Anda di halaman 1dari 62

Portal circulation

and
portal hypertension
in Pediatrics
Overview
• INTRODUCTION
• ANATOMY
• ETIOLOGY/CLASSIFICATION
• CLINICAL MANIFESTATION
• DIAGNOSIS
• TREATMENT
• SUMMARY
• Portal hypertension, defined as an elevation of
portal pressure >10-12 mm Hg(normal value
being 7 mmHg).

 10 mmHg(varices)
 12mmHg(variceal bleed,ascites)
Anatomy
• Dual blood supply
Portal vein 75 % and Hepatic artery 25%

• Portal circulation
• high compliance,
low-resistance
system
• SMA and Splenic vein
• IMA
• Coronary vein
• Umbilical vein
• The portal vein enters the liver at the porta
hepatis in two main branches, one to each
lobe and has a segmental intrahepatic
distribution, accompanying the hepatic artery.
• Portal hypertension occurs as a result of
increased portal resistance, increased portal
blood flow, or both.
Hepatic Venous Pressure Gradient (HVPG)

• To obtain a measurement of the portal pressure gradient, a


catheter can be wedged into the hepatic vein and a wedged
hepatic venous pressure (WHVP) measurement can be
obtained.
• The catheter is then retracted into a free-flowing hepatic vein
and free hepatic venous pressure (FHVP) is measured.
• HVPG = WHVP - FHVP.
• Normal is 1 to 4 mm Hg
Collateral circulation
• There are four main groups of collaterals
formed during intrahepatic obstruction:
Group 1-
• (a)At cardia of stomach: left gastric
vein, posterior gastric and short gastric veins
of the portal system anastomose with the
intercostal, diaphragmo - oesophageal and
azygos minor veins of the caval system.
Collateral circulation
• (b) At anus: superior haemorrhoidal vein of
the portal system anastomoses with the
middle and inferior haemorrhoidal veins of
the caval system.
Group II: paraumblical veins form collaterals
with abdominal wall veins
Group III: where the abdominal organs are in
contact with retroperitoneal tissues or
adherent to the abdominal wall.
Collateral circulation
• Group IV:splenic vein forms collaterals with
left renal vein via diaphragmatic, pancreatic,
left adrenal or gastric veins.
Sites of portal systemic
collateral circulation
Classification

