3 chapter 2 12

section

PORTAL HYPERTENSION
KEY POINTS
Dened as a portal venous pressure >10mmHg (1.3kPa), portal hypertension usually results from the sinusoidal obstrction of liver cirrhosis. The prognosis depends upon the degree of hepatocellular dysfunction, which is assessed clinically by the ChildPugh classication. Ascites and encephalopathy indicate decompensated liver failure. Presinusoidal obstruction (portal vein thrombosis) and postsinusoidal obstrcution (hepatic vein thrombosis or BuddChiari syndrome) should be distinguished from cirrhosis because liver function is generally preserved in these conditions. Splanchnic hyperemia contributes to the development of portosystemic collaterals. Emergency treatment of patients who present with bleeding esophagogastric varices includes drugs to reduce portal pressure, balloon tamponade and endoscopic measures (injection, banding) aimed at thrombosing the varices. Long-term portal decompression can be achieved by radiologic or surgical means. Transjugular intrahepatic portosystemic stenting (TIPS) has encroached upon the role of operative shunts, whether partial or total. Liver transplantation is reserved for those with end-stage liver disease.

J Michael Henderson
decompression of varices with the introduction of the radiologically placed intrahepatic shunt.13 However, the greatest impact on portal hypertension has been the clinical coming of age of liver transplantation through the 1980s.14 Transplantation deals not only with the consequences of portal hypertension, but also with the underlying disease. All therapies in the 1990s must be judged against the need for, availability of and outcome of liver transplantation (see Chapter 6.5).

PORTAL HYPERTENSION

PORTAL HYPERTENSION: DEFINITION AND CAUSES

DEFINITION
Portal hypertension is present if portal venous pressure exceeds 10mmHg (1.3kPa). Normal portal venous pressure is 510mmHg (0.71.3kPa), which is sufficient to maintain a portal flow through the hepatic sinusoids of approximately 1 liter/min.

CLASSIFICATION AND CAUSES

Portal hypertension can be classied into presinusoidal, sinusoidal and postsinusoidal causes (Fig. 12.1). The clinical usefulness of this classication is in separating patients who have normal hepatocellular function from those who have hepatocellular damage. This separation has important implications in outcome.

Presinusoidal portal hypertension

This may be either extrahepatic or intrahepatic. Thrombosis of the extrahepatic portal vein or one of its major tributaries may lead to extrahepatic portal hypertension. Portal vein thrombosis may be seen either: in early life associated with umbilical sepsis; or in older patients who have a hypercoagulable state such as polycythemia rubra vera or a myeloproliferative disorder. The other etiologies for portal or splenic vein thrombosis are tumors of the pancreas, pancreatitis or metastatic nodes at the hepatic hilus. Isolated splenic vein thrombosis produces left-sided portal hypertension,

INTRODUCTION
The association between liver disease and ascites was recognized by the Egyptians and was frequently documented in medical writings in the Middle Ages. However, it was not until 1900 that the relationship between cirrhosis, varices and gastrointestinal bleeding was established.1 The term portal hypertension was rst coined by Gilbert and Villaret in 19062 when cirrhosis, high portal pressure and ascites were linked pathophysiologically. At the beginning of the 20th century, it was believed that portal hypertension resulted from splenomegaly rather than causing it and this belief gave rise to the forward flow theory of portal hypertension. Portal pressure was first measured intraoperatively in 1936 by Rousselot3 and in 1937 by Thompson et al.4 Hepatic vein catheterization was rst developed in the 1940s and lead to indirect methods for measuring portal pressure by outow occlusion.5 Evolving techniques in the past two decades for direct pressure measurements with quantitation of portal, arterial and shunt ows and for vascular imaging have led to an improved understanding of the pathophysiology of portal hypertension.6,7 Historic landmarks in the management of portal hypertension have largely focused around surgical events. In 1877, Eck8 declared that a portacaval shunt could safely be performed in humans based on his studies in eight dogs: seven of his animals died and the eighth ran away! The response was Pavlovs classic paper9 in 1893 describing the effects of total portal diversion leading to liver failure and encephalopathy, which he described as meat intoxication. Splenectomy, omentopexy and various degrees of devascularization gained some acceptance in the early part of this century, but clearly did not deal with the underlying portal hypertension.10 Endoscopic therapy for varices was initially introduced in the 1930s11 and resurfaced in the 1970s, it was initially provided by surgeons who used rigid endoscopes, then by gastroenterologists following the introduction of exible endoscopy. Surgical shunts were first attempted in the early 1900s and came into the clinical arena in the 1940s.12 Total shunts, partial shunts and selective variceal decompression evolved over the next three decades and have provided the methods of surgical decompression. The 1990s have seen a resurgence of interest in

CAUSES AND SITES OF BLOCK FOR PORTAL HYPERTENSION Portal vein Extrahepatic Intrahepatic Sinusoids Hepatic veins

Congenital Umbilical sepsis Trauma Hypercoagulation state Malignant occlusion

Schistosomiasis Congenital hepatic fibrosis Primary biliary cirrhosis

Cirrhosis

BuddChiari syndrome Veno-occlusive disease

Fig. 12.1 Etiologies of portal hypertension. These are divided into those associated with normal liver function (presinusoidal) and those associated with liver damage (sinusoidal).

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GASTROINTESTINAL SURGERY

which involves the spleen and gastric fundus. It is important to identify this because it is cured by splenectomy. Congenital hepatic brosis, schistosomiasis and the early stages of primary biliary cirrhosis are pathologic processes that affect the terminal portal venules in the presinusoidal position and result in intrahepatic presinusoidal portal hypertension. In all of these situations, liver function is well preserved and the emphasis of management is on dealing with variceal bleeding, which is the major complication of these types of portal hypertension.

Sinusoidal obstruction
This is caused by cirrhosis, and viral hepatitis and ethanol abuse are the most common etiologies. Less common causes of cirrhosis such as autoimmune liver disease, hemochromatosis, Wilsons disease, more advanced stages of primary biliary cirrhosis, sclerosing cholangitis and 1-antitrypsin deciency should be considered if the etiology is less clear. The importance of cirrhosis as the etiology for portal hypertension is that there is concurrent hepatocellular damage as well as the increased resistance to portal venous ow. Sinusoidal anatomy is destroyed, regenerative nodules form, fibrosis further obstructs portal venous flow and the normal metabolic processes are disrupted. The emphasis in this group of patients is on careful evaluation of liver function and an assessment of the activity of the underlying liver disease, its rate of progression and the hepatic reserve. The question to be answered is whether the cirrhosis is compensated or decompensated.

vein forms the left intrahepatic portal vein. The left vein also communicates directly with the sinus venosus, which connects to the inferior vena cava; the ductus venosus therefore allows blood to bypass most of the hepatic sinusoids in the fetal circulation. The portal vein is formed behind the neck of the pancreas as the superior mesenteric joins the splenic vein (Fig. 12.2). The portal vein itself is approximately 68cm long and 11.2cm in diameter. It runs in the free edge of the lesser omentum from the pancreas to the right end of the porta hepatis. The superior mesenteric vein forms from the draining jejunal and ileal veins, its major tributaries being the ileocolic, right colic and middle colic veins. The left and right gastric veins and the gastroepiploic veins drain into the main portal vein. Two major tributaries of importance in portal hypertension are the: left gastric (coronary) vein; and inferior mesenteric vein. These enter the portal system close to the splenic portal vein junction, the left gastric vein superiorly and the inferior mesenteric vein inferiorly. The other important surgical anatomy in portal hypertension is the venous anatomy of the gastroesophageal junction, which has been extensively studied by several investigators in the past two decades.15,16 Zones of venous drainage at the gastroesophageal junction are shown in Figure 12.3.

PATHOPHYSIOLOGY
The major theories in the pathophysiology of portal hypertension have been the backward and the forward ow theories: backward theory postulates that all consequences of portal hypertension result from obstruction to portal venous ow; and forward ow theory postulates that increased inow to the portal venous system (usually because of splenomegaly) causes the changes that lead to the clinical ndings of portal hypertension. Current evidence indicates that both theories contribute to the pathophysiology.17 The stages in developing portal hypertension have been claried over the past decade with a sequence of events that is summarized in Figure 12.4 The primary event is an increased resistance to portal venous flow. The increased pressure on the splanchnic venous bed leads to the development of collaterals to bypass the blockage to flow. The compensatory increase in splanchnic inow perpetuates and aggravates the portal hypertension. The major importance in clarifying this pathophysiology is that it has implications for clinical management, particularly in pharmacologic therapy.

