OVERVIEW OF DISEASE
Cirrhosis results from diff rent mechanisms of liver injury that lead to necro-
inflammation and fibro genesis; histologically it is characterized by diff use nodular
regeneration surrounded by dense fibrotic septa with subsequent parenchymal extinction
and collapse of liver structures, together causing pronounced distortion of hepatic
vascular architecture.This distortion results in increased resistance to portal blood flow
and hence in portal hypertension and in hepatic synthetic dysfunction. Clinically,
cirrhosis has been regarded as an end-stage disease that invariably leads to death, unless
liver transplantation is done, and the only preventive strategies have been screening for
esophageal varices and hepatocellular carcinoma.
Cirrhosis is an increasing cause of morbidity and mortality in more developed countries.
It is the 14th most common cause of death in adults worldwide but the fourth in central
Europe; it results in 1·03 million deaths per year worldwide 8 170 000 per year in
Europe, and 33 539 per year in the USA.Cirrhosis is the main
indication for 5500 liver transplants each year in Europe.The main causes in more
developed countries are infection with hepatitis C virus, alcohol misuse, and,
increasingly, non-alcoholic liver disease; infection with hepatitis B virus is the most
common cause in sub-Saharan Africa and most parts of Asia. The prevalence of cirrhosis
is diffi cult to assess and probably higher than reported, because the initial stages are
asymptomatic so the disorder is undiagnosed. Prevalence was estimated at 0·3% in a
French screening programme, and the annual incidence was 15·3–132·6 per 100 000
people in studies in the UK and Sweden.
The transition from chronic liver disease to cirrhosis involves inflammation, activation of
hepatic stellate cells with ensuing fi brogenesis, angiogenesis, and parenchymal
extinction lesions caused by vascular occlusion.This process leads to pronounced hepatic
microvascular changes, characterised by sinusoidal remodelling (extracellular matrix
deposition from proliferating activated stellate cells resulting in capillarisation of hepatic
sinusoids), formation of intra hepatic shunts (due to angiogenesis and loss of
parenchymal cells), and hepatic endothelial dysfunction. The endothelial dysfunction is
characterised by insuffi cient release of vasodilators, of which the most important is nitric
oxide. Release of nitric oxide is inhibited by low activity of endothelial nitric oxide
synthetase (as a result of insuffi cient protein-kinase-B-dependent phosphorylation, lack
of cofactors, increased scavenging resulting from oxidative stress, and high
concentrations of endogenous inhibitors of nitric oxide), with concomitant increased
production of vasoconstrictors (mainly adrenergic stimulation and thromboxane A2, but
also activation of the renin-angiotensin system, antidiuretic hormone, and
endothelins).Increased hepatic resistance to portal blood flow is the primary factor
increasing portal pressure in cirrhosis. It results from the combination of structural
disturbances associated with advanced liver disease (accounting for about 70% of total
hepatic vascular resistance) and of functional abnormalities leading to endothelial
dysfunction and increased hepatic vascular tone; portal pressure could perhaps therefore
be decreased by 30% if this functional abnormality were antagonized. The molecular
mechanisms of these abnormalities are being delineated and represent new targets for
therapy. Splanchnic vasodilation with an ensuing increase in the inflow of blood into the
portal venous system contributes to aggravate the increase in portal pressure. Splanchnic
vasodilation is an adaptive response to the changes in intrahepatic hemodynamics in
cirrhosis; its mechanisms are directly opposite to those of the increased hepatic vascular
tone. Because of this opposition, attempts to correct portal hypertension by acting on
hepatic resistance or portal blood inflow should be ideally based on strategies acting as
selectively as possible on the intrahepatic or the splanchnic circulation. In advanced
cirrhosis, splanchnic vasodilation is so intense as to determine a hyper dynamic
splanchnic and systemic circulation, which together with portal hypertension has a major
role in the pathogenesis of ascites and hepatorenal syndrome. Systemic vasodilation
further causes pulmonary ventilation/perfusion mismatch that in severe cases leads to
hepatopulmonary syndrome and arterial hypoxaemia. Portopulmonary hypertension is
characterized by pulmonary vaso constriction, which is thought to be due to endothelial
dysfunction in the pulmonary circulation. Formation and increase in size of varices is
driven by anatomical factors, increased portal pressure and collateral blood flow, and by
angiogenesis dependent on vascular endothelial growth factor, all of which contribute to
variceal bleeding. Dilation of gastric mucosal vessels leads to portalhypertensive
gastropathy. In addition, the shunting of portal blood to the systemic circulation through
the portosystemic collaterals is a major determinant of hepatic encephalopathy, of
decreased first-pass effect of orally administered drugs, and of decreased
reticuloendothelial system function. However, capillarisation of sinusoids and
intrahepatic shunts are also important because these changes interfere with effective
hepatocyte perfusion, which is a major determinant of liver failure.
