOIDAL
AND POSTSINUSOIDAL PHT USUALLY NOT SEEN
IN PRESINUSOIDAL AND EXTRAHEPATIC PHT

27.  HEPATIC ENCEPHALOPATHY; NEUROPSYCHITRIC
SYNDROME CHARECTERIZED BY ALTERED
CONSIOUSNESS,IMPAIRED INTELLECTUAL
ABILITY AND NEURO MUSCULAR SIGN LIKE
ASTERIXIS
 CAPUT MEDUSA PROMINENT COLLATERAL
VESSEL IN PERIUMBILICAL REGION IN
SINUSOIDAL PHT

28. Pot belly & smiling umbilicus:
smalathi 28

29. Physical signs of chronic liver
disease

30. smalathi 30
•Neuropsychological tests
•Asterixis
•Foetor Hepaticus

31. smalathi 31
• 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
•SOME TIME SPLEEN BECOME IMPALPABLE AFTER MASSIVE
BLEEDING THIS CONDITION IS CALLED AS “SMITH HOWARD
SYNDROME”
Splenomegaly

32. smalathi 32
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)

33. smalathi 33
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

34. smalathi 34
• 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

35. smalathi 35
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%.

36. Diagnosis of PHT
 Clinical
 Upper GI Endoscopy
 Ultrasound/Doppler
smalathi 36

37. Lab Diagnosis :
• CBC with Platelet Count, and Differential
A complete blood count (CBC) is necessary to assess the
level of blood loss. CBC should be checked frequently(q4-6h)
during the first day.
• Hemoglobin Value, Type and Crossmatch Blood
The patient should be cross matched for 2-6 units, based on
the rate of active bleeding. The hemoglobin level should be
monitored serially in order to follow the trend. An unstable
Hb level may signify ongoing hemorrhage requiring further
intervention.

38. • LFT- to detect underlying liver disease
• RFT- to detect underlying renal disease
• Calcium level- to detect hyperparathyroidism
and in monitoring calcium in patients receiving
multiple transfusions of citrated blood
• Gastrin level

39. • The patient's prothrombin time (PT), activated
partial thromboplastin time, and International
Normalized Ratio (INR) should be checked to
document the presence of a coagulopathy

40. • Prolongation of the PT based on an INR of more than
1.5 may indicate moderate liver impairment.
• A fibrinogen level of less than 100 mg/dL also indicates
advanced liver disease with extremely poor synthetic
function

41. ENDOSCOPY
 Site,Grade
 Predictors of bleed
 Portal hypertensive
gastropathy
smalathi 41

42. Imaging :
• CHEST X-RAY-Chest radiographs should be
ordered to exclude aspiration pneumonia, effusion,
and esophageal perforation.
• Abdominal X-RAY- erect and supine films should
be ordered to exclude perforated viscous and ileus.

43. • Computed tomography (CT) scanning and
ultrasonography may be indicated for the
evaluation of liver disease with cirrhosis,
cholecystitis with hemorrhage, pancreatitis with
pseudocyst and hemorrhage, aortoenteric fistula,
and other unusual causes of upper GI hemorrhage.
• Nuclear medicine scans may be useful in
determining the area of active hemorrhage

44. Angiography :
 Angiography may be useful if bleeding persists
and endoscopy fails to identify a bleeding site.
 Angiography along with transcatheter arterial
embolization (TAE) should be considered for all
patients with a known source of arterial UGIB that
does not respond to endoscopic management,
with active bleeding and a negative endoscopy.
 In cases of aortoenteric fistula, angiography
requires active bleeding (1 mL/min) to be
diagnostic.

45. Nasogastric Lavage
A nasogastric tube is an important diagnostic
tool.
This procedure may confirm recent bleeding
(coffee ground appearance), possible active
bleeding (red blood in the aspirate that does not
clear), or a lack of blood in the stomach (active
bleeding less likely but does not exclude an
upper GI lesion).