Postsinusoidal portal hypertension

This results from hepatic venous outflow obstruction, which falls under the broad category of BuddChiari syndrome. This is the least common cause of portal hypertension, but may be reversible if identied and treated early.

SURGICAL ANATOMY
The embryologic development of the portal circulation is from: two vitelline veins, which bring blood from the yolk sac; and two umbilical veins, which return blood from the placenta. The vitelline veins intercommunicate in the septum transversum, which is the site of development of the liver sinusoids. The extrahepatic portal venous system develops primarily from the left vitelline vein. The right umbilical vein is the main contributor to the intrahepatic right portal vein and the left umbilical

PORTAL VEIN ANATOMY

Portal vein

Left gastric vein Right gastric vein

ZONES OF VENOUS DRAINAGE AT THE GASTROESOPHAGEAL JUNCTION Zone 1: Gastric Site 23cm below the gastroesophageal junction (junctional zone) Veins Veins run longitudinally towards esophagus rather than via an irregular network as seen in the rest of the stomach Evenly distributed longitudinal veins correspond to major mucosal folds of the esophagus; no perforating veins Large looping collaterals between internal and external venous plexuses of the esophagus 45 longitudinal veins in the lamina propria and perforating veins from the submucosa to external venous plexus

2: Palisade

23cm above the gastric zone extending into lower esophagus

Pancreas Splenic vein Superior mesenteric vein Inferior vena cava Gastroepiploic vein Inferior mesenteric vein

3: Perforating

2cm up the esophagus above the palisade zone

4: Truncal

810cm up the esophagus above the perforating zone

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12.2

Fig. 12.2 Portal venous anatomy in portal hypertension. The superior mesenteric, splenic and inferior mesenteric veins unite behind the neck of the pancreas to form the portal vein. The right and left gastric and gastroepiploic veins enter the portal and superior mesenteric veins, respectively.

Fig. 12.3 Zones of venous drainage at the gastroesophageal junction.

PORTAL HYPERTENSION

HEMODYNAMIC PATHOPHYSIOLOGIC CHANGES

These can be broadly divided into splanchnic and systemic changes. Portal venous pressure increases, usually to 20mmHg or greater, and is transmitted back into the whole splanchnic bed. The major sites for collateral pathway development are the natural watersheds for portosystemic collaterals: gastroesophageal; hemorrhoidal; periumbilical; and retroperitoneal. Increased portal venous pressure, dilatation of the splanchnic venous bed and the development of collaterals all act as stimuli for splanchnic hyperemia. The marked increase in total splanchnic ow perpetuates the initial changes. Local release of nitric oxide through various mediators appears to be the relevant mechanism.17 The systemic hemodynamic changes occur whatever the etiology of portal hypertension. In parallel with the splanchnic vascular changes of vasodilatation and hyperemia, there is an increase in plasma volume. Total systemic vascular resistance decreases, primarily in the splanchnic bed, but also to a lesser degree in the peripheral circulation. The clinical consequences are that a hyperdynamic systemic circulation develops with a high cardiac output and a low total systemic vascular resistance and a low normal blood pressure.

30%). Overall, it is estimated that once there has been an initial bleed, the risk of rebleeding is 7580% within the rst year. These natural history factors are important in making management decisions.

CLINICAL PRESENTATION
The main clinical presentations of portal hypertension are shown in Figure 12.5. The major factor in determining the clinical course, management and outcome of patients who have portal hypertension is the status of the underlying liver disease. Approximately 90% of patients in the USA and Europe who present with portal hypertension have underlying cirrhosis and the critical determinant is whether this is compensated or decompensated. Ascites and encephalopathy are the two clinical presentations indicative of decompensation. In contrast, variceal bleeding, splenomegaly and hypersplenism or portal hypertension as an incidental nding are presentations that may be associated with compensated disease and have a very different prognosis. Classication of the severity of the underlying liver disease at the time of presentation of a patient who has portal hypertension is important. Childs classification19 has stood the test of time and, although it has undergone several modifications, it remains the standard for the initial evaluation of patients. Childs original classification used the three clinical parameters of ascites, encephalopathy and nutrition plus the two biochemical parameters of bilirubin and albumin with a grading of 13 for each parameter. The Childs classification modification most commonly used is that of Pugh,20 in which nutritional status is replaced by prothrombin time. The ChildPugh classication is given in Figure 12.6 and has proved to be of clinical use over the past 20 years in grading disease severity for patients presenting with portal hypertension. ChildPugh A patients have a score of 5 or 6, ChildPugh B patients have a score of 79 and ChildPugh C patients have a score of 1015. Each of the clinical presentations referred to above is discussed in more detail below.

NATURAL HISTORY OF PORTAL HYPERTENSION

This has been claried over the past two decades and is of clinical importance.18 When cirrhosis is first diagnosed approximately 30% of patients have compensated disease and 60% who have decompensated disease have varices. It is estimated that about 8% of patients who have cirrhosis develop varices each year. Small varices develop into large varices and large varices have a greater risk of bleeding. The clinical implication of these observations is that all patients who are diagnosed with cirrhosis should have a screening endoscopy to look for varices and to determine the need to start therapy to reduce the risk of an initial bleed. The risk of the rst variceal bleed may be predicted from: variceal size; red color signs; portal pressure gradient greater than 12mmHg (1.6kPa); and poor liver function. An acute variceal bleed carries a mortality rate of approximately 30%, most deaths occurring among poorer risk patients with a Child score 8 points. Early rebleeding occurs in 2050% of patients in the first 710 days after the first variceal bleed and is a poor prognostic sign. After the rst 6 weeks, the rebleeding risk drops to approximately the same as before the initial bleed (i.e. about

INVESTIGATION
HISTORY AND PHYSICAL EXAMINATION
The key components of the history in patients who have portal hypertension relate to clarifying the underlying liver disease and any complications of portal hypertension. Exposure to hepatitis, a history of excessive alcohol ingestion or a family history of liver disease, are relevant in history taking. With respect to complications, a history of upper or lower gastrointestinal bleeding, uid accumulation, confusional episodes or other nonspecic stigmas of chronic liver disease such as fatigue and itching, should be sought. Any history of upper gastrointestinal bleeding should be claried in detail, with particular emphasis on the severity, occurrence of multiple episodes and management modalities used. General physical examination should include a visual inspection for muscle wasting, jaundice and spider angiomas, and in males for gynecomastia and testicular atrophy. Abdominal examination focuses on hepatosplenomegaly and ascites, but may also reveal prominent abdominal wall veins. Other nonspecific findings that may point towards established portal hypertension are the systemic hemodynamic changes, which are manifested as a mild persistent tachycardia and a low blood pressure.

STAGES IN THE DEVELOPMENT OF PORTAL HYPERTENSION Increased resistance to portal venous flow Formation of portal systemic collaterals Dilatation of the splanchnic venous bed and increased splanchnic flow Expansion of the intravascular plasma volume Peripheral and splanchnic vasodilatation leading to development of a hyperkinetic systemic circulation

Fig. 12.4 Stages in the development of portal hypertension.

ALLOCATION OF POINTS USING THE CHILDPUGH CLASSIFICATION PRESENTING FEATURES OF PORTAL HYPERTENSION Variceal bleeding Ascites Encephalopathy Splenomegaly, hypersplenism Incidental diagnosis Parameter Bilirubin (mg/dl) Albumin (g/dl) Increase in prothrombin time (s) Ascites Encephalopathy 1 point <2 >3.5 13 None None 2 points 23 2.83.5 46 Slight 12 3 points >3 <2.8 >6 Moderate 34

Fig. 12.5 Presenting features of portal hypertension.

Fig. 12.6 ChildPugh classication. The grades are A for 56 points, B for 79 points and C for 1015 points.