Diagnosis Most chronic liver disease is notoriously asymptomatic until cirrhosis with
clinical decompensation occurs. Decompensating events include ascites, sepsis, variceal
bleeding, encephalopathy, and non-obstructive jaundice. Imaging by ultrasonography,
CT, or MRI of an irregular and nodular liver together with impaired liver synthetic
function is suffi cient for the diagnosis of cirrhosis. Other fi ndings include small and
shrunken liver, splenomegaly, and evidence of portosystemic collaterals. Diff erential
diagnosis includes congenital hepatic fibrosis (fi brosis without regenerative nodules),
nodular regenerative hyperplasia (nodules but no fibrosis), and non-cirrhotic portal
hypertension. A liver biopsy is seldom needed but study of a sample can provide a defi
nitive diagnosis and confi rm the aetiology in cases of uncertainty. The transjugular
approach yields samples of equal quality to the percutaneous one, is safe, and adds
additional prognostic information through measurement of hepatic-vein pressure gradient
(HVPG).In early cirrhosis, however, conventional imaging can lead to false-negative
diagnosis so other strategies are needed. Non-invasive markers of fi brosis are
increasingly used; they are more informative at the extremes of the liver fi brosis range—
ie, little or no fi brosis, and cirrhosis.They include indirect serum markers (simple, widely
available indices), direct serum markers that measure biomarkers of fi brosis, and
imaging modalities, such as transient elastography . These tests should be used and
interpreted only once the aetiology is known.
Cirrhosis should no longer be regarded as a terminal disease and the concept of a
dynamic process is increasingly accepted. A prognostic clinical subclassifi cation with
four distinct stages has been proposed with substantially diff ering likelihoods of
mortality: stage 1 (compensated with no oesophageal varices) has an estimated mortality
of 1% per year, and stages 2 (compensated with varices), 3 (decompensated with ascites),
and 4 (decompensated with gastrointestinal bleeding) have annual mortality rates of
3·4%, 20%, and 57%, respectively.3 Infections and renal failure have been considered as
stage 5, with 67% 1-year mortality.16,17 Acute decompensating events that lead to organ
failure have mortality of 30%;18 notably, mortality is higher in previously compensated
patients than in those with previous decompensation, which suggests greater tolerance of
the latter through the eff ects of the infl ammatory response.18 Decompensating events
are generally triggered by precipitating factors that include infection, portal-vein
thrombosis, surgery, and hepatocellular carcinoma.
Prevention and treatment of complications The focus of this Seminar is on
prevention and therapy in the initial stages of cirrhosis, including the fi rst
HE is a condition that occurs in people with advanced cirrhosis or severe liver damage.
The damaged liver cannot remove the toxins (ammonia) that a healthy liver normally
would. These toxins then travel through your body until they reach your brain. They can
then affect the brain and cause HE.
The symptoms can be different for each person. Some individuals may not have any
symptoms and the condition is noticed by family members and/or friends. The symptoms
can depend on the extent of your liver disease. You may get confused about who or
where you are. It may seem as if you are not yourself, and you may not be aware of what
you are doing. HE may also change your normal sleep patterns. You may feel wide
awake at night and sleep all day. But with treatment, HE can be controlled, and
symptoms can improve and even stop. So it’s very important that you take care of
yourself and continue any treatment to keep it from coming back.
How are HE and liver disease connected?
Your liver filters everything that enters your body, such as food, drinks, and medicine.
After your intestines break these down into basic substances, your liver then separates the
toxins from the useful substances. Your body then removes the toxins, while the liver
sends the nutrients and vitamins into your bloodstream for your body to use. If your liver
is damaged, it can’t filter out everything it’s supposed to. That means that toxins can
build up and get into your brain. The buildup of these toxins in the brain can lead to HE.
What are the signs of HE?
HE has several stages. At first, people with HE may not even know they have it. To
individuals with HE, things seem fine. But things may not seem fine to family or friends.
