J E J Krige, I
The portal vein
carries about 1500 ml/min of blood from the small and large
bowel, spleen, and stomach to the liver at a pressure
of 5-10 mm Hg. Any obstruction or increased resistance to flow
or, rarely, pathological increases in portal blood flow
may lead to portal hypertension with portal pressures
over 12 mm Hg. Although the differential diagnosis is
extensive, alcoholic and viral cirrhosis are the
leading causes of portal hypertension in Western countries,
whereas liver disease due to schistosomiasis is the main
cause in other areas of the world. Portal vein
thrombosis is the commonest cause in children.
portal pressure cause development of a portosystemic collateral
circulation with resultant compensatory portosystemic
shunting and disturbed intrahepatic circulation. These factors
are partly responsible for the important complications of
chronic liver disease, including variceal bleeding,
hepatic encephalopathy, ascites, hepatorenal
syndrome, recurrent infection, and abnormalities in
coagulation. Variceal bleeding is the most serious complication
and is an important cause of death in patients with
cirrhotic liver disease.
Causes of portal
resistance to flow
Prehepatic (portal vein obstruction)
atresia or stenosis
- Thrombosis of
- Thrombosis of
compression (for example, tumours)
- Acute alcoholic
- Idiopathic portal
hypertension (hepatoportal sclerosis)
portal blood flow
- Increased splenic
countries variceal bleeding accounts for about 7% of episodes of
gastrointestinal bleeding, although this varies
according to the prevalence of alcohol related liver disease
(11% in the United States, 5% in the United Kingdom).
Patients with varices have a 30% lifetime risk of
bleeding, and a third of those who bleed will die.
Patients who have bled once from oesophageal varices
have a 70% chance of bleeding again, and about a third
of further bleeding episodes are fatal.
important considerations influence choice of treatment and
prognosis. These include the natural course of the disease
causing portal hypertension, location of the bleeding
varices, residual hepatic function, presence of
associated systemic disease, continuing drug or
alcohol misuse, and response to specific treatment.
The modified Child-Pugh classification identifies three risk
categories that correlate well with
Prompt resuscitation and restoration of circulating
blood volume is vital and should precede any diagnostic studies.
While their blood is being cross matched, patients should
receive a rapid infusion of 5% dextrose and colloid
solution until blood pressure is restored and urine
output is adequate. Saline infusions may aggravate
ascites and must be avoided. Patients who are haemodynamically
unstable, elderly, or have concomitant cardiac or
pulmonary disease should be monitored by using a
pulmonary artery catheter as injudicious
administration of crystalloids, combined with vasoactive drugs,
can lead to the rapid onset of oedema, ascites, and
hyponatraemia. Concentrations of clotting factors are
often low, and fresh blood, fresh frozen plasma, and
vitamin K1 (phytomenadione) should be
given. Platelet transfusions may be necessary. Sedatives should
be avoided, although haloperidol is useful in patients
with symptoms of alcohol withdrawal.
classification of liver failure
No of points
Moderate to severe
Moderate to severe
points, grade B=7-9 points, grade C=10-15 points.
Drug treatment, aimed at controlling the acute bleed
and facilitating diagnostic endoscopy and emergency
sclerotherapy, may be useful when variceal bleeding
is rapid. Octreotide, a synthetic somatostatin
analogue, reduces splanchnic blood flow when given
intravenously as a constant infusion (50 µg/h) and can be used
before endoscopy in patients with active bleeding.
Vasopressin (0.4 units/min), or the long acting
synthetic analogue terlipressin, combined with
glyceryl trinitrate administered intravenously or
transdermally through a skin patch is also effective but has
more side effects than octreotide. Glyceryl
trinitrate reduces the peripheral vasoconstriction
caused by vasopressin and has an additive effect in
lowering portal pressure.
diagnostic fibreoptic endoscopy is essential to confirm that
oesophageal varices are present and are the source of
bleeding. Most patients will have stopped bleeding spontaneously
before endoscopy (60% of bleeds) or after drug treatment.
Endotracheal intubation may be necessary during
endoscopy, especially in patients who are bleeding
heavily, encephalopathic, or unstable despite
vigorous resuscitation. In 90% of patients variceal bleeding
originates from oesophageal varices. These are
treated by injection with sclerosant or by banding.