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HEMATOLOGY
A complete blood count including hemoglobin concentration, hematocrit and white cell and platelet counts should be performed: hematocrit is often reduced, either secondary to chronic bleeding or as a result of the expanded plasma volume; platelet count may be less than 100,000/mm3 and the white cell count less than 4000/mm3 because of hypersplenism. Other useful hematologic parameters to measure more advanced liver disease are the prothrombin time and the plasma brinogen levels. Prothrombin time is used in calculating the ChildPugh score (see above): prolongation of the international normalized ratio by more than 1.5 indicates moderate liver impairment. A plasma fibrinogen level of less than 100mg/dl indicates advanced liver disease associated with poor synthetic function. The therapeutic implication is an increased risk of bleeding and the need to give fresh frozen plasma to patients who are bleeding or require any interventional procedure.

The grading of the severity of portal hypertensive changes has been extensively studied by Japanese21 and European workers.22 The classication of the severity of varices is based on size, extent and the color signs.

RADIOLOGIC IMAGING
For a patient who has established or suspected portal hypertension, abdominal vascular imaging is required as a further investigation. The initial imaging study is ultrasound with Doppler evaluation of the major vessels. An ultrasound scan of the liver should inspect overall morphology; it is the initial screening procedure for focal intrahepatic lesions that may suggest hepatocellular carcinoma. The vessel imaging focuses on the main portal vein, the splenic vein, the hepatic veins and the infra- and intrahepatic inferior vena cava.23 The goal of this study in screening of patients who have portal hypertension is to evaluate portal vein patency and directional ow (Fig. 12.7). It identies most of the extrahepatic causes of portal hypertension. The value of portal vein size measurement and ow velocities as measures of the severity of portal hypertension has been extensively studied, but their value in clinical practice has not been fully established. A CT scan or MRI of the abdomen may be indicated for some patients who have portal hypertension to evaluate the liver morphology in greater detail and to image the intra-abdominal venous system. All patients do not need all of these studies and centers must decide which modality in their experience provides the best clinically useful information.

BIOCHEMISTRY
Biochemical evaluation of the patient who has portal hypertension emphasizes several areas. Serum electrolytes, blood urea nitrogen and serum creatinine provide valuable information at initial presentation and need to be followed during therapy. In portal hypertension total body sodium is increased, but intravascular sodium is depleted, particularly following aggressive attempts at diuresis. As the liver is the sole site of urea production in the body, a low blood urea nitrogen at presentation may be indicative of poor liver function, whereas an elevated blood urea nitrogen in a patient who has cirrhosis may indicate more severe renal insufficiency than suspected. The same principle holds true for serum creatinine: patients who have advanced liver disease have a decreased lean body mass, so an elevated serum creatinine indicates more severe renal insufciency than in a normal subject. Other biochemical parameters of importance in investigating patients who have liver disease are serum bilirubin, albumin, aminotransferases, alkaline phosphatase and -glutamyltranspeptidase. These indices assess the severity and activity of the underlying liver disease: serum bilirubin and albumin are used to calculate the ChildPugh score and carry greater prognostic signicance; aminotransferases are elevated as a result of acute hepatocellular injury and are used as an index of ongoing hepatitis, but do not help in differentiating the etiology of the hepatitis; and increases in alkaline phosphatase and -glutamyltranspeptidase are indicative of cholestasis either at large duct or canalicular levels and are indices for measuring the severity of cholestatic liver disease.

ANGIOGRAPHY
Angiography is the gold standard for abdominal vascular imaging in patients who have portal hypertension.24 Several techniques are used to visualize the portal venous system either through a transvenous or a transarterial approach. This is used to image the hepatic arterial supply and may have some benet in detecting small vascular lesions suggestive of hepatocellular carcinoma. The venous phase imaging of arterial injection is used to delineate fully the portal venous anatomy in patients being considered for decompressive shunt surgery. Splenic artery injection followed through the venous phase gives the best imaging of the splenic vein and its tributaries (Fig. 12.8). Superior mesenteric artery injection through the venous phase is the preferred method of imaging for the superior mesenteric and portal vein (Fig. 12.9). Identication of the site, size and ow parameters of the major collaterals such as the left gastric and inferior mesenteric vein are best dened on arteriography. These studies provide data on the pathophysiology for specic patients that cannot be obtained by noninvasive methods.

Venography
This is used to assess the hepatic veins, the inferior vena cava and the left renal vein and may be carried out by either a transfemoral or a transjugular approach. Hepatic venography combines both pressure measurements and imaging. Pressures are measured in the hepatic vein in both the occluded and free vein

SEROLOGY
Investigation of patients who have chronic liver disease requires measurement of serologic markers of liver disease. Hepatitis A, B and C serologies should be carried out routinely and for those who are positive for hepatitis B or C, a more in-depth analysis for evidence of active viral replication by testing for hepatitis B DNA or hepatitis C RNA titers should be performed. Other serologic tests that may help dene the etiology of chronic liver disease are tests for: antimitochondrial antibody (primary biliary cirrhosis); antinuclear antibody (for evidence of autoimmune hepatitis); ceruloplasmin for Wilsons disease; and 1-antitrypsin to reveal deciency.

ENDOSCOPY
All patients conrmed or suspected of having cirrhosis should have a screening upper gastrointestinal endoscopy. The rationale for this policy is based on the natural history, which denes a bleeding risk if varices are present. If varices are identied at screening, patients should be started on appropriate prophylactic pharmacologic therapy to reduce the risk of an initial bleed. For a patient who has cirrhosis and has an upper gastrointestinal bleed, endoscopy is the rst step in evaluation of the bleeding site and in making decisions on further management. The major aims at the time of endoscopy are to nd out whether the patient has esophageal and/or gastric varices and to establish whether there is portal hypertensive gastropathy. Other pathologies such as peptic ulcer disease, gastric antral vascular ectasia or malignancies should be excluded.

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12.4

Fig. 12.7 Ultrasound scan with Doppler evaluation of the portal vein showing patency and prograde ow. MPV, main portal vein.

PORTAL HYPERTENSION

position, using the same principle as pulmonary pressure measurement with a SwanGanz catheter. The occluded pressure is an indirect measurement of hepatic sinusoidal pressure. The difference between the occluded and free pressure the hepatic venous pressure gradient (HVPG) is used as a clinical measurement of portal hypertension. If a catheter is placed in the wedged position in a hepatic vein and contrast or carbon dioxide is injected, the contrast refluxes into the hepatic sinusoids and, in patients who have sluggish portal venous ow, into the main portal vein and/or its feeding tributaries (Fig. 12.10). This can serve as an excellent imaging modality for portal vein patency. Imaging of and pressure measurements within the inferior vena cava and left renal vein may be important in making decisions for surgical intervention. The vena cava may be compressed by an enlarged and cirrhotic liver with distortion or pressure gradient. The left renal vein has anomalies in 20% of the population, with a circumaortic vein present in 16% and a totally retroaortic left renal vein in 4%. The transjugular route of venous access has become the preferred method because it allows easier intervention for liver biopsy and is the route for a transjugular intrahepatic portal systemic shunt (TIPS) if indicated.

have Wilsons disease before copper chelation. For all types of cirrhosis, the morphologic ndings of acute cellular injury and the extent of brosis are parameters that help in assessing the degree of underlying liver disease and dictate therapy. Finally, image-guided biopsy may be required for focal lesions. There are three techniques for liver biopsy: percutaneous, transjugular or laparoscopic.

Percutaneous biopsy
The blind percutaneous biopsy is satisfactory for evaluating diffuse liver disease, but is inadequate if focal changes need to be dened. Any biopsy may be performed using either a suction-type Menghini needle25 or a Tru-Cut biopsy needle. The same techniques can be used for percutaneous guided biopsy under either ultrasound or CT imaging. The most serious complication of percutaneous biopsy is bleeding, which occurs in 0.10.2% of patients.26

Transjugular biopsy
This may be combined with angiographic images as outlined above and is useful for patients in whom percutaneous biopsy may be difficult or dangerous (e.g. in those who have massive ascites or who have severe coagulopathy). A catheter is advanced through the transjugular route into a major hepatic vein and a biopsy is taken with either biopsy forceps or a needle.27

LIVER BIOPSY
A histologic diagnosis is often needed before initiating therapy, for example for patients who have hemochromatosis before phlebotomy or patients who
Fig. 12.8 Venous phase imaging of splenic artery injection demonstrating venous anatomy.