People with uncontrolled HE may not be able to drive a car or work a steady job. They
may also need to rely on a caregiver to take care of them. In serious cases,
hospitalization, coma, and even death can occur. Knowing the signs of HE can help slow
it down before it gets worse. Let a doctor know right away if you or a loved one is
beginning to show these signs or has shown them before.
What can cause HE to get worse?
When HE symptoms return and worsen, it’s also known as an “HE recurrence.” It
is not known for certain why some people experience HE recurrences, but several
possible triggers are listed below:
Dehydration—not getting enough water or electrolytes
Low levels of sodium and potassium in the blood (this can happen when
you are dehydrated)
Bleeding in the stomach and intestines
Too much protein in the diet
Drugs that act on the central nervous system, such as sedatives, opioids,
Liver injury from other drugs
A hepatic portal shunt—this is a tube that is placed in a vein in the liver
to relieve pressure
Liver cancer ü Progression of underlying liver condition/cirrhosis of
How important is it to treat HE?
It is unlikely that your HE will get better without treatment. Tell your doctor about any
warning signs as soon as you notice them. Your doctor may give you a medicine for your
HE. If so, it’s very important to take it exactly as you’re told, for as long as you’re told to
What medications are used to treat HE?
There are 2 types of medicine that are used most often to treat HE:
lactulose and antibiotics. Lactulose is a complex sugar. It works by causing you to have
more bowel movements or diarrhea. This will help to flush the toxins out of your system.
Lactulose may also help reduce the amount of toxins that are made in your intestines.
Lactulose has been shown to help during HE recurrences and also to make them less
likely to happen. Antibiotics also work by reducing the amount of toxins that are made in
your intestines. They stop the growth of certain bacteria that create toxins as you digest
food. By reducing bacteria, antibiotics reduce the amount of toxins. There are several
different antibiotics that are used to treat HE. Your doctor will choose the one that is best
for you. Antibiotics have been shown to help prevent HE recurrences and keep patients
from being hospitalized for HE. Some individuals may need a combination of the
lactulose and antibiotics. Talk to your doctor about the best way to approach and manage
8 things you should remember about HE
The normal liver has the ability to accommodate large changes in portal blood flow
without appreciable alterations in portal pressure. Portal hypertension results from a
combination of increased portal venous inflow and increased resistance to portal blood
Patients with cirrhosis demonstrate increased splanchnic arterial flow and, accordingly,
increased splanchnic venous inflow into the liver. Increased splanchnic arterial flow is
explained partly by decreased peripheral vascular resistance and increased cardiac output
in the patient with cirrhosis. Nitric oxide appears to be the major driving force for this
Furthermore, evidence for splanchnic vasodilation exists. Putative splanchnic
vasodilators include glucagon, vasoactive intestinal peptide, substance P, prostacyclin,
bile acids, tumor necrosis factor-alpha (TNF-alpha), and nitric oxide.
Increased resistance across the sinusoidal vascular bed of the liver is caused by fixed
factors and dynamic factors. Two thirds of intrahepatic vascular resistance can be
explained by fixed changes in the hepatic architecture. Such changes include the
formation of regenerating nodules and, after the production of collagen by activated
stellate cells, deposition of the collagen within the space of Disse.
Dynamic factors account for one third of intrahepatic vascular resistance. Stellate cells
serve as contractile cells for adjacent hepatic endothelial cells. The nitric oxide produced
by the endothelial cells, in turn, controls the relative degree of vasodilation or
vasoconstriction produced by the stellate cells. In cirrhosis, decreased local production of
nitric oxide by endothelial cells permits stellate cell contraction, with resulting
vasoconstriction of the hepatic sinusoid. (This contrasts with the peripheral circulation,
where there are high circulating levels of nitric oxide in cirrhosis.) Increased local levels
of vasoconstricting chemicals, such as endothelin, may also contribute to sinusoidal
The portal hypertension of cirrhosis is caused by the disruption of hepatic sinusoids.
However, portal hypertension may be observed in a variety of noncirrhotic conditions.
Pre hepatic causes
Prehepatic causes include splenic vein thrombosis and portal vein thrombosis. These
conditions commonly are associated with hypercoagulable states and with malignancy
(eg, pancreatic cancer).
Intrahepatic causes of portal hypertension are divided into presinusoidal, sinusoidal, and
postsinusoidal conditions. The classic sinusoidal cause of portal hypertension is cirrhosis.