In sclerotherapy a sclerosant solution (ethanolamine
oleate or sodium tetradecyl sulphate) is injected into the
bleeding varix or the overlying submucosa. Injection
into the varix obliterates the lumen by thrombosis
whereas injection into the submucosa produces
inflammation followed by fibrosis. The first injection controls
bleeding in 80% of cases. If bleeding recurs, the
injection is repeated. Complications are related to
toxicity of the sclerosant and include transient
fever, dysphagia and chest pain, ulceration,
stricture, and (rarely) perforation.
Band ligation is achieved by a banding device
attached to the tip of the endoscope. The varix is aspirated
into the banding chamber, and a trip wire dislodges a
rubber band carried on the banding chamber, ligating
the entrapped varix. One to three bands are applied
to each varix, resulting in thrombosis. Band ligation
eradicates oesophageal varices with fewer treatment sessions
and complications than sclerotherapy.
The balloon tube tamponade may be life saving in
patients with active variceal bleeding if emergency
sclerotherapy or banding is unavailable or not
technically possible because visibility is obscured.
In patients with active bleeding, an endotracheal
tube should be inserted to protect the airway before attempting
to place the oesophageal balloon tube.
balloon tube has four lumens, one for gastric aspiration, two to
inflate the gastric and oesophageal balloons, and one
above the oesophageal balloon for suction of secretions
to prevent aspiration. The tube is inserted through the
mouth, and correct siting within the stomach is
checked by auscultation while injecting air through
the gastric lumen. The gastric balloon is then
inflated with 200 ml of air. Once fully inflated, the
gastric balloon is pulled up against the oesophagogastric
junction, compressing the submucosal varices. The
tension is maintained by strapping a split tennis
ball to the tube at the patient's mouth.
balloon is rarely required. The main complications are gastric
and oesophageal ulceration, aspiration pneumonia, and
oesophageal perforation. Continued bleeding during balloon
tamponade indicates an incorrectly positioned tube or
bleeding from another source. After resuscitation,
and within 12 hours, the tube is removed and
endoscopic treatment repeated.
intrahepatic portosystemic shunt
Transjugular intrahepatic portosystemic shunt is the
best procedure for patients whose bleeding is not controlled
by endoscopy. It is effective only in portal hypertension
of hepatic origin. The procedure is performed via the
internal jugular vein under local anaesthesia with
sedation. The hepatic vein is cannulated and a tract
created through the liver parenchyma from the hepatic
to the portal vein, with a needle under ultrasonographic and
fluoroscopic guidance. The tract is dilated and an
expandable metal stent inserted to create an
intrahepatic portosystemic shunt. The success rate is
excellent. Haemodynamic effects are similar to those found
with surgical shunts, with a lower procedural morbidity
intrahepatic portosystemic shunting is an effective salvage
procedure for stopping acute variceal haemorrhage,
controlling bleeding from gastric varices, and congestive
gastropathy after failure of medical and endoscopic
treatment. However, because encephalopathy occurs in
up to 25% of cases and up to 50% of shunts may
occlude by one year, its primary role is to rescue failed
endoscopy or as a bridge to subsequent liver
After the acute
variceal haemorrhage has been controlled, treatment should be
initiated to prevent rebleeding, which occurs in most
Repeated endoscopic treatment eradicates oesophageal
varices in most patients, and provided that follow up is
adequate serious recurrent variceal bleeding is
uncommon. Because the underlying portal hypertension
persists, patients remain at risk of developing
recurrent varices and therefore require lifelong regular
Options for long
- Long term
- Surgical shunt
The use of blockers after variceal bleeding has been
shown to reduce portal blood pressures and lower the
risk of further variceal bleeding. All patients should take
blockers unless they have contraindications. Best results
are obtained when portal blood pressure is reduced by
more than 20% of baseline or to below 12 mm Hg.