Laparoscopic biopsy
This has some advantage in that it is combined with direct imaging of the liver. Financial costs are higher, however and it should not be used routinely. It may be particularly useful when looking for pathology which varies in different parts of the liver such as in BuddChiari syndrome and also for directed biopsy of focal lesions, when laparoscopic visualization can be combined with laparoscopic ultrasound to localize lesions. The complications of laparoscopy are low, but include bleeding, perforation of a viscus and infection. Morbidity rates of 0.72.2% have been reported.28

QUANTITATIVE LIVER FUNCTION TESTING

This eld has received attention over the past 25 years, but has not achieved widespread clinical use. Quantitative tests can be broadly divided into those: limited by hepatocyte function; and dependent upon liver blood ow. Markers such as galactose, indocyanine green, antipyrine or lidocaine (lignocaine) have been most widely used for quantitative testing. Recently, monoethylglycinexylidide (MEGX) formation from lidocaine has received attention, but has not been widely accepted because the rate limiting metabolic step varies at different stages in liver disease. Other methods used to quantitate liver function include rates of urea synthesis and high rst-pass clearance methods to estimate liver blood ow, such as sorbital and galactose. The major difficulty with quantitative liver function testing is the high inducibility of many of the pathways being studied, such as the P450 enzymes.

Fig. 12.10 Portal venous anatomy demonstrated by retrograde injection of carbon dioxide using a catheter wedged in the main right hepatic vein.

Fig. 12.9 Venous phase imaging of superior mesenteric artery injection demonstrating the superior mesenteric and portal veins.

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TREATMENT STRATEGIES FOR ESOPHAGASTRIC VARICES Pharmacologic therapy Endoscopic therapy Tamponade Decompression radiologic, surgical Devascularization operation Liver transplantation TIPS

METHODS OF DECOMPRESSION FOR PORTAL HYPERTENSION AND GASTROESOPHAGEAL VARICES

Nonoperative, radiologic Portosystemic surgical shunts Total Portacaval (using portal vein) Mesocaval, mesorenal, mesoatrial (using superior mesenteric vein) Portacaval or mesocaval (interposition graft of restricted diameter to allow some prograde portal flow)

Fig. 12.11 Treatment strategies for esophagastric varices.

Partial

TIME POINTS IN THE TREATMENT OF ESOPHAGOGASTRIC VARICES Selective variceal decompression Prophylaxis to prevent the initial bleed Treatment of acute variceal bleeding Treatment to prevent recurrent variceal bleeding first-line treatment, second-line treatment Treatment of end-stage liver disease Distal splenorenal shunt

Fig. 12.13 Methods of decompression for portal hypertension and gastroesophageal varices.

Fig. 12.12 Time points in the treatment of esophogastric varices.

MANAGEMENT OF ESOPHAGOGASTRIC VARICES

Esophagogastric varices can be treated with a variety of therapies (Fig. 12.11).29 The time points of treatment become important in deciding upon the most appropriate therapy (Fig. 12.12). Each therapy is briefly reviewed and management schemes are presented for the different time points of presentation.

PHARMACOLOGIC THERAPY
The patient who has acute variceal bleeding may be treated initially with intravenous vasopressin and nitroglycerin30 or somatostatin or one of its analogs.31 In the elective situation, noncardioselective -blockers32 and the long-acting nitrates are the main drugs used to lower portal pressure. Acute pharmacologic therapy is most easily administered using octreotide infused at 50g/h. This agent is as effective as vasopressin and nitroglycerin given in combination, has fewer side effects and requires less monitoring. Vasopressin, administered by continuous intravenous infusion at 0.20.4units/min has been used for 30 years, but requires a concurrent infusion of nitroglycerin, which should be initiated at 40g/min and titrated to bring the systolic blood pressure to 90100mmHg (13.013.3kPa). These regimens can help prevent early recurrent bleeding in patients who have presented with an acute variceal bleed. The more recently introduced glypressin (available in Europe, but not in the USA) has gained considerable popularity because of its longer duration of action than vasopressin. Oral pharmacologic therapy was introduced in 1980 when it was demonstrated that portal pressure could be lowered by about 20% with propranolol. A similar effect can be obtained with the long-acting nitrates. The goal of oral pharmacologic therapy, which prevents variceal bleeding, is to: reduce the HVPG to 12mmHg (1.6kPa) or less; or achieve a 20% pressure reduction from the baseline portal pressure measurement. Other drugs that have been studied are the 2-agonists, calcium channel blockers, serotonin antagonists and prokinetic agents, but these have not shown clinical usefulness.29

sclerotherapy involves the use of one of several agents to directly thrombose a varix or to create brosis in the mucosa overlying a varix; and in banding the varix is sucked into an applicator on the end of the exible endoscope and a rubber band is red to strangulate the varix; the bands slough off in 710 days with minimal scarring and less ulceration than with sclerotherapy. Endoscopic ligation was tedious when rst instituted as each band had to be individually loaded at the end of the scope, but the introduction of multiple band ligators has rekindled interest in this method. Endoscopic banding has been shown to be more effective, resulting in earlier obliteration of varices and fewer complications than endoscopic sclerotherapy.29

TAMPONADE
This is rarely indicated in the 1990s, but may be needed for the 10% of patients whose acute bleeding is not controlled endoscopically. The Sengstaken Blakemore tube has both gastric and esophageal balloons. Attention to detail is essential in placing a tamponade tube. The tube must rst be fully introduced into the stomach. A small amount of air is then placed in the gastric balloon and a radiograph is taken to conrm its location. The gastric balloon should then be inated to 300350ml and pulled up into the gastric fundus. This will control virtually all bleeding. Occasionally, persistent bleeding may occur above the gastric balloon in the esophagus, requiring ination of the esophageal balloon to 40mmHg (5.3kPa) as tested through a sphygmomanometer. Conrmation of the position of the tube should be obtained radiologically when both balloons are inated. The tube should not be left in place for more than 1224 hours and should only be used as a temporary measure while the patient is resuscitated and prepared for either repeat endoscopic therapy or decompression.

DECOMPRESSION
This may be carried out by either radiologic or surgical methods and the aim is to reduce intravariceal pressure to less than 12mmHg (1.6kPa). This fall may be achieved either by total, partial or selective variceal decompression (Fig. 12.13). Total decompression of portal hypertension is achieved when an anastomosis that is over 12mm in diameter is made between the portal vein or one of its major tributaries and the inferior vena cava or one of its major tributaries. This procedure may be an end-to-side portacaval shunt or any type of side-toside anastomosis. A partial portosystemic shunt is achieved between these vessels with any type of side-to-side anastomosis that is 810mm in diameter. These shunts will reduce portal pressure to approximately 12mmHg (1.6kPa) and control variceal bleeding. Pathophysiologically, the same outcome can be achieved radiologically by a TIPS. Transjugular intrahepatic portal systemic shunt (TIPS) has become the most popular method for decompression in the 1990s and its effectiveness is currently

ENDOSCOPIC THERAPY
Endoscopic therapy for variceal bleeding was initially introduced in 1939 using the rigid esophagoscope, revived in the 1970s and assured of its place in the treatment of variceal bleeding with the introduction of exible endoscopy. It has a denite role in acute variceal bleeding and is the rst-line therapy in preventing rebleeding. The two main techniques are sclerotherapy33 or variceal banding:34

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PORTAL HYPERTENSION

being investigated by prospective randomized controlled trials. It is a side-toside portosystemic shunt and acts as a total or partial shunt depending upon its diameter, as outlined above. Selective variceal decompression is usually achieved by a distal splenorenal shunt, which selectively decompresses gastroesophageal varices through the spleen and splenic vein to the left renal vein; portal hypertension is maintained in the superior mesenteric and portal vein to maintain portal ow to the cirrhotic liver. The difference in these types of variceal decompression lies in the degree of maintenance of portal venous perfusion. Loss of portal ow accelerates liver failure and increases the rate of encephalopathy. Each of these methods of decompression are discussed in more detail below.