The classic form of presinusoidal portal hypertension is caused by the deposition
ofSchistosoma oocytes in presinusoidal portal venules, with the subsequent development
of granulomata and portal fibrosis. Schistosomiasis is the most common noncirrhotic
cause of variceal bleeding worldwide. Schistosoma mansoniinfection is described in
Puerto Rico, Central and South America, the Middle East, and Africa. S japonicum is
described in the Far East. S hematobium, observed in the Middle East and Africa, can
produce portal fibrosis but more commonly is associated with urinary tract deposition of
The classic postsinusoidal condition is an entity known as veno-occlusive disease.
Obliteration of the terminal hepatic venules may result from ingestion of pyrrolizidine
alkaloids in Comfrey tea or Jamaican bush tea or following the high-dose chemotherapy
that precedes bone marrow transplantation.
Post hepatic causes
Posthepatic causes of portal hypertension may include chronic right-sided heart failure
and tricuspid regurgitation and obstructing lesions of the hepatic veins and inferior vena
cava. The latter conditions, and the symptoms they produce, are termed Budd-Chiari
syndrome. Predisposing conditions include hypercoagulable states, tumor invasion into
the hepatic vein or inferior vena cava, and membranous obstruction of the inferior vena
cava. Inferior vena cava webs are observed most commonly in South and East Asia and
are postulated to be due to nutritional factors.
Symptoms of Budd-Chiari syndrome are attributed to decreased outflow of blood from
the liver, with resulting hepatic congestion and portal hypertension. These symptoms
include hepatomegaly, abdominal pain, and ascites. Cirrhosis ensues only later in the
course of disease. Differentiating Budd-Chiari syndrome from cirrhosis by history or
physical examination may be difficult. Thus, Budd-Chiari syndrome must be included in
the differential diagnosis of conditions that produce ascites and varices.
Hepatic vein patency is checked most readily by performing abdominal ultrasonography,
with Doppler examination of the hepatic vessels. Abdominal computed tomography (CT)
scanning with intravenous (IV) contrast, abdominal magnetic resonance imaging (MRI),
and visceral angiography also may provide information regarding the patency of hepatic
Widespread use of the transjugular intrahepatic portosystemic shunt (TIPS) procedure in
the 1990s for the management of variceal bleeding led to a resurgence of clinicians'
interest in measuring portal pressure. During angiography, a catheter may be placed
selectively via either the transjugular or transfemoral route into the hepatic vein. In the
healthy patient, free hepatic vein pressure (FHVP) is equal to inferior vena cava pressure.
FHVP is used as an internal zero reference point.
Wedged hepatic venous pressure (WHVP) is measured by inflating a balloon at the
catheter tip, thus occluding a hepatic vein branch. Measurement of the WHVP provides a
close approximation of portal pressure. The WHVP actually is slightly lower than the
portal pressure because of some dissipation of pressure in the sinusoidal bed. The WHVP
and portal pressure are elevated in patients with sinusoidal portal hypertension, as is
observed in cirrhosis.
The hepatic venous pressure gradient (HVPG) is defined as the difference in pressure
between the portal vein and the inferior vena cava. Thus, the HVPG is equal to the
WHVP value minus the FHVP value (ie, HVPG = WHVP - FHVP). The normal HVPG
is 3-6mm Hg.
Portal hypertension is defined as a sustained elevation of portal pressure above normal.
An HVPG of 8mm Hg is believed to be the threshold above which ascites potentially can
develop. An HVPG of 12mm Hg is the threshold for the potential formation of varices.
High portal pressures may predispose patients to an increased risk of variceal hemorrhage
Ascites, which is an accumulation of excessive fluid within the peritoneal cavity, can be a
complication of either hepatic or nonhepatic disease. The 4 most common causes of
ascites in North America and Europe are cirrhosis, neoplasm, congestive heart failure,
and tuberculous peritonitis.
In the past, ascites was classified as being a transudate or an exudate. In transudative
ascites, fluid was said to cross the liver capsule because of an imbalance in Starling
forces. In general, ascites protein would be less than 2.5g/dL in this form of ascites. A
classic cause of transudative ascites would be portal hypertension secondary to cirrhosis
and congestive heart failure.
In exudative ascites, fluid was said to weep from an inflamed or tumor-laden peritoneum.