• Prehepatic
• Intrahepatic
• Post-hepatic
EHPVO
• Infections
• Hypercoagulable states
• Trauma
• Invasion and compression
• Intrahepatic causes of PH
• Idiopathic
Intrahepatic Causes of PH
• Noncirrhotic portal fibrosis
– obliterative portal venopathy resulting in PH, well
tolerated episodes of variceal bleeding and preserved
liver function.
• Chronic liver disease
– Cirrhosis and chronic hepatitis.
– The major cause of cirrhosis in children are viral
hepatitis, neonatal cholestasis syndrome and
metabolic liver diseases like Wilson’s disease, glycogen
storage disease, α1 antitrypsin deficiency, etc.
Post sinusoidal block
• Veno-occlusive disease (VOD) or endophlebitis
obliterans
(1) Plant alkaloids,
(2) Irradition,
(3) Drug-immunosuppresants,
antineoplastics and indigenous system of
therapy
• Budd-Chiari syndrome or hepatic venous
outflow tract obstruction (HVOO
• In patients with underlying hepatic disease,
physical examination might show jaundice and
stigmata of cirrhosis such as palmar erythema
and vascular telangiectasias.
EPHVO
• Baveno V workshop consensus statement defined
EHPVO as (De Franchis, 2010):
– EHPVO is defined by obstruction of the extra-hepatic
portal vein with or without involvement of the intra-
hepatic portal veins and does not include isolated
thrombosis of splenic vein or superior mesenteric vein
(SMV).
– EHPVO is characterized by features of recent
thrombosis or of portal hypertension with portal
cavernoma as a sequel of portal vein obstruction
– Presence of cirrhosis and/or malignancy should be
stated.
Epidemiology
• EHPVO is the most common cause of upper
gastrointestinal bleeding in children.
• EHPVO is responsible for 54% of portal
hypertension.
• Most (85-92%) of the upper gastrointestinal
bleeding in Indian children was result of portal
hypertension due to EHPVO
Etiology
• Infection
• Trauma and surgery
• Congenital anomaly
• Prothrombotic state
• Idiopathic
Pathogenesis
• Portal vein changes
– usually the entire length of the portal vein is
occluded with extension into the splenic vein and
sometimes into the superior mesenteric vein.]
– On gross examination, the original portal vein is
difficult to identify as it is usually replaced by a
cluster of variable-sized vessels arranged
haphazardly within a connective tissue support,
called as the Portal Cavernoma.
– intrahepatic venous pressure is normal
– intrasplenic pressure is increased.
– The hepatic blood flow is relatively normal
– hyperkinetic circulatory state
• Liver changes
– liver is normal and the architectural pattern is
preserved.
Clinical presentation
• Recent Or Acute And Chronic
Recent or acute EHPVO
• This can present with abdominal pain, ascites or fever.
• there is no evidence of porto-systemic collaterals and portal
cavernoma.
• The extent of obstruction in portal vein and speed of
evolution of thrombosis predicts the clinical manifestation.
• If associated with infection, features of sepsis may be there.
• may be passed as episodes of acute abdomen or sepsis.
Chronic EHPVO
• typical presenting symptoms like
– episodes of variceal or gastrointestinal bleeding,
– lump in the abdomen and
– hypersplenism.
• EHPVO in childhood is most often chronic and
presents with features of variceal bleeding
and splenomegaly, whereas in adults it could
be either acute or chronic.
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.
Noncirrhotic Portal Fibrosis
• NCPF is not an uncommon entity in pediatric
population usually presenting in the early
second decade.
• Children usually present with splenomegaly
and growth retardation with a small
proportion presenting with well tolerated
episodic variceal bleeding
Clinical Presentation
• GI hemorrhage
• Splenomegaly
• Abdominal Venous Patterning
• Ascites
• Pulmonary Complications
GASTROINTESTINAL BLEEDING
• Gastrointestinal bleeding secondary to
esophageal or gastric varices, presenting as
hematemesis and melena, may be the initial
manifestation of portal hypertension.
• Development of varices requires portal pressure
gradient of at least 10 mm Hg, and a portal
pressure gradient of at least 12 mm Hg is thought
to be required for varices to bleed.
• Variceal hemorrhage may take the form of
hematemesis,hematochezia, melena, or chronic
anemia.
Grading of oesophageal varices
• The severity of the grading is considered to be
a risk factor for bleeding:
– Grade I varices are flattened by insufflation.
– Grade II varices are not flattened by insufflation
and are separated by areas of healthy mucosa.
– Grade III varices are confluent and not flattened
by insufflation.

by the Japanese Research Society for Portal Hypertension


Gastric varices
• Gastric varices are larger in diameter than
esophageal varices and tend to lie deeper within
the submucosa under the gastric mucosa,
whereas esophageal varices are more superficial.
• As with esophageal varices, the risk of bleeding
increases with the size of varices and the
presence of red spots on the varices.
• Gastric varices in continuity with esophageal
varices may regress following treatment of
esophageal varices.
Portal hypertensive gastropathy
• Portal hypertensive gastropathy (PHG) is
characterized by dilation of the mucosal and
submucosal vessels of the stomach.
• This is secondary to increased submucosal and
gastric perfusion and can cause significant
bleeding.
• Rectal bleeding may occur as a result of inferior
mesenteric-internal iliac venous collaterals. One
study demonstrated a prevalence of rectal
hemorrhoids or anorectal varices in about 33% of
children with pediatric portal hypertension.
• Gallbladder varices seem to be more common in
children with portal hypertension from
extrahepatic portal vein obstruction and can be
easily diagnosed by ultrasonography of the biliary
tract
Splenomegaly
• About 10% of the children present with isolated abdominal
mass due to splenomegaly
• EHPVO is associated with splenomagaly and size of spleen
usually increases with age and it may even reach the right iliac
fossa.
• In cases of acute variceal bleeding, the spleen size may reduce
temporarily and reappear on hemodynamic stabilization.
Hyersplenism
• Massive splenomegaly may cause pooling and excessive
destruction of the pooled blood cell components leading to
thrombocytopenia, leucopenia and anemia.
• It can also predispose to infection and or bleeding.
• The hypersplenism does not correlate with splenic size.
• Splenic sequestration crisis may occur leading to hypovolemic
shock and death.
Ascites
• Ascites arises when the hydrostatic and osmotic pressures
within the hepatic and mesenteric capillaries result in a net
transfer from blood vessels to lymphatics at a rate that
overcomes the drainage capacity of the lymphatics.