46. 1. Better visualization during endoscopy
2. Give crude estimation of rapidity of bleeding
3. Prevent the development of Porto systemic
encephalopathy in cirrhosis
4. Increases PH of stomach, and hence, decreases clot
desolation due to gastric acid dilution
5. Tube placement can reduce the patient's need to vomit
 During gastric lavage use saline and not use large volume of
to avoid water intoxication.
 Gastric lavage should be done in alert and cooperative patient
to avoid bronco-pulmonary aspiration
BENEFITS OF LAVAGE :

47. MANAGEMENT

48. Portal hypertension
Asymptomatic Gastrointestinal bleeding
No varices
or low risk
varices
High risk
varices
Monitor for
progression
Prophylac
tic
Blocker/
variceal
obliterizat
ion
Resuscitation
Haemodynamically
stable
Haemodynamically
unstable
Endoscopy
Appropriate
therapy
EST/EVL
with/without
pharmacotherapy
Variceal
source
Bleeding controlled
repeat EST/EVL; chronic
pharmacotherapy
Pharmacotherapy
Bleeding
controlled
EST/EVL
Bleeding
continues,balllloon
tamponade
Bleeding continues,
emergency shunt Sx
Non variceal
source
Endoscopy

49. Management of portal
hypertension
 Therapy of portal hypertension is primarily directed at
the management of variceal hemorrhage.
 Variceal bleeding is a life threatening medical
emergency
 The management can be divided into :
 Emergency therapy
 Primary prophylaxis of the first episode of bleeding
 Secondary prophylaxis of subsequent bleeding
episodes.
 (World Journal of Gastroenterology; 2012 March)

50. Emergency management
 The initial management of variceal bleeding is
stabilization of the patient.
 Vital signs, particularly tachycardia or hypotension,
can be especially helpful in assessing blood loss.
 Fluid resuscitation in the form of crystalloid initially,
followed by red blood cell transfusion, is critical.
 One needs to administer these carefully to avoid
overfilling the intravascular space and increasing
portal pressure.
 Optimal hemoglobin levels in patients with variceal
hemorrhage are between 7 and 9 g/dl

51. EMERGENCY MN CONTINUE
 EVALUATION OF BLOOD LOSS-
 DIPROPORTIONATE TACHYCARDIA IF PR IS
>150/MIN IT SUGGEST PT HS LOSS ABOUT 20% OF
BLOOD
 CAPILLARY REFILL TIME >5 SEC HS LOST ABOUT
25%
 IF PT IS IN SHOCK MEANS HE HS LOSS ABOUT
40%OF BLOOD
 ALL PT HAVE LOSS OF 20-25% BLOOD SHOULD
GIVE OXYGEN TO MAINTAIN SPO2 ≥95%

52. MONITORING OF VITALS
 PULSE,RESPIRATION,BP,SENSORIUM SHOULD BE
MONITOR
 PR MAINTAIN <100,BP >100MM HG
 FLUID INTAKE-OUTPUT CHART SHOULD BE
MONITOR
 IF PT IS IN SHOCK SHOULD BE CATHETERIZE TO
MONITOR URINE OUTPUT
 CVP MONITORING HELP TO GUIDE
REPLACEMENT THERAPY

53. Emergency management contd
 Nasogastric tube placement is safe, allows documentation
of the rate of ongoing bleeding and removal of blood, a
protein source that may precipitate encephalopathy.
 Platelets should be administered for levels less than 50 ×
109/L and coagulopathy corrected with vitamin K and FFP
 Intravenous antibiotic therapy should be considered for all
patients with variceal bleeding due to high risk of
potentially fatal infectious complications.
 Once the patient is stabilized, endoscopy should be
performed

54. Emergency management contd
 Continued bleeding at the time of endoscopy is a
finding that portends poor prognosis.
 Pharmacotherapy of acute hemorrhage should not be
withheld until endoscopy can be performed.
 At the time of initial endoscopy, management can
begin in the form of sclerotherapy or band ligation.
 Bleeding that lasts more than 6 h or requires more
than one red blood cell transfusion necessitates
further investigation.