Patients with good liver function in whom endoscopic
management fails or who live far from centres where
endoscopic sclerotherapy services are available are candidates
for surgical shunt procedures. A successful portosystemic
shunt prevents recurrent variceal bleeding but is a
major operation that may cause further impairment of
liver function. Partial portacaval shunts with 8 mm
interposition grafts are equally effective to other
shunts in preventing rebleeding and have a low rate of
transection and gastric devascularisation are now rarely
performed but have a role in patients with portal and
splenic vein thrombosis who are unsuitable for shunt procedures
and continue to have serious variceal bleeding despite
endoscopic and drug treatment.
transplantation is the treatment of choice in advanced liver
disease. Hepatic decompensation is the ultimate decompressive
shunt for portal hypertension and also restores liver
function. Transplantation treats other complications
of portal hypertension and has one year and five year
survival rates of 80% and 60% respectively.
with portal hypertension never bleed, and it is difficult to
predict who will. Attempts at identifying patients at
high risk of variceal haemorrhage by measuring the size or
appearance of varices have been largely unsuccessful.
Beta blockers have been shown to reduce the risk of
bleeding, and all patients with varices should take
them unless contraindicated.
- Variceal bleeding
is an important cause of death in cirrhotic patients
- Acute management
consists of resuscitation and control of bleeding by
sclerotherapy or balloon tamponade
- After a bleed
patients require treatment to eradicate varices and lifelong
surveillance to prevent further bleeds
- All patients with
varices should take Beta blockers to reduce the risk of
bleeding unless contraindicated by coexisting medical
- Surgery is now
rarely required for acute or chronic control of variceal
are the source of bleeding in 5-10% of patients with variceal
haemorrhage. Higher rates are reported in patients
with left sided portal hypertension due to thrombosis of the
splenic vein. Endoscopic control of gastric varices
is difficult unless they are located on the proximal
lesser curve in continuation with oesophageal
varices. Endoscopic administration of cyanoacrylate
monomer (superglue) is useful for gastric varices. The
transjugular intrahepatic portosystemic shunt is
increasingly used to control bleeding in this group.
portal hypertensive gastropathy accounts for 2-3% of bleeding
episodes in cirrhosis. Although serious bleeding from
these sources is uncommon, when it occurs its diffuse nature
precludes the use of endoscopic treatment, and optimal
management is with a combination of terlipressin and
Bleeding Management Procedures
portal hypertension and variceal bleeding?
The variceal bleeding you
have had is caused by portal hypertension. Portal hypertension
is an increase in the pressure within the portal vein (the vein
that carries blood from the digestive organs to the liver). This
increase in pressure is caused by a blockage in the blood flow
throughout the liver.
pressure in the portal vein causes large veins (varices) to
develop across the esophagus and stomach to bypass the blockage.
The varices become fragile and can bleed easily. Symptoms of
portal hypertension include:
- Bleeding -- black
stools and/or vomiting of blood due to the spontaneous
rupture and hemorrhage from varices
- Ascites -- an
accumulation of fluid in the abdomen
- Encephalopathy --
confusion and forgetfulness caused by poor liver function
and the diversion of blood flow away from your liver
studies and lab work confirm that you have variceal bleeding.
Further treatment is necessary to reduce the risk of rebleeding.
variceal bleeding treated?
Once the bleeding episode
has been stabilized, treatment options are prescribed based on
the severity of your symptoms and how well your liver is
When you were first
diagnosed with variceal bleeding, you may have been treated with
endoscopic therapy and/or medications. Endoscopic therapy
consists of either sclerotherapy or banding. Medications such as
beta blockers or nitrates may have been prescribed alone or in
combination with endoscopic therapy to reduce the pressure in
your varices and further reduce the risk of rebleeding.
first level of treatment has not successfully controlled your
variceal bleeding, you now require decompression (reducing the
pressure) of your varices.
Two procedures for
Intrahepatic Porto systemic Shunt (TIPS) -- a radiological
procedure in which a stent (a tubular device) is placed in
the middle of the liver to reroute the blood flow
Splenorenal Shunt (DSRS) -- a surgical procedure that
connects the splenic vein to the left kidney vein
comparing the TIPS and DSRS procedures
By decreasing the pressure
(decompression) in your veins, either the TIPS or DSRS procedure
can provide control of your bleeding. There are advantages and
disadvantages of both procedures. Neither procedure has been
proven to be a better treatment for long-term control of
bleeding, or for the overall management of patients with
cirrhosis and portal hypertension. A study directly comparing
both procedures is now being funded by the National Institute of
Health (NIH). This study is being conducted at several medical
endoscopic therapy and medications are no longer controlling
your bleeding, we encourage you to participate in this study.