TRANSJUGULAR INTRAHEPATIC PORTAL SYSTEMIC SHUNTS

Shunts have evolved over two decades. Initial animal studies13 demonstrated the feasibility of connecting the portal and hepatic veins. Angioplasty techniques developed in the 1970s and 1980s allowed a larger track to be made through the liver.35 Finally, the development of expandable metal stents in the mid 1980s made it possible to keep the track open (see Fig. 12.14).36 It takes about 12 hours to place a TIPS, and this is usually carried out under conscious sedation. The right internal jugular vein is cannulated and either the right or middle hepatic vein is accessed. Coaxial catheter systems are used to maintain access to a major hepatic vein and other catheters are advanced: initially to traverse the hepatic parenchyma; then to access the portal venous system; and nally to allow the advancing balloon-dilating catheter into the track. When the track has been dilated, a metal stent can be deployed. The most popular stent is the Wallstent, which is self-expanding and is usually dilated up to 1012mm. Attention to detail in placing the stent is important as it shortens during expansion. There must be sufcient but not excessive length at the portal and hepatic venous ends. The stent should not be advanced too far into the portal vein or protrude from the hepatic vein up into the suprahepatic vena cava. Once the stent has been placed, the pressures in the portal vein, the stent and the right atrium are measured. The goal is to reduce the portal-to-atrial pressure gradient to less than 12mmHg (1.6kPa). Contraindications to TIPS are hepatoma, polycystic liver and right heart failure. The major complication is hemorrhage from either perforation of the liver capsule or the portal venous system outside the liver. Major complications occur in less than 2% of patients.

of the vein to the infrahepatic inferior vena cava or anastomosis using an interposition graft. Exposure for these operations is achieved through a long right subcostal incision, with the patient rotated right side up. The portal vein is approached by mobilization of the duodenum and retraction of the hepatic artery and bile duct to the left. The portal vein needs to be isolated over an adequate distance to allow safe clamping for anastomosis. The inferior vena cava also needs to be mobilized sufciently to complete the anastomosis safely. The need to use an interposition graft depends upon the size of the caudate lobe, which is often hypertrophied in patients who have cirrhosis. A graft may be needed if the two vessels are separated by an enlarged caudate lobe. Mesocaval, mesorenal and mesoatrial shunts involve isolating the superior mesenteric vein just below the pancreas.38 The vein is identied by lifting up the transverse colon and dissecting at the base of the mesocolon, following the middle colic vein to the superior mesenteric vein. It is then freed over sufficient distance for safe anastomosis. The decision about whether to anastomose it to the inferior vena cava or left renal vein is based on the surgeons preference for exposing either of these structures. To isolate the inferior vena cava, the third and fourth portion of the duodenum need to be mobilized sufciently and pushed cranially to expose an adequate length of inferior vena cava. An interposition graft is required and can either be made with a 1418mm ringed polytetrauoroethylene (PTFE) or an internal jugular vein graft. A mesoatrial shunt may be required if the intrahepatic inferior vena cava is obstructed.

PARTIAL PORTOSYSTEMIC SHUNTS

These may be placed in either the portacaval (Fig. 12.16) or mesocaval position. If the graft diameter is reduced to 8mm with ringed PTFE grarft, the portal venous system is only partially decompressed, allowing some maintenance of prograde portal ow.39 At this size, the pressure is reduced to approximately 12mmHg (1.6kPa), which is sufcient to control variceal bleeding. The operative exposure is the same as described above for total shunts.

SELECTIVE VARICEAL DECOMPRESSION

This is most commonly achieved by a distal splenorenal shunt (Fig. 12.17).40 Exposure is obtained through a bilateral subcostal incision, opening the gastrocolic omentum, and then mobilizing and elevating the inferior border of the pancreas. The splenic vein is identied and isolated from the back of the pancreas right up to its junction with the superior mesenteric and portal vein. The vein is divided at this point and sufficient vein is freed from the back of the pancreas to allow it to be moved down to the left renal vein without kinking. The second component to this operation is portalazygos disconnection, which involves ligation of the left gastric vein at its entry into the portal or splenic vein and again just above the pancreas. More complete portalazygos

TOTAL PORTOSYSTEMIC SURGICAL SHUNTS

These are most commonly portacaval shunts (Fig. 12.15).37 These require dissection of the portal vein at the hepatic hilus and either direct anastomosis

TRANSJUGULAR INTRAHEPATIC PORTAL SYSTEMIC SHUNT Inferior vena cava Shunt Hepatic vein Esophagus Liver Stomach Varices Spleen

SIDE-TO-SIDE PORTACAVAL SHUNT

Cystic duct Common bile duct Stomach Pylorus Portal vein Inferior vena cava Duodenum

Portal vein Superior mesenteric vein Coronary vein

Splenic vein

Fig. 12.14 TIPS. The metallic shunt maintains the transparenchymal track, which has been made through the liver between the portal and hepatic veins.

Fig. 12.15 Side-to-side portacaval shunt with direct vein-to-vein anastomosis. The portal vein is dissected from the right side, posterior to the bile duct. The intrahepatic vena cava is fully mobilised. If there is a larger caudate lobe, an interposition graft may be used.

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GASTROINTESTINAL SURGERY

disconnection can be achieved by totally isolating the splenic vein from the pancreas41,42 and meticulously ligating the right gastric and gastroepiploic veins.

DEVASCULARIZATION PROCEDURES
These have the components of splenectomy, gastric and esophageal devascularization, and esophageal transection, with the goal of reducing inow to bleeding varices (Fig. 12.18).43,44 Their success depends upon the extent of the devascularization: the more extensive, the lower the subsequent rebleeding rate. The operative approach requires sufcient exposure of the upper abdomen and left upper quadrant to perform a safe splenectomy and to immobilize fully both the stomach and at least 7cm of the distal esophagus. Particular attention needs to be paid to the posterior aspect of the distal esophagus where there are large penetrating veins from the paraesophageal venous plexus. The upper two-thirds of the lesser curve of the stomach should be devascularized in a manner similar to that performed during highly selective vagotomy. The whole of the greater curve should be devascularized from the pylorus to the gastroesophageal junction. The role of esophageal transection is controversial because most patients have had extensive sclerotherapy and the thickened esophagus does not lend itself to stapled transection. Most surgeons therefore now omit this component of the procedure.

a recent meta-analysis indicates that sclerotherapy cannot be recommended for prevention of a rst bleed.29 Further randomized controlled trials of bleeding prophylaxis in high-risk patients may alter this recommendation.46 Pharmacologic therapy to prevent the rst bleed has been most widely studied using -blockers. Among nine prospective randomized trials, the risk of bleeding was reduced in seven and meta-analysis showed a signicant decrease in the risk of bleeding for patients receiving -blockers.29 Side effects, however, occur in approximately 20% of patients. An alternative pharmacologic option is isosorbide-5-mononitrate, which has been shown to have a similar hemodynamic effect and clinical efcacy as that of -blockers.47

ACUTE VARICEAL BLEEDING

This requires a team approach in management. The general measures for managing patients who have portal hypertension and a major variceal bleed differ from those of the typical volume resuscitation for other bleeding patients. If volume resuscitation is carried out in the conventional manner with saline and/or Ringers lactate, the patient will rapidly develop ascites and/or edema. For patients who have cirrhosis, crystalloid administration should be limited. Resuscitation for patients who have established liver disease requires blood replacement and blood products. Other important measures in these patients include clearing the gastrointestinal tract of blood, protecting the airway and making an early diagnosis using endoscopy. In the patient who has advanced liver disease, gastrointestinal bleeding can easily precipitate encephalopathy because blood in the gastrointestinal tract represents a heavy protein load. Patients who have cirrhosis are susceptible to infection so intravenous antibiotics should be given as a component of acute phase management. Gastrointestinal tract catharsis should be achieved using magnesium citrate or polyethylane glycol 3350 (Go-Lytely); if the patient is comatose, enemas may be required. Most patients should be monitored in the intensive care unit for at least 24 hours or until they are hemodynamically stable. The need for more invasive intravascular monitoring with arterial lines, central venous catheters and pulmonary artery catheters is decided on a case-by-case basis. Urine output should be monitored hourly. Patients who have cirrhosis and upper gastrointestinal bleeding should have an endoscopy as soon as they are hemodynamically stable. This investigation is both diagnostic and potentially therapeutic. If an active bleeding site is identied or there are stigmas of a recent bleed, such as a platelet plug on a varix, the endoscopist should proceed directly to sclerotherapy or banding. This maneuver will control bleeding in approximately 90% of patients. The goals

LIVER TRANSPLANTATION
This is part of the surgical repertoire of managing patients who have portal hypertension and variceal bleeding. The indication for transplant remains endstage liver disease, and for Childs class C patients this treatment has altered the long-term outcome. Liver transplantation is dealt with in more detail in Chapter 6.5, so the technical aspects are not discussed here.