In general, ascites protein in exudative ascites would be greater than 2.5g/dL. Causes of
the condition would include peritoneal carcinomatosis and tuberculous peritonitis.
Attributing ascites to diseases of nonperitoneal or peritoneal origin is more useful.
Thanks to the work of Bruce Runyon, the serum-ascites albumin gradient (SAAG) has
come into common clinical use for differentiating these conditions. Nonperitoneal
diseases produce ascites with a SAAG greater than 1.1g/dL
Salt restriction is the first line of therapy. In general, patients begin with a diet containing
less than 2000mg of sodium daily. Some patients with refractory ascites require a diet
containing less than 500mg of sodium daily. However, ensuring that patients do not
construct diets that might place them at risk for calorie and protein malnutrition is
important. Indeed, the benefit of commercially available liquid nutritional supplements
(which often contain moderate amounts of sodium) often exceeds the risk of slightly
increasing the patient's salt intake.
Diuretics should be considered the second line of therapy. Spironolactone (Aldactone)
blocks the aldosterone receptor at the distal tubule. It is dosed at 50-300mg once daily.
Although the drug has a relatively short half-life, its blockade of the aldosterone receptor
lasts for at least 24 hours. Adverse effects of spironolactone include hyperkalemia,
gynecomastia, and lactation. Other potassium-sparing diuretics, including amiloride and
triamterene, may be used as alternative agents, especially in patients complaining of
Furosemide (Lasix) may be used as a solo agent or in combination with spironolactone.
The drug blocks sodium reuptake in the loop of Henle. It is dosed at 40-240mg daily in 1-
2 divided doses. Patients infrequently need potassium repletion when furosemide is dosed
in combination with spironolactone. An Italian study by Angeli et al found sequential
dosing with a potassium-sparing diuretic plus furosemide to be superior for patients with
moderate ascites without renal failure when compared with potassium-sparing diuretic
Aggressive diuretic therapy in hospitalized patients with massive ascites can safely
induce a weight loss of 0.5-1kg daily, provided that patients undergo careful monitoring
of renal function. Diuretic therapy should be held in the event of electrolyte disturbances,
azotemia, or induction of hepatic encephalopathy.
Thus far, evidence-based medicine has not firmly supported the use of albumin as an aid
to diuresis in a patient with cirrhosis who is hospitalized. The author's anecdotal
experience suggests that albumin may increase the efficacy and safety of diuretics. The
author's practice in hospitalized patients who are hypoalbuminemic is to administer IV
furosemide following IV infusion of albumin at 25g twice daily, in addition to providing
ongoing therapy with spironolactone. One article supported the use of chronic albumin
infusions to achieve diuresis in patients with diuretic-resistant ascites.
Albumin infusion may protect against the development of renal insufficiency in patients
with SBP. Patients receiving cefotaxime and albumin at 1g/kg daily experienced a lower
risk of renal failure and a lower in-hospital mortality rate than patients treated with
cefotaxime and conventional fluid management.
V2 receptor antagonists
Vasopressin V2 receptor antagonists are a class of agents with the potential to increase
free-water excretion, improve diuresis, and decrease the need for paracentesis. However,
no such agent has received US Food and Drug Administration (FDA) approval for this
Tolvaptan (Samsca, Otsuka Pharmaceutical Co; Tokyo, Japan) is an oral V2 receptor
antagonist; it received FDA approval in 2009 only for the management of hyponatremia.
A black box warning cautions against treatment initiation in outpatients. Furthermore, it
may be associated with an increased incidence of GI bleeding in patients with cirrhosis.
The author advises against its use for ascites management at this time.
Aggressive diuretic therapy is ineffective in controlling ascites in approximately 5-10%
of patients. Such patients with massive ascites may need to undergo large-volume
paracentesis to obtain relief from symptoms of abdominal discomfort, anorexia, or
dyspnea. The procedure also may help to reduce the risk of umbilical hernia rupture.
Large-volume paracentesis was first used in ancient times. It fell out of favor from the
1950s through the 1980s with the advent of diuretic therapy and following a handful of
case reports describing paracentesis-induced azotemia. In 1987, Gines and colleagues
demonstrated that large-volume paracentesis could be performed with minimal or no
impact on renal function. This and other studies showed that 5-15L of ascites could be
removed safely at one time.