55. Emergency management-
pharmacotherapy
 The pharmacologic therapy of variceal bleeding
usually consists of vasopressin or somatostatin (or
their analogs) infusions.
 Vasopressin acts by increasing splanchnic vascular
tone and thus decreasing portal blood flow.
 Side effects due to vasoconstriction include left
ventricular failure, bowel ischemia, angina and chest/
abdominal pain.
 Combination it with nitroglycerine which is potent
venous dilater may reduce adverse cardiac effect
 Vasopressin has a half-life of 30 min and is usually
given as a bolus followed by continuous infusion.
 The recommended dose for children is 0.33 U/kg as a
bolus over 20 min, followed by an infusion of 0.2
U/1.73 m2 per minute or .33unit/kg/hr

56. Emergency management-
pharmacotherapy
 Terlipressin, a long-acting synthetic analogue of vasopressin, has shown
similar effects and does not require continuous infusion. In adult it is given in
dose 2mg iv every 6hrly until bleeding stop then 1mg every 6hrly for further
next 24 hr
 Side effects appear to be reduced compared to vasopressin, but data in children
are lacking.
Somatostatin and its synthetic homologue octreotide also have been
shown to decrease splanchnic blood flow with fewer side effects .in adult it is
given in dose of 250microgram iv bolus followed by continous infusion of
250microgram/hr .dose schedule in children need further study
Octreotide - synthetic analogue of somatostatin , appears to be effective but may
need to be initiated by the administration of a bolus. Having long half life a
loading dose of 1 microgram/kg given through iv infusion over 30 min than
.5microgram/kg/hr
 New longer-acting somatostatin analogues are currently under investigation.

57.  Drug to decrease gastric acidity
H2 blocker and ppi

58. Emergency management-
endoscopy Approximately 15% of children will have persistent
hemorrhage despite conservative management
 The most common secondary approach is
endoscopic sclerotherapy or endoscopic band
ligation.
 Endoscopic therapy is very effective although
technically challenging
 Endoscopic sclerotherapy : A variety of sclerosing
agents have been used such as
ethanolamine/tetradecyl sulfate
 These are injected either intra-or para-variceal until
bleeding has stopped.
 Associated with significant incidence of bacteremia,
hence antibiotic prophylaxis is required

60. Emergency management-
endoscopy
Endoscopic band ligation of varices may be a
preferable approach as it is easier and safer.
 Similar control of active bleeding and recurrence of
hemorrhage with significantly lower overall
complications and mortality.
 A potential concern of this technique in children is
entrapment of the full thickness of the esophageal wall
by the rubber band with subsequent ischemic necrosis
and perforation.

62. Emergency management-
mechanical
 The Sengstaken-Blackmore tube (SSBT) stops
hemorrhage by mechanically compressing esophageal and
gastric varices.
 The device consists of a rubber tube with at least two
balloons.
 It is passed into the stomach, where the first balloon is
inflated and pulled up snug against the gastroesophageal
junction.
 Once the tube is secured in place, the second balloon is
inflated in the esophagus at a pressure (60-70 mmHg) that
compresses the varices without necrosing the esophagus.

63. Emergency management-
mechanical
 High incidence(33%-60%) of re-bleeding when the tube is
removed.
 Most patients find the treatment uncomfortable
 Use in children requires significant sedation.
 Increases the risk of aspiration pneumonia, which can be a
life threatening complication
 Hence it is reserved for severe uncontrollable hemorrhage
and generally serves as a temporizing measure until a more
definite procedure can be performed.
 OTHER SIMILAR TUBE IS LINTON NACHOLUS TUBE
HAVING LESS SIDE EFFECT

64. ENDOSCOPIC SCLEROTHERAPY
 BEST T/T FOR VARICEAL BLEEDING AT PRESENT BOTH
IN EMERGENCY AND ELECTIVE PROCEDURES.
 SCLEROSANTS--- ETHALAMINE OLEATE,SODIUM
MORRHUATE,SODIUM TETRA DECYL SULFATE
 COMPLICATION OF EST
1. OESOPHAGEAL ULCERATION OR OESOPHAHEAL
STRICTURE
2. BRONCHO OESHOPHAGEAL FISTULA
3. THROCIC DUCT DAMAGE
4. RECURRENCE OF VARICES
5. THROACIC DUCT DAMAGE
6. ETC

65. surgical and interventional
radiology
 Surgical therapy is usually a last resort and is associated
with high mortality, increased incidence of encephalopathy
and greater difficulty for subsequent liver transplantation.
 The surgical procedures available can be divided into
transection, devascularization, and portosystemic
shunting.
 The first two techniques are rarely used and work by
interrupting blood flow through the esophagus.
 Liver transplantation may be an effective means of
treating esophageal variceal bleeding.
 Variceal embolization via a percutaneous transhepatic or
transsplenic approach has been advocated by some
hepatologists

66. Surgical management-tips
 Transjugular intrahepatic portosystemic shunt (TIPS)
placement may be the optimal approach for intractable
bleeding
 A catheter is inserted into the jugular vein and is advanced
into the hepatic vein where a needle is used to form a tract
between the portal vein and the hepatic vein.
 This tract is expanded with a balloon angioplasty catheter,
and a stent is then placed forming a permanent
portosystemic shunt.
 Though experience in children is limited TIPS may be the
treatment of choice in the emergent setting, espe- cially
when liver transplantation is imminent.