MANAGEMENT OF VARICEAL BLEEDING AT DEFINED TIME POINTS

PROPHYLACTIC THERAPY
Treatment should be initiated before the rst variceal bleed because prophylactic therapy can reduce the risk of an initial bleed from 30 to 20% and the mortality rate of that rst bleed is approximately 30%. Most of the treatments discussed above have been used for prophylaxis, but in the 1990s the recommended treatment is pharmacologic therapy.29 Total portosystemic shunts were tested in clinical trials in the 1960s and although the rst bleed was prevented there was an increased mortality rate in the surgical groups from encephalopathy and liver failure.45 No studies have looked at the role of TIPS in the prophylactic setting. Sclerotherapy has been extensively studied in clinical trials as a treatment modality to prevent the initial bleed. These trials have shown mixed results and

DISTAL SPLENORENAL SHUNT PARTIAL PORTOSYSTEMIC SHUNT PARTIAL PORTOSYSTEMIC SHUNT

Splenic vein

Portal vein 8mm PTFE graft Vena cava Left renal vein

3
12.8

Fig. 12.16 Partial portosystemic shunt with an 8mm graft between the portal vein and inferior vena cava. Exposure is the same as for the total portacaval shunt.

Fig. 12.17 Distal splenorenal shunt, with anastomosis of the splenic to the left renal vein. The gastric fundus, esophagus and spleen are decompressed. Portal hypertension and prograde portal ow are maintained.

PORTAL HYPERTENSION

GASTROESOPHAGEAL DEVASCULARIZATION

First-line treatment of prevention of rebleeding is initially the same irrespective of the severity of the underlying liver disease, involving a combination of pharmacologic and endoscopic therapy. This therapy buys time to evaluate the liver disease fully, educate the patient about the disease and assess the likely course and the chance of reversibility of the underlying liver disease.

First-line treatment using pharmacologic and endoscopic therapy

This aims to reduce the risk of rebleeding from 7580% to 2530%. The highest risk time of rebleeding is within the rst 6 weeks, necessitating an aggressive early treatment policy. Endoscopic therapy is based on band ligation, which should aim to obliterate esophageal varices over 46 weeks. Current data indicate that this goal can be achieved with 34 ligation sessions. Patients should be started on a noncardioselective -blocker (propranolol or nadolol) at a dose sufcient to produce -blockade.29 The objective is to obtain a 20% reduction in HVPG or an HVPG of no more than 12mmHg (1.6kPa), but this is impractical for all patients; a target of reducing the pulse rate by 20% is usually sufcient. Outcomes from this dual approach to primary therapy indicate that the rebleeding rate can be reduced to 2530%. Data that compare banding and sclerotherapy show that banding results in a signicantly lower rebleeding rate, but that the overall mortality rate is not signicantly reduced.49 However, banding is the favored endoscopic method because fewer treatment sessions are required to achieve variceal obliteration and there are fewer side effects. Trials continue to evaluate the role of combination pharmacologic therapy and combination endoscopic and pharmacologic therapy in the search for an optimal regimen.

Anterior vagus nerve

Fig. 12.18 Gastroesophageal devascularization interrupts all variceal inow to most of the stomach and the distal 7cm of the esophagus. Splenectomy and desvascularization of the greater curve of the stomach complete the procedure.

Second-line therapy to prevent recurrent bleeding

This should be considered for patients who rebleed during primary therapy. The decision to move to second-line treatment can be difcult due to timing, severity of the rebleeding and the status of the liver disease. The severity and rate of progression of the underlying liver disease are the major factors in the decision. Does the patient need a transplant now or is he or she likely to need a transplant in the next few years? If so, the decision for the best bridge to transplantation depends upon the patients current liver function. For the patient who has well-preserved hepatic function (Childs A and Childs B), decompression may be preferable to transplantation. For the Childs C patient, the bridge to transplantation is shorter and the rebleeding may be the event that leads to transplantation. The other major dilemma is the severity of the rebleed and its timing during the course of rst-line treatment. The managing physician is faced with a question of how much rebleeding is acceptable during rst-line treatment. For the patient who is rebleeding early because of failure to obliterate varices, there should be an expeditious move towards decompression. For the patient who has had an initial successful obliteration of varices and has a minor rebleeding episode 12 years later, it may be reasonable to repeat the endoscopic therapy. Decisions on optimal decompression can be based on outcomes from available studies. Data on TIPS are based on several large series and the initial set of randomized controlled trials comparing TIPS to endoscopic therapy.5056 The procedure is technically successful in 93100% of patients. Reported 30-day mortalities are in the range of 1416%, most deaths occurring in Childs class C patients as a result of progressive liver disease and multisystem organ failure. The major problem with TIPS has been stenosis, either midstent or at the hepatic vein, which occurs in 5075% of patients by 1 year. Stenosis has led to rebleeding rates of 20% at 1 year. Careful monitoring with Doppler ultrasound of the TIPS, with invasive pressure measurements and redilation if the ow pattern changes, has led to primary assisted patency rates of 8385% at 1 year and will probably allow better control of bleeding. Encephalopathy rates are 2530% higher than pre-TIPS rates, but in most instances this is not a debilitating problem and is readily managed with protein restriction and lactulose. In the series with the longest follow up, reported 3-year survival rates are 75% for Childs A and 68% for Childs B patients.

of this initial endoscopy are therefore to exclude other pathologies as the source of bleeding and to initiate rst-line therapy for the varices.

Should patients receive concomitant pharmacologic therapy?

Opinion is divided on this question as the highest risk time for rebleeding is in the following week. Administration of a vasopressinnitroglycerin combination or octreotide may reduce the risk of this early rebleed. There are no randomized trials to support this view, but many centers do give pharmacologic therapy.

Balloon tamponade
This is reserved for patients in whom the acute bleeding is not controlled by endoscopic therapy (i.e. 10% or less of all patients). Management requires a strict protocol as outlined above and the tube should not be left in place for longer than necessary to move to the next level of treatment. Pressure on the mucosa from the tamponade leads to further damage and the risk of early rebleeding.

Decompression
This may be required for the patient who has required balloon tamponade or for the patient who rebleeds soon after endoscopic therapy, particularly from gastric varices. A TIPS will usually control the bleeding and is the next step, particularly for the poor-risk (Childs C) patient.48 Occasionally there is a place for selective variceal decompression in the good-risk patient who is readily stabilized during episodes of rebleeding. Acute surgical intervention in the patient who has acute bleeding is advocated by Orloff et al.37 who have an excellent outcome with emergency portacaval shunt for all-comers admitted to their unit with acute variceal bleeding. These data have not been duplicated elsewhere.

PREVENTION OF RECURRENT VARICEAL BLEEDING

The essential step in making management decisions to prevent recurrent variceal bleeding is patient evaluation as outlined above. Once the patient has been stabilized from the acute variceal bleed, this evaluation should be completed within 4872 hours, focusing on the varices themselves, the intra-abdominal venous vasculature and the underlying liver disease. The principles for prevention of rebleeding can be summarized as: rst-line treatment with pharmacologic and endoscopic therapy; second-line treatment with variceal decompression; and liver transplantation for end-stage liver disease.

Surgical decompression
Surgical decompression to prevent recurrent bleeding is associated with rebleeding rates of less than 10%, but is used relatively infrequently because of the use of endoscopic therapy and transplantation, and more recently TIPS. However, the excellent control of bleeding and long-term durability of surgical shunts may lead to a renewed interest stimulated by the resurgence of interest in decompression using TIPS. Is one shunt superior to the others in controlling bleeding?