Large-volume paracentesis is thought to be safe in patients with peripheral edema and in
patients not currently treated with diuretics. Debate exists whether colloid infusions (eg,
with 5-10g of albumin per 1L of ascites removed) are necessary to prevent intravascular
volume depletion in patients who are receiving ongoing diuretic therapy or in patients
with mild or moderate, underlying renal insufficiency.
LeVeen shunts and Denver shunts are devices that permit the return of ascites fluid and
proteins to the intravascular space. Plastic tubing inserted subcutaneously under local
anesthesia connects the peritoneal cavity to the internal jugular vein or subclavian vein
via a pumping chamber. These devices are successful at relieving ascites and reversing
protein loss in some patients. However, shunts may clot and require replacement in 30%
Serious complications are observed in at least 10% of the recipients of these devices,
including peritoneal infection, sepsis, disseminated intravascular coagulation, congestive
heart failure, and death. The author considers peritoneovenous shunts to be a last resort
for patients with refractory ascites who are not candidates for TIPS or liver
transplantation. The safety of repeat large-volume paracentesis procedures may actually
outweigh the safety of peritoneovenous shunt placement.
Portosystemic shunts and transjugular intrahepatic portosystemic shunts
The prime indication for portocaval shunt surgery is the management of refractory
variceal bleeding. Since 1945, however, the medical field has recognized that portocaval
shunts, by decompressing the hepatic sinusoid, may improve ascites. The performance of
a side-to-side portocaval shunt for ascites management must be weighed against the
approximate 5% mortality rate associated with this surgery and the chance (as high as
30%) of inducing hepatic encephalopathy.
A TIPS is an effective tool in managing massive ascites in some patients. Ideally, TIPS
placement produces a decrease in sinusoidal pressure and in plasma renin and aldosterone
levels, with subsequent improved urinary sodium excretion. In one study, 74% of patients
with refractory ascites achieved complete remission of ascites within 3 months of TIPS
placement. Typically, about one half of appropriately selected patients undergoing TIPS
achieve significant relief of ascites.
Multiple studies have demonstrated that a TIPS is superior to large-volume paracentesis
when it comes to the control of ascites. One meta-analysis of individual patient data
demonstrated an improvement in transplant-free life expectancy in patients whose
massive ascites was treated with a TIPS, as opposed to large-volume paracentesis.
However, the creation of a TIPS has the potential to worsen preexisting hepatic
encephalopathy and exacerbate liver dysfunction in patients with severe, underlying liver
Both a pre-TIPS bilirubin level of greater than 3mg/dL and a pre-TIPS Model for End-
Stage Liver Disease (MELD) score of greater than 18 are associated with an increased
mortality rate when a TIPS is created for the management of ascites. In the author's
opinion, TIPS use should be reserved for patients with Child Class B cirrhosis or patients
with Child Class C cirrhosis without severe coagulopathy or encephalopathy.
In the 1990s, shunt stenosis was observed in one half of cases within 1 year of TIPS
placement, necessitating angiographic revision. Although the advent of coated stents
appears to have reduced the incidence of shunt stenosis, patients must still be willing to
return to the hospital for Doppler and angiographic follow-up of TIPS patency.
Patients with massive ascites have 1-year survival rate of less than 50%. Liver
transplantation should be considered as a potential means of salvaging the patient prior to
the onset of intractable liver failure or hepatorenal syndrome.
Esophageal varices are abnormal, enlarged veins in the lower part of the esophagus —
the tube that connects the throat and stomach. Esophageal varices occur most often in
people with serious liver diseases.
Esophageal varices develop when normal blood flow to the liver is obstructed by scar
tissue in the liver or a clot. Seeking a way around the blockages, blood flows into smaller
blood vessels that are not designed to carry large volumes of blood. The vessels may leak
blood or even rupture, causing life-threatening bleeding.
A number of drugs and medical procedures can help prevent and stop bleeding from
Esophageal varices usually don't cause signs and symptoms unless they bleed. Signs and
symptoms of bleeding esophageal varices include:
Black, tarry or bloody stools
Shock (in severe case)
Your doctor may suspect varices if you have any of the following signs of liver
Yellow coloration of your skin and eyes (jaundice)
A cluster of tiny blood vessels on the skin, shaped like a spider (spider nevi)
Reddening of the skin on the palm of your hands (palmar erythema)
A hand deformity known as Dupuytren's contracture
Fluid buildup in your abdomen (ascites)
Esophageal varices sometimes form when blood flow to your liver is obstructed, most
often by scar tissue in the liver caused by liver disease. The blood flow to your liver
begins to back up, increasing pressure within the large vein (portal vein) that carries
blood to your liver. This pressure (portal hypertension) forces the blood to seek alternate
pathways through smaller veins, such as those in the lowest part of the esophagus. These
thin-walled veins balloon with the added blood. Sometimes the veins can rupture and
Severe liver scarring (cirrhosis). A number of liver diseases can result in cirrhosis, such
as hepatitis infection, alcoholic liver disease, fatty liver disease and a bile duct disorder
called primary biliary cirrhosis. Esophageal varices occur in about 40 percent of people
who have cirrhosis.