• It is the presenting sign of portal hypertension in 7%-21 % of


children
• increased sodium retention
• raised portal pressure
• Impaired lymphatic drainage
Hepatopulmonary syndrome (HPS)
• characterized by a partial pressure of O2 less than 70 mm Hg
or an increase in the alveolar-arterial oxygen gradient of
greater than 15 mm Hg associated with portal hypertension
and intrapulmonary vascular dilation
• IPVD normal 8 to 10 μm
• In PH 50 to 500 μm
• Patients may develop complaints of dyspnea and on occasion
orthodeoxia,which is arteriolar oxygen desaturation on
standing
Portal biliopathy
• refers to abnormalities of the extrahepatic and intrahepatic
bile ducts in patients with portal hypertension.
• compression by paracholedochal collaterals on bile ducts
resulting in displacement, narrowing, strictures, angulation,
dilatations and irregularity of bile ducts.
• Extrinsic compression, ischemia, and a combination of both
• may become severe enough to cause obstructive jaundice and
choledocholithiasis and cholelithiasis due to bile stasis.
• ERCP is the definitive method for diagnosis of biliopathy.
Specific issues in children
• Growth retardation
• Mental function and encephalopathy
Growth retardation
• EHPVO in children often reported to be associated with
growth retardation.
– Poor substrate utilization or/and malabsorption due to
portal hypertensive enteropathy
– Impaired synthesis of growth factors due to shunting of
blood away from the liver
– Frequency of bleeding
Mental function and encephalopathy
• Mild cognitive and psychomotor deficit
• generalized low grade cerebral edema
• minimal hepatic encephalopathy
Investigative Approach to PH
1. Assessment of the current hematological
status of the child,
2. Concomitant information on the liver
function and etiology of the liver disease,
3. Demonstration of the site of bleeding
collaterals, patency or block of the portal
vein.
Non-invasive Investigations
• Ultrasound and Doppler
– EPHVO
• echogenic thrombus within the portal veins is dilation
of the vessel proximal to the occlusion,
• lack of identifiable portal vein,
• cavernoma formation around the site of block,
• Doppler - absence of portal venous signal
Ultrasound and Doppler
• Cirrhosis
– Collaterals In Splenic Hilum,
– Hepatomegaly,
– Ascites And
– Splenic Infarct
Other Features On USG
• 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)
• Contrast enhanced CT scan shows the thrombus as non-
enhancing filling defect within the lumen of the portal vein
and dilatation of may small veins at the hilum.

• MRI shows an area of abnormal signal within the lumen of the


portal vein which appears iso-intense on T1-weighted image
with a more intense signal on T2-weighted image.
Invasive Imaging
• Splenoportovenography
• Splenoportovenography
• Percutaneous transhepatic portovenography
Medical and Endoscopic Management of PH in
Children
• Upper gastrointestinal bleeding is a dramatic event and
is associated with loss of large volume of blood.
Variceal bleeding in children differs from adults in
many respects:
i. Predominance of EHPVO (at least 50% more
prevalent than IHPH)
ii. Difficulties to perform shunt surgery.
iii. Apparent development of natural shunts with
growth.
• Patients with EHPVO has good liver function.
• Treatment should be directed to control acute bleeding
and prevention of recurrent bleeding.
Management of variceal bleed
 Pharmacotherapy

 Endosopy-
EST,EVL.

 Surgery

smalathi
Management of EHPVO

• Without bleed

• With Bleed Acute


massive

• Following bleed.
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
EHPVO with bleed

• Medical:Resuscitation,vasoactives,vasodil
a tors.
• Octreotide and somatostatin
recommended.
• Endoscopic:Endoscopic sclerotherapy
or Endoscopic variceal ligation.
EHPVO following bleed.
• Medical:Continue b blockers.

• Endoscopic:regular EST till varices


are sclerosed

• Surgery:Shunt procedures
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.
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.


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.

Anda mungkin juga menyukai