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Each type of shunt has a bleeding control of more than 90%. The difference between the types of shunts is in the rate of encephalopathy. Total shunts are associated with a higher rate of encephalopathy than partial or selective shunts. Data from the many randomized controlled trials that have compared shunts show that:5764 bleeding is controlled in 90% of patients after operative decompression; encephalopathy occurs in 1015% of patients after a distal splenorenal shunt, 1020% of patients after a partial shunt and 3040% of patients after a total shunt; and survival is not signicantly different for any of the shunt procedures. Review of the data on surgical shunts in uncontrolled series in the 1990s supports these generalizations.6571 The operative mortality rate is 5% or less and bleeding control is excellent. The risk of complications and death is related to the underlying liver disease. Patients who have well-maintained liver function do well with any type of decompressive procedure, at least in the short term. Total portosystemic shunts have been championed by Orloff et al.,37,70 who have had an excellent outcome overall. Reviewing a 30-year experience shows that recently there has been an improved survival; bleeding control has been excellent at every time point. Orloff attributes much of his success to overall patient care, rehabilitation and intensive follow up. Another recent series of total portosystemic shunts comes from Stipa et al.71 who have documented excellent bleeding control, but a 50% rate of encephalopathy and liver failure. Partial shunts have been championed in the past decade by Sarfeh and colleagues,39,63 building on the work of Bismuth et al.72 and Johansen.73 Bleeding has been controlled in over 90% of patients, with shunt stenosis occurring in 1020%. These series have also documented maintenance of portal perfusion in over 80% of patients if the graft is reduced to 8mm in diameter. The encephalopathy rate is approximately 15% in these uncontrolled series. Selective shunts have been used most commonly in the 1990s worldwide.6569 Bleeding control of over 90% has been achieved, with encephalopathy in the 1015% range. Various modications of selective shunting have involved portalazygos disconnection or pancreatic disconnection and the ability to maintain selectivity has been evaluated; selectivity is best achieved in patients who have nonalcoholic portal hypertension. Survival in these series is primarily determined by the severity of the underlying liver disease. Decompression of varices in the 1990s is reserved as a second-line treatment for patients who have failed to respond to endoscopic and pharmacologic therapy. It is not clear whether to use TIPS or a surgical shunt and this is being investigated by many ongoing prospective randomized trials. A preliminary report of one trial comparing TIPS to a partial shunt shows no significant difference between the procedures.74 Liver transplantation is indicated for patients who have end-stage liver disease and in approximately 30% of these patients variceal bleeding may be a component of that end-stage disease.65,7577

MANAGEMENT OF EXTRAHEPATIC PORTAL VEIN THROMBOSIS

The management of patients who have variceal bleeding secondary to extrahepatic portal vein thrombosis differs in important aspects from the management of patients who have cirrhosis. As these patients have a normal liver, the emphasis is on controlling the bleeding. In addition, the etiology of the portal vein thrombosis should be identified as this may indicate the need for other hematologic management. The evaluation of these patients follows the same principles outlined above. Angiography is more important for this group of patients than for those who have cirrhosis, as accurate identication of the vessels that are still open will dictate further therapy. Cavernous transformation of the portal vein can produce a confusing picture on Doppler ultrasound, particularly if there is one dominant large collateral, which may be mistaken for the portal vein. The picture can usually be clarified with adequate angiography (Fig. 12.20) and the patency or occlusion of the superior mesenteric and splenic veins are key factors in management decisions. A liver biopsy is often required to document a normal liver. Management of this group of patients is initially by endoscopic therapy, but if the varices are not readily obliterated and there is rebleeding, there should be a low threshold for proceeding to definitive decompression.79,80 Of these patients 80% have a patent splenic vein and are ideal candidates for a distal splenorenal shunt. Bleeding is controlled by the shunt and portal perfusion is maintained through cavernous transformation to the normal liver. Total portosystemic shunts have been used in the group of patients using either central splenorenal or mesocaval shunts. The classic mesocaval shunt used for portal vein thrombosis divides the inferior vena cava just above its bifurcation and swings the inferior vena cava up in an end-to-side fashion to the superior mesenteric vein.81 There has been controversy as to the long-term outcome of such total shunts. Portosystemic encephalopathy has been documented at 1020 years in this group of patients82 and this has been conrmed by other reports. However, there is conicting evidence from other investigators,83 who have found no late development of portosystemic encephalopathy. Devascularization procedures may be indicated for this group of patients if there are no shuntable vessels. The operation should follow the same principles as those outlined above for extensive gastroesophageal devascularization. An added caveat is that splenectomy may unmask an underlying myeloproliferative disorder in adult patients who have portal vein thrombosis. A normal

MANAGEMENT OF VARICEAL BLEEDING Varices Prophylaxis Acute variceal bleed Acute Evaluation Endotherapy, somatostatin, TIPS -blocker

Effect of the treatment of portal hypertension on subsequent liver transplantation

First-line treatment has no impact on subsequent liver transplantation, but there may be some thickening around the gastroesophageal junction from endoscopic therapy. No data have documented an increased rate of portal vein thrombosis with endoscopic therapy.78 Decompressive procedures may, however, have an impact on transplantation. Careful placement of a TIPS is important because if it is placed too far down the portal vein or extrudes into the suprahepatic inferior vena cava it may be technically challenging to remove the stent at the time of transplant. The effect of surgical shunts on subsequent liver transplant has been extensively reviewed, and in summary although the operative procedure may be more complex and entail a higher blood loss, the overall outcome is not influenced by the presence of a previous surgical shunt. As a general guideline, a pretransplant arteriogram is helpful to dene the full anatomy and any portal venous changes that may have occurred as a result of the previous surgical shunt. An overall scheme for the management of variceal bleeding can be summarized in an algorithm (Fig. 12.19). The absolute ordering of therapies may differ between centers depending upon available expertise, but all centers should have in common general concepts of therapy for: prophylaxis; acute bleeding; and preventing recurrent hemorrhage.

Good liver function

Poor liver function

Recurrent

Endotherapy -blocker

Rebleed

Progressive disease

DSRS or TIPS

Transplant

Fig. 12.19 Management of variceal bleeding. DSRS, distal splenorenal shunt.

PORTAL HYPERTENSION

platelet count in the face of massive splenomegaly should trigger a careful hematologic evaluation before proceeding to splenectomy.

ASCITES
Ascites is a sign of decompensation in liver disease and so has a much more sinister signicance than variceal bleeding. Because it is a visible manifestation of severe underlying liver disease to both the patient and the physician, it evokes the response to treat it aggressively. This temptation should be resisted as iatrogenic complications resulting in azotemia, hypokalemia, hyponatremia and encephalopathy can easily be precipitated. The most ominous complications of ascites are spontaneous bacterial peritonitis and hepatorenal syndrome, which carry a high mortality rate.

PATHOGENESIS
Ascites results from a combination of:84 portal hypertensio; altered renal sodium and water handling; and hypoalbuminemia. It forms as a hepatic lymph from the surface of the liver as a result of uid exuding from the damaged, high venous pressure sinusoids of cirrhosis. The kidneys role in pathogenesis is through reduction in renal blood flow, decreased glomerular filtration and decreased effective plasma volume. Hypoalbuminemia decreases the intravascular oncotic pressure and further contributes to ascites formation.

sodium reabsorption has been adequately blocked by an aldosterone antagonist, further diuresis may be required using a loop diuretic such as furosemide (frusemide). The goal should be a gentle diuresis so that the patient loses up to 2kg/week rather than seeking more rapid fluid mobilization, which will precipitate other complications. If ascites is refractory to medical management, the next line of therapy is paracentesis. Large-volume paracentesis can be safely performed with minimal risk and the patient who requires this once a month may nd this treatment very acceptable. If large-volume paracentesis is needed on a weekly basis other therapeutic interventions should be considered. The primary surgical treatment for intractable ascites is liver transplantation because the patient has decompensated liver disease. However, for patients who are not transplant candidates or for those who are awaiting transplant, other interventions may be indicated. Peritoneovenous shunts have largely disappeared from the repertoire of options for these patients because they thrombose easily, carry the risk of infection and have been shown to precipitate variceal bleeding. A TIPS has now become the decompressive option for patients who have intractable ascites.86 This side-to-side shunt decompresses the sinusoids and can greatly improve ascites. Experience is relatively limited, but several reports indicate that most patients have a partial response at least, and some have a complete response. Two prospective randomized controlled trials have supported the use of TIPS for patients who have intractable ascites, but because of the advanced liver disease the treatment is not without complications in the form of bleeding and spontaneous bacterial peritonitis.