67. Surgical management-
portosystemic shunts
A variety of procedures have been used to divert portal
blood flow and decrease portal blood pressure:
 Portacaval Shunts: formed by side-to-side anastomosis of
the portal vein and the inferior vena cava
 The portacaval shunt diverts nearly all the portal blood
flow into the subhepatic inferior vena cava.
 This is very effective in decompressing the portal system,
 But also diverts a significant amount of blood from its
normal hepatic metabolism, predisposing to the
development of hepatic encephalopathy

68. Surgical management-
portosystemic shunts Mesocaval Shunts: formed with the insertion of a
graft between the superior mesenteric vein and the
inferior vena cava
 This decompresses the portal system while allowing a
greater amount of portal blood flow into the liver.
 The use of grafts unfortunately is associated with
increased risk of thrombosis and many times with
worsening retrograde flow.
 Distal Splenorenal Shunt: formed by end-to-side
anastomosis of the splenic vein and the left renal vein
which can be done nonselectively (central) or
semiselectively (distal splenorenal shunt).

69. Surgical management-
portosystemic shunts
 An alternative shunting procedure for children with
extrahepatic portal vein thrombosis is the Meso-Rex
bypass
 This procedure involves the placement of an autologous
venous graft from the mesenteric vasculature to the left
intrahepatic portal vein.
 One of the major advantages of this approach is the
restoration of normal portal blood flow which eliminates
the risk of hepatic encephalopathy and should preserve
hepatic function.
 Some have advocated that this procedure be considered in
all children with portal vein thrombosis.

70. Surgical management-
portosystemic shunts
 In contrast to the excellent results in portal vein
thrombosis, there are generally poor results of
portosystemic shunts in children with decompensated liver
disease.
 The incidence of recurrent bleeding and death approaches
50%.
 Hepatic encephalopathy is a frequent and serious
complication of portosystemic shunting in decompensated
liver disease
 Overall, surgical portosystemic shunting is an excellent
approach to the long-term management of children with
intractable variceal bleeding in the setting of compensated
cirrhosis.

72. Primary prophylaxis
 The issue of prophylaxis of the first episode of variceal
bleeding in children is controversial
 Beta blocker use in children has reduced the frequency
of bleeding episodes and has improved long-term
survival in patients with esophageal varices.
 A goal of at least 25% reduction in resting heart rate
and HVPG below 12 mm Hg needs to be achieved to
realize these effects which may be problematic in
children.

73. Primary prophylaxis contd
 A wide range of dosing (1-2 mg/kg per day) divided into
two to four doses of propranolol has been required in
children in order to observe a “therapeutic effect”.
 Unfortunately, propranolol often does not reduce
HVPG below 12 mmHg,
 Therefore a combination of beta blockade and
vasodilatation therapies like carvedilol and Isosorbide-5-
mononitrate are now under investigation.
 As with beta blockade, endoscopic band ligation therapy
cannot be recommended for routine use in children with
high risk varices.

74. Secondary prophylaxis
 The long term management of portal hypertension in children with a
previous episode of variceal bleeding is complex.
 The therapeutic modalities include:
 Pharmacologic therapy
 Endoscopic sclerotherapy or band ligation at 2-3 week inteval
SURGERY NEED IN SPITE OF EST
IF PT CONTINUE TO BLEED FROM VARICES,HYPERSPLENISM,PT
HAVING VERY RARE BLOOD GROUP DIFFICULT TO MANAGE
SURGERY INCLUE DECOMPRESSIVE
SHUNT,DEVASCULARISATION,LIVER TRANSPLANT
 Portosystemic shunting procedures
 Some patients might develop end-stage liver disease and ultimately be
candidates for liver transplantation.
(World Journal of Gastroenterology; 2012 March)

75. THANK YOU