Blood clot (thrombosis). A blood clot in the portal vein or in a vein that feeds into the
portal vein called the splenic vein can cause esophageal varices.
A parasitic infection. Schistosomiasis is a parasitic infection found in parts of Africa,
South America, the Caribbean, the Middle East and Southeast Asia. The parasite can
damage the liver, as well as the lungs, intestine and bladder.
Budd-Chiari syndrome. This rare condition causes blood clots that can block the veins
that carry blood out of your liver
Although many people with advanced liver disease develop esophageal varices, most
won't experience bleeding. Varices are more likely to bleed if you have:
High portal vein pressure. The risk of bleeding increases with the amount of
pressure in the portal vein (portal hypertension).
Large varices. The larger the varices, the more likely they are to bleed.
Red marks on the varices. When viewed through an endoscope passed down your
throat, some varices show long, red streaks or red spots. These marks indicate a
high risk of bleeding.
Severe cirrhosis or liver failure. Most often, the more severe your liver disease,
the more likely varices are to bleed.
Continued alcohol use. Your risk of variceal bleeding is far greater if you
continue to drink than if you stop, especially if your disease is alcohol related.
The most serious complication of esophageal varices is bleeding. Once you have had a
bleeding episode, your risk of another bleeding episode is greatly increased. In some
cases, blood loss is so great that you go into shock. This can lead to death.
The primary aim in treating esophageal varices is to prevent bleeding. Bleeding
esophageal varices are life-threatening. If bleeding occurs, treatments are available to try
to stop the bleeding.
Treatments to lower blood pressure in the portal vein may reduce the risk of bleeding
esophageal varices. Treatments may include:
Medications to reduce pressure in the portal vein. A type of blood pressure drug
called a beta blocker may help reduce blood pressure in your portal vein,
decreasing the likelihood of bleeding. These medications include propranolol
(Inderal, Innopran) and nadolol (Corgard).
Using elastic bands to tie off bleeding veins. If your esophageal varices appear to
have a very high risk of bleeding, your doctor may recommend a procedure called
band ligation. Using an endoscope, the doctor snares the varices and wraps them
with an elastic band, which essentially "strangles" the veins so they can't bleed.
Esophageal band ligation carries a small risk of complications, such as scarring of
Bleeding varices are life-threatening, and immediate treatment is essential.
Treatments used to stop bleeding include:
Using elastic bands to tie off bleeding veins.
Medications to slow blood flow into the portal vein. Medications can slow the
flow of blood from the internal organs to the portal vein, reducing the pressure in
the vein. A drug called octreotide (Sandostatin) is often used in combination with
endoscopic therapy to treat bleeding from esophageal varices. The drug is usually
continued for five days after a bleeding episode.
Diverting blood flow away from the portal vein. Your doctor may recommend a
procedure called transjugular intrahepatic portosystemic shunt (TIPS). The shunt
is a small tube that is placed between the portal vein and the hepatic vein, which
carries blood from your liver back to your heart. By providing an additional path
for blood, the shunt reduces pressure in the portal vein and often stops bleeding
from esophageal varices. But TIPS can cause a number of serious complications,
including liver failure and mental confusion, which may develop when toxins that
would normally be filtered by the liver are passed through the shunt directly into
the bloodstream. TIPS is mainly used when all other treatments have failed or as a
temporary measure in people awaiting a liver transplant.
Replacing the diseased liver with a healthy one. Liver transplant is an option for
people with severe liver disease or those who experience recurrent bleeding of
esophageal varices. Although liver transplantation is often successful, the number
of people awaiting transplants far outnumbers the available organs.
Ineffective breathing pattern
Fluid and electrolyte imbalance
Ineffective tissue perfusion
Imbalance nutrition less than body requirements