BUDDCHIARI SYNDROME
EVALUATION
This requires analysis of ascitic uid obtained by paracentesis and assessment of renal function. Ascitic uid should be examined for cell count, culture and total protein and albumin content. Measurement of the serum:ascites albumin gradient can help differentiate the etiology of ascites. Renal function is assessed primarily from urine and serum electrolytes and blood urea nitrogen and serum creatinine. Further management requires a 24-hour urine creatinine clearance. BuddChiari syndrome encompasses a group of disorders characterized by hepatic venous outflow obstruction. The severity of the syndrome may vary from mild nonspecic upper abdominal symptoms to a fulminant course with severe hepatic necrosis. Most patients present with a subacute form that is progressive over weeks to months.

ETIOLOGY MANAGEMENT
Ascites is managed primarily by medical therapy.85 Dietary sodium restriction and diuretics form the first-line treatment. Spironolactone is the first-choice diuretic to counter the secondary hyperaldosteronism of chronic liver disease. This drug blocks tubular sodium reabsorption and allows further diuresis. Once There is usually some other underlying pathology that leads to hepatic vein thrombosis.87 The most common etiologies are summarized in Figure 12.21. The vast majority reflect an underlying hematologic disorder, polycythemia vera being the most common.88 Other conditions such as tumors causing pressure on the hepatic veins are important to identify because of their exceedingly poor prognosis. Membranous webs of the suprahepatic vena cava are uncommon in the USA or Europe, but are a frequent cause of BuddChiari syndrome in the Far East.

PATHOPHYSIOLOGY
This is the same regardless of etiology.87 The sequence of outow obstruction, sinusoidal dilatation and congestion, hepatocyte necrosis from the increased pressure and progressive liver damage produce the syndrome. The process frequently involves only one or two of the three main hepatic veins, therefore leading to differential involvement of the main liver lobes. The extent of involvement dictates the degree of liver damage. If only one hepatic vein is initially involved, the symptoms may be relatively mild and simply lead to scarring and brosis of that lobe. However, the sequence then usually repeats itself,

CAUSES OF THE BUDDCHIARI SYNDROME Cause Myeloproliferative disorder Idiopathic Tumor Estrogen use, pregnancy Hypercoagulable state Proportion of cases (%) 50 20 10 10 5 5

Fig. 12.20 Portal vein thrombosis with cavernous transformation (arrow) and several larger collateral veins carrying portal blood to the low pressure sinusoids.

Paroxysmal nocturnal hemoglobinuria

Fig. 12.21 Causes of the BuddChiari syndrome.

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GASTROINTESTINAL SURGERY

involving one of the other main hepatic veins and leading to progressive liver damage and scarring. The caudate lobe of the liver is spared from this damage because it has a separate drainage with vessels passing directly into the inferior vena cava and may therefore undergo considerable hypertrophy.

and splenomegaly. Routine hematologic evaluation (including a blood smear) should be augmented with bone marrow examination, leukocyte alkaline phosphatase measurement, platelet aggregometry and specic tests for protein C, protein S, antithrombin III and factor V Leiden.

CLINICAL PRESENTATION
This is usually subacute with relatively mild symptoms and signs of ascites, hepatomegaly and right upper quadrant abdominal pain. Other stigmas of chronic liver disease are usually absent and standard liver tests are only minimally abnormal. As BuddChiari syndrome is uncommon, the diagnosis is frequently missed. Patients are often thought to have a gynecologic or gastrointestinal malignancy or an intra-abdominal inammatory process. An awareness of the syndrome together with a high index of suspicion when a patient presents with ascites that does not t a classic pattern can lead to the correct diagnosis.

MANAGEMENT
The management of BuddChiari syndrome is dictated by the liver biopsy ndings87 and is summarized in Figure 12.23. The options are: nonsurgical management; surgical decompression; and liver transplantation. If the liver biopsy shows congestion, but no histologic evidence of ongoing necrosis, the patient may be managed by symptomatic management of the ascites and treatment of any underlying hematologic disorder. Embarking on this course mandates careful follow up, with a repeat liver biopsy approximately 3 months later to ensure that there is no further liver damage with hepatocellular necrosis.87 If the biopsy shows an acute injury with ongoing hepatocellular necrosis, sinusoidal decompression is indicated. This procedure usually requires a sideto-side portosystemic shunt, thereby using the open portal vein as a decompressive route from the obstructed sinusoids. This can be achieved by: TIPS;89,90 side-to-side portacaval shunt;91,92 and mesoatrial shunt if the inferior vena cava is obstructed.87,93 The decision to use a decompressive shunt requires a full assessment of which of these three options is needed to achieve sinusoidal decompression. Evaluation of the inferior vena cava is key in making this decision and this involves venographic imaging and pressure measurements. Liver transplantation is indicated for patients who have advanced disease, usually those who have had repeated thrombotic episodes with progressive brosis and cirrhosis.87,9496 Transplant is technically difcult in these patients because of the massive collaterals to the diaphragm, which make hepatectomy hazardous. Management of the hematologic disorder must parallel the therapy for liver damage. It is important that the hepatologist, the surgeon and the hematologist all work together in evaluating these patients and making appropriate combined treatment decisions.
MANAGEMENT OF BUDDCHIARI SYNDROME

INVESTIGATION
This depends upon the use of appropriate laboratory studies, radiologic imaging and liver biopsy.87 Laboratory studies are often minimally disturbed in BuddChiari syndrome, with mild elevations of the bilirubin, transaminases and alkaline phosphatase. These laboratory abnormalities are disproportionately mild when compared with the damage frequently seen on biopsy. Analysis of the ascites usually shows a serum:ascites albumin gradient greater than 1.1g/dl and a white cell count that is usually less than 100/mm3. Radiologic imaging initially depends upon Doppler ultrasound. The three major hepatic veins can usually be readily visualized in a normal subject and have a characteristic phasic ow pattern. Inability to demonstrate these veins or to show phasic flow should raise a suspicion of hepatic vein occlusion. Ultrasound scan may also indicate that the liver is enlarged. The portal vein should be imaged both for patency and for any dampening of portal ow suggestive of sinusoidal obstruction. Other radiologic imaging techniques such as hepatic scintigraphy, CT and MRI have been used, but although they can contribute to the diagnosis of BuddChiari syndrome, they are not rst-line diagnostic modalities. The gold standard of radiologic imaging is hepatic venography, which may be performed through either a transfemoral or a transjugular approach and all three hepatic veins need to be studied. The classic appearance is the spiders web, which reects the multiple collaterals attempting to drain the obstructed sinusoids (Fig. 12.22). Liver biopsy should be carried out concurrent with hepatic venography. Biopsies should be taken through all three hepatic veins or at their occluded locations. If this is not possible, differential biopsies of the right and left lobes should be made percutaneously. The axiom that no patient is too sick to biopsy is important, as further management decisions are based on biopsy findings. The essential features on liver biopsy are: centrilobular congestion; hepatocellular necrosis; and the degree of lobular collapse. These injuries may lead ultimately to brosis and cirrhosis. Hematologic evaluation is essential to look for an underlying hematologic disorder, which may be compounded by the development of portal hypertension

Ascites, abdominal pain, clinical suspicion of BCS

Doppler ultrasound No hepatic veins, lack of phasic flow, equivocal findings Normal hepatic veins with phasic flow

BCS excluded Angiography and liver biopsy

Normal

Severe fibrosis, cirrhosis

Necrosis, mild or no fibrosis

Congestion, no necrosis

Hematalogic evaluation

OLT

Side-to-side shunt

Treat ascites, follow-up biopsy and ultrasound

Fig. 12.22 Spider s web of BuddChiari syndrome as the sinusoids attempt to decompress to the liver capsule.

Fig. 12.23 Evaluation and management of BuddChiari syndrome. OLT, orthotopic liver transplantation.

PORTAL HYPERTENSION

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