What is the
During the TIPS procedure,
a radiologist makes a tunnel through the liver with a needle,
connecting the portal vein (the vein that carries blood from the
digestive organs to the liver) to one of the hepatic veins (the
three veins that carry blood from the liver). A metal stent is
placed in this tunnel to keep the track open.
procedure reroutes blood flow in the liver and reduces pressure
in all abnormal veins, not only in the stomach and esophagus,
but also in the bowel and the liver.
procedure is not a surgical procedure -- the radiologist
performs the procedure within the vessels in the X-ray room
under X-ray guidance. The procedure lasts 1 to 3 hours. You
should expect to stay in the hospital 2 to 3 days after the
procedure controls bleeding immediately in over 90% of patients,
but has a late rebleeding rate of about 20% because the shunt
What are the
potential complications of the TIPS procedure?
- Shunt narrowing
or occlusion (blockage) -- this could happen within the
first year after the procedure. Follow-up ultrasounds are
performed frequently after the TIPS procedure to detect
these complications. The signs of occlusion include
increased ascites or rebleeding. This condition can be
treated by a radiologist who re-expands the shunt with a
balloon or repeats the procedure to place a new stent.
- Encephalopathy --
mental changes caused by abnormal functioning of the brain
that occur with severe liver disease. Encephalopathy can be
worse when blood flow to the liver is reduced by TIPS, which
may result in toxic substances reaching the brain without
being metabolized first by the liver. This condition can be
treated with medications, diet or by revising the shunt.
What is the
The DSRS is a surgical
procedure. During the surgery, the vein from the spleen (called
the splenic vein) is detached from the portal vein and
reattached to the left kidney (renal) vein. This surgery
selectively reduces the pressure in your varices and controls
the bleeding. Figure 3 illustrates the distal splenorenal
anesthetic is given to you before the surgery. The surgery lasts
about 4 hours. You should expect to stay in the hospital from 7
to 10 days. DSRS controls bleeding in over 90% of patients, with
the highest risk of any rebleeding in the first month. However,
the DSRS procedure provides good long-term control of bleeding.
What is the
potential complication of the DSRS surgery?
Ascites -- an accumulation
of fluid in the abdomen. This condition can be treated with
medications called diuretics and restricted sodium intake.
are required before the TIPS and DSRS procedures?
Before these procedures,
you will have had the following tests to determine the extent
and severity of your portal hypertension condition:
- Evaluation of
your medical history
- A physical
- Blood tests
- Galactose liver
the TIPS or DSRS procedure, your physician may ask you to come
to the Clinic for pre-operative tests. The tests may include an
electrocardiogram (also called an EKG), chest x-ray or
additional blood tests. If your physician thinks you will need
additional blood products (such as plasma), they will be ordered
at this time.
medical care for both procedures
- Ten days after
your hospital discharge date, you will meet with the surgeon
or hepatologist and nurse coordinator to evaluate your
progress. Lab work will be done at this time.
- Six weeks after
the TIPS procedure (and again 3 months after the procedure),
you will have an ultrasound so your physician can check that
the shunt is functioning properly. You will have an
angiogram only if the ultrasound indicates that there is a
problem. You will also have lab work done at these times and
visit the surgeon or hepatologist and nurse coordinator.
- Six weeks after
the DSRS procedure (and again 3 months after the procedure),
you will meet with the surgeon and nurse coordinator to
evaluate your progress. Lab work will be done at this time.
- Six months after
either the TIPS or DSRS procedure, you will have an
ultrasound to make sure the shunt is working properly. You
will also visit the surgeon or hepatologist and nurse
coordinator to evaluate your progress. Lab work and a
galactose liver function test will also be done at
- Twelve months
after either procedure, you will have another ultrasound of
the shunt. You will also have an angiogram so your physician
can check the pressure within your veins across the shunt.
You will meet with the surgeon or hepatologist and the nurse
coordinator. Lab work and a galactose liver function test
will be done at this time.
- If the shunt is
working well, every 6 months after the first year of
follow-up appointments you will have an ultrasound, lab work
and you will visit with your physician and nurse
- More frequent
follow-up visits may be necessary, depending on your
What do I
need to do to maintain my health after these procedures?
- Attend all
follow-up appointments, as scheduled, to ensure that the
shunt is properly functioning.
- Be sure to follow
the dietary recommendations provided by your health care