Historical Background
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CHAPTER 108
Portal
Hypertension
Riccardo Superina
A man who thinks that a science can perform what is outside
its province, or that nature can accomplish unnatural
things, is guilty of ignorance more akin to madness than to
lack of learning. Our practice is limited by the instruments
made available by Nature or by Art. When a man is attacked
by a disease more powerful than the instruments of
medicine, it must not be expected that medicine should
prove victorious.
—Hippocrates
The treatment of portal hypertension in children is always
evolving as the nature of underlying disorders is better under-
stood. Treatment is often successful, Hippocrates notwith-
standing, and the strategy usually involves medical, surgical,
or combined therapy. This improved outlook is a departure
from past years because of the improved understanding of
the underlying pathophysiology. More effective imaging tech-
niques and a wider array of options in pharmacologic manage-
ment have resulted in opportunities to apply both established
and newer surgical techniques precisely and deliberately to
address underlying disease processes that cause portal
hypertension.
The Italian physician and pathologist Guido Banti first de-
scribed the relationship among splenic enlargement, anemia,
and cirrhosis of the liver. The first splenectomy done for
hypersplenism was done in Florence in 1903 on his advice.
The constellation of symptoms later became known as Banti
syndrome.1
Earlier, Nikolai Eck, a German anatomist, had produced a
connection between the portal vein and the inferior vena cava
in a dog.2 Later studies described the association between the
direct communication of portal and systemic venous blood
and the onset of encephalopathy associated with hyperammo-
nemia and protein ingestion.3–5
The first applications of portosystemic shunting in patients
were reported in the 1950s and 1960s. More selective shunt-
ing procedures were devised when it was discovered that
diversion of mesenteric blood flow into the systemic circu-
lation could precipitate severe encephalopathy. Nonshunt
procedures aimed at interrupting the blood flow to gastric
and esophageal varices were also described.
Finally, with the advent of liver transplantation, portal
hypertension caused by advanced liver disease was treated
by replacement of the diseased organ, rather than palliative
procedures that produced significant morbidity and often
hastened the demise of the patient.
Embryology and Anatomy
------------------------------------------------------------------------------------------------------------------------------------------------
The portal vein develops through a complex system involving
the formation and involution of primitive vessels, followed by
circulatory changes at birth that affect the flow of blood in the
mature portal circulation (Fig. 108-1).
The left and right omphalomesenteric veins drain the de-
veloping gut as it is formed from the yolk sac during the fourth
to sixth weeks of embryonic life. At the same time, the umbil-
ical veins transport blood from the placenta to the embryo.
Blood from both the umbilical and the omphalomesenteric
veins drains through the nascent hepatic sinusoids in the
developing liver and into the hepatic veins back to the devel-
oping heart. Some of the blood from the umbilical veins
bypasses the liver and drains into the sinus venosus through
the ductus venosus. Although the omphalomesenteric veins
communicate by a series of intermediary vessels early in
embryonic life, the right one involutes and disappears by
the sixth week of gestation. The left one develops into the
permanent portal vein, which drains the mesenteric venous
bed through the superior mesenteric vein and its branches.
The right umbilical vein also involutes, and the left remains
patent until shortly after birth, when it thromboses and
becomes the ligamentum teres.
The portal vein is formed by the confluence of the splenic
and superior mesenteric veins posterior to the head of the
pancreas. The coronary vein draining the gastric venous bed
inserts into the portal vein at or just distal to the splenomesen-
teric confluence, and the inferior mesenteric vein drains into
the splenic vein anywhere along its length, also behind the
pancreas.
1355
1356 PART VII ABDOMEN
Venae revehentes
Anterior detached
portions of umbilical
veins
Stomach
Venae
advehentes
Bileduct
Right umbilical
vein
Portal vein
Obliterated portions
Vitelline veina
of venous rings
FIGURE 108-1 The portal vein forms from the union of the two vitelline
veins draining blood from the yolk sac. The left vitelline vein joins with the
splenic vein to form the extrahepatic portion of the portal vein. The intra-
hepatic portal vein forms from the umbilical veins. In addition, the left
umbilical vein communcates with the sinus venosus, allowing placental
blood to bypass the liver during intrauterine life. The right vitelline and
umbilical veins involute.
Despite the relative variability in the anatomy of the liver’s
arterial supply, the portal vein anatomy varies little. The prin-
cipal extrahepatic vein divides into a left and a right branch.
The left branch supplies the left lobe as it courses under
the surface of segment IV of the liver; turns anteriorly in the
recessus of Rex among segments II, III, and IV; and ends by
dividing into the branches that supply those segments. The
right branch divides into the posterior and anterior sectoral
branches at or just before the liver plate at the capsule.
A cavernous venous malformation may be the result of a
disordered sequence of biologic steps. Thrombus in the
postnatal umbilical vein may also propagate into the hepatic
portal vein and occlude flow in the extrahepatic portion of
the vein. Both processes may lead to the syndrome of extra-
hepatic or prehepatic portal hypertension.
Definition
------------------------------------------------------------------------------------------------------------------------------------------------
Portal hypertension occurs whenever the resistance to the flow
of blood from the mesenteric venous circulation through or to
the liver increases. The causes of portal hypertension are most
commonly obstructive in nature. That is, there is an impedi-
ment to the forward flow of blood from the omphalomesen-
teric vascular bed through the liver and toward the heart.
However, portal hypertension can also be caused by an
increase in the volume of blood going to the liver or by a direct
communication between the arterial and venous supplies to
the liver that exposes the venous bed to abnormally high
pressures.
Portal hypertension may be defined as pressure in the
portal venous bed that exceeds 5 to 8 cm H2O or a pressure
gradient of more than 5 cm H2O between the hepatic veins
and the portal circulation. When the pressure in the portal
circulation exceeds those values, a series of physiologic
changes leads to the symptoms common to all forms of portal
hypertension, regardless of the cause.
Ductus
venosus Collateral Circulation
------------------------------------------------------------------------------------------------------------------------------------------------
Liver
As a consequence of the resistance to flow and the increase in
pressure in the mesenteric venous circulation that includes the
Pancreas superior mesenteric, inferior mesenteric, splenic, and coro-
nary veins, blood that normally goes to the liver from the por-
Left umbilical tal vein must find alternative or additional routes back to the
vein heart. The abnormal communications that form between the
mesenteric and systemic venous systems are called varices
Duodenum or “shunts” because blood is shunted away from the liver
and back to the heart. The hemorrhoidal plexus in the rectum,
the paraumbilical network between the portal vein and the
paraumbilical veins that form through the recanalization of
A. Boutwell
the umbilical vein and ligamentum teres (forming the “caput
medusa” around the umbilicus), and the communications in
the gastroesophageal area between the coronary and splenic
veins through the esophageal and paraesophageal veins back
to the hemiazygous system are the typical areas where the ab-
normal shunts between the systemic and mesenteric venous
systems occur.
Other locations of spontaneous shunts are between the
veins of the colon and duodenum and the left renal vein;
the accessory portal system of Sappey, whose branches in
the round ligament unite with the epigastric and internal
mammary veins through the diaphragmatic veins to unite with
the azygos vein; the veins of Retzius, which connect the intes-
tinal veins with the inferior vena cava and its retroperitoneal
branches; the inferior mesenteric veins and the hemorrhoidal
veins that open into the hypogastrics; and, rarely, the patent
ductus venosus, affording a direct connection between the
portal vein and the inferior vena cava.
Causes: A Spectrum of Disorders
------------------------------------------------------------------------------------------------------------------------------------------------
Portal hypertension can be divided into two categories:
(1) portal hypertension from hepatocellular injury and liver
fibrosis and (2) portal hypertension from primary vascular
causes (Table 108-1).
HEPATOCELLULAR INJURY
Table 108-2 illustrates some of the common liver disorders in
children that can lead to portal hypertension. Hepatocellular
injury from a variety of toxins leads to cell death, initiation and
progression of fibrosis, and ultimately cirrhosis. Progressive
injury to hepatic tissue and replacement of normal liver with
fibrous tissue lead to increased resistance to blood flow
through the liver. The process of deposition of collagen in re-
sponse to hepatocyte injury appears to be a complex process
mediated through the transcription of proinflammatory cyto-
kines such as osteopontin and through stellate cells.6 The stel-
late cell, a normal constituent of hepatic sinusoids, is the
primary source of excess extracellular matrix proteins in liver
fibrosis.7 It can change its phenotype from one that is fairly
quiescent during normal liver homeostasis to one that

TABLE 108-1
Classification of Portal Hypertension
Type Description
Hepatocellular Intrinsic liver disease with increased liver fibrosis
(see Table 108-2)
Vascular
Prehepatic Extrahepatic portal vein thrombosis
Cavernous transformation of portal vein
Extrinsic compression of portal vein
Posthepatic Budd-Chiari syndrome
Intrinsic web
Stenosis of hepatic vein orifice
High-flow Arteriovenous communication—intrahepatic or
(hyperkinetic) extrahepatic
Congenital
Acquired
TABLE 108-2
Hepatocellular Diseases Leading to Portal Hypertension
Biliary atresia
Postinfectious cirrhosis
Congenital hepatic fibrosis
Congenital disorders of bile acid metabolism
Sclerosing cholangitis
Autoimmune hepatitis
Drug toxicity
Metabolic diseases (e.g., alpha-1 antitrypsin deficiency)
undergoes myofibroblastic differentiation or activation during
episodes of hepatocyte injury. Dysregulation in the activity
and number of stellate cells through faulty apoptosis or per-
petuation of the insult that led to hepatocyte injury leads to
ongoing deposition of proteins in the extracellular compart-
ment of the liver. Ultimately, excess deposition of collagen
in sinusoids results in portal hypertension.
VASCULAR CAUSES
Prehepatic Portal Hypertension
Prehepatic portal hypertension is caused by obstruction at the
level of the portal vein proximal to its branching at the liver
plate. Extrahepatic portal vein thrombosis has been recog-
nized and treated in both adults and children; it may be a con-
genital lesion or acquired at a later time. Even though the
symptoms may be dramatic, patients, particularly children, tol-
erate the bleeding from varices well because of the well-
preserved hepatic function.8 Occlusion of the main trunk of
the portal vein may lead to recanalization of the vein and its
transformation into a series of smaller collateral veins that as-
sume the appearance of a venous cavernoma visible on ultraso-
nography or CT scanning (Fig. 108-2). In most instances the
intrahepatic architecture of the portal vein is preserved.
Thrombosis of the portal vein is associated with omphalitis, in-
strumentation and cannulation of the umbilical vein at birth in-
cluding umbilical vein catheters for intravenous access,9,10
sepsis and dehydration in infancy, and mass lesions that exert
CHAPTER 108 PORTAL HYPERTENSION 1357
S
FIGURE 108-2 Preoperative computed tomography scan of a child with
extrahepatic portal vein thrombosis showing the typical cavernoma, the
tangle of varices in the hilum of the liver (white arrow) that replaces the
normal portal vein. Also of note is the typically small size of the intrahepatic
portal vein branches (black arrow) and the large spleen (S) that appears
larger than the liver.
extrinsic compression on the vein. These lesions may be inflam-
matory or malignant; examples include reactive enlargement of
the lymph nodes in the hilum of the liver from histoplasmosis
or compression from non-Hodgkin lymphoma involving the
hilum of the liver.
Unlike children with preexisting liver disease, children
with extrahepatic portal vein thrombosis are usually com-
pletely well before the sudden onset of symptoms. More than
50% of children present with unheralded hematemesis or
hematochezia from gastroesophageal varices.11 Others are di-
agnosed with splenomegaly of unknown cause and may be re-
ferred to an oncologist for suspected hematologic malignancy.
Posthepatic Portal Hypertension
Posthepatic portal hypertension is caused by occlusion of
large or small veins draining blood from the liver into the in-
ferior vena cava. Obstruction at this level leads to passive con-
gestion of the liver and necrosis of the hepatocytes in the
central areas of the hepatic lobule. Acute venous obstruction
may lead to massive hepatic necrosis and cell death unless it is
relieved in an urgent fashion. The Budd-Chiari syndrome,
sometimes referred to as Chiari disease, was first described
in 1845.12,13 It is uncommon in children.14 Outflow occlu-
sion is caused by congenital venous webs in the hepatic veins,
hydatid disease,15 myeloproliferative diseases, hypercoagu-
lable states, and increased estrogen levels, most commonly
from oral contraceptives. It can also occur after liver transplan-
tation (Fig. 108-3).
Microvascular nonthrombotic occlusion of hepatic venules
is termed venoocclusive disease, and it is being seen with in-
creasing frequency in patients following bone marrow trans-
plantation.14,16 Cytoreductive regimens involving busulfan
and cyclophosphamide are thought to induce obliterative
phlebitis in the small veins of the liver, and damage to the en-
dothelial cells is induced through the depletion of endogenous
1358 PART VII ABDOMEN
FIGURE 108-3 Acute hepatic venous obstruction after split liver trans-
plantation, caused by a blood clot in the left hepatic vein of a segment
II-III transplant that resulted in hepatic necrosis. The child was
retransplanted.
glutathione S transferase stores within the cells.17 Other
causes of venoocclusive disease such as ingestion of herbal teas
containing pyrrolizidine alkaloids have been described. Other
diverse agents including contrast media, estrogen, and thiogua-
nine have also been associated with the development of venooc-
clusive disease. This disease has a high mortality rate, and
treatment is limited to withdrawal of the offending agents,
if possible, coupled with implementation of thrombolytic ther-
apy and transjugular intrahepatic portosystemic shunting if
feasible.18–20
High-Flow States
A congenital or acquired communication between an artery
and vein in the mesenteric circulation is a rare but known
cause of portal hypertension (Fig. 108-4). Portal hypertension
from such abnormal communications between arteries and
veins in the portal circulation is termed hyperkinetic21 because
of the hyperdynamic circulation responsible for the increased
portal pressure. Patients have well-preserved liver function
and present with bleeding from gastrointestinal (GI) varices,
ascites, or splenomegaly. The site of the communication be-
tween the artery and vein is quite varied. The splenic and gas-
tric arteries have been reported as sites of these fistulas, as well
as the more common site of the hepatic artery within the pa-
renchyma of the liver. Arteriovenous fistulas causing portal
hypertension have been reported in all age groups.22–24 Most
often they are congenital, but some are thought to be acquired
through a pathologic process or iatrogenically.25,26 Patients
may present with varied symptoms that depend on the site
of the arteriovenous communication. Bleeding from varices
may be severe, and because of the exposure to arterial pres-
sures, the usual therapeutic maneuvers may be inadequate
to control it.27
Treatment of high-output fistulas requires interruption of
the abnormal communication between the artery and vein. Al-
though surgical procedures including hemihepatectomy22
and surgical ligation of extrahepatic fistulas27,28 have been de-
scribed as a means of removing the fistula, access by radiologic
FIGURE 108-4 Contrast flow directly from the smaller hepatic artery (A)
into the larger portal vein (P) without traversing the parenchyma of the
liver. This child had an arteriovenous fistula of unknown etiology resulting
in esophageal and gastric varices. The fistula was treated by embolizing
the branch of the hepatic artery.
means and the use of selective transcatheter embolization to
interrupt flow has become the treatment of choice for this
condition.29,30
Clinical Presentation
------------------------------------------------------------------------------------------------------------------------------------------------
GASTROINTESTINAL HEMORRHAGE
Bleeding from the GI tract is one of the most common, dra-
matic, and ominous signs of portal hypertension. Bleeding
most commonly occurs from varices in the distal esophagus
and gastric cardia. Although the incidence of bleeding in
adults with portal hypertension from nonvariceal sites may
be as high as 25%, there is no evidence that the same holds
true in children. In one study of children presenting with up-
per GI bleeding, the vast majority of unselected patients had
bleeding from varices secondary to portal hypertension, with
no other source.31 The importance of portal hypertension as a
cause of upper GI bleeding in children may vary in different
parts of the world. Peptic ulcer disease is still a frequent find-
ing at endoscopy in children in the Western Hemisphere.32 In
children with portal hypertension of any cause, upper GI
bleeding is almost universally caused by variceal hemorrhage,
although portal hypertensive gastropathy may cause less acute
bleeding. Therefore variceal hemorrhage is usually the only
source of bleeding in children with portal hypertension,
and it is the most common cause of bleeding from the upper
GI tract in nonhospitalized children older than 2 years.
Variceal hemorrhage may take the form of hematemesis,
hematochezia, melena, or chronic anemia. Bleeding most
commonly originates from the lower esophagus and the cardia
of the stomach. Thin-walled varices that contain blood under
raised venous pressure are eroded from gastric acid or medi-
cations such as aspirin or nonsteroidal antiinflammatory
drugs, and blood fills the stomach. In the absence of advanced
liver disease, the blood clots and gathers in the stomach,
producing gastric distention and eventually hematemesis.

The bleeding may be sudden and dramatic and is usually not
accompanied by abdominal pain. A previously well child can
vomit large quantities of altered or fresh blood. Although this
can be extremely frightening to both the child and the parents,
the bleeding usually stops spontaneously. Varices may be
present in the lower GI tract as well. Rectal bleeding is less
common and occurs from rectal and sigmoid varices. Melena
or the passage of gross blood through the rectum or accom-
panying a bowel movement may occur in the absence of
hematemesis.
Although most patients with portal hypertension have
varices, not all varices bleed. Predicting the risk of bleeding
from varices is based on Laplace law, which states that the
transmural tension in the wall of a vessel is directly related
to the pressure inside the vessel and inversely related to
the thickness of the vessel. Unfortunately, intravariceal
pressure cannot be easily measured33; therefore the risk of
bleeding in adults has been linked to the size of the varix,
the severity of liver disease, and the appearance of red wale
markings on the vessel wall, which may indicate epithelial
thickness.34 More recently, detailed measurements of the
cross-sectional diameter of the varices have been correlated
with the risk of bleeding35 in adults. Similar studies are nec-
essary in children.
Predicting which patient is likely to bleed from varices is
important in determining treatment options, particularly for
those who may be candidates for prophylactic therapy of
any kind.36 It is estimated that 30% to 50% of patients bleed
from varices once they are diagnosed37 and that 10% to 50%
of patients with liver disease die every year after the first
hemorrhage.38
Aggressive therapy is indicated after the first bleed in pa-
tients with or without liver disease. This may include medical
therapy in addition to local control of varices with banding or
sclerotherapy, portosystemic shunting in patients with stable
and well compensated cirrhosis, or liver transplantation in
those with more advanced disease. There may be a role for
noninvasive or even invasive surgical therapy in those who
are at high risk for bleeding but have not yet bled, provided
the morbidity and mortality associated with bleeding can be
reduced with an acceptably low risk of complications.
In children with portal hypertension from extrahepatic
portal vein thrombosis, mortality from bleeding is much less
than in those with liver disease and bleeding is generally well
tolerated. However, the same principles apply: early inter-
vention and implementation of a long-term plan to reduce
hospital admissions and invasive procedures. Because these
children have a vascular problem rather than hepatic disease,
they are more amenable to surgical intervention and generally
obtain excellent long-term results.
Portal Hypertensive Gastropathy
The gastric mucosa develops distinct pathologic changes in
the presence of portal hypertension and liver disease. The
symptoms include chronic blood loss and iron deficiency
anemia, but occasionally portal gastropathy may also cause
acute GI bleeding and hematemesis. The diagnosis is generally
made during endoscopy, when the severity of the lesion can
also be graded. The severity of the lesion has been correlated
with the severity and frequency of bleeding and the necessity
of treating the consequent anemia.
CHAPTER 108 PORTAL HYPERTENSION 1359
Hypersplenism
Silent splenomegaly without any other history suggestive of
portal hypertension is often the first sign of a serious under-
lying disorder. Splenomegaly may be detected in infancy or
early childhood and can easily be misinterpreted as a sign
of hematologic malignancy, particularly in children with extra-
hepatic portal vein thrombosis and no other stigmata of liver
disease. With the advent of Doppler ultrasound examinations
of the abdomen, portal vein thrombosis is more readily diag-
nosed and can be appropriately monitored. In addition to
splenic enlargement, patients may present with severe throm-
bocytopenia and leukopenia from splenic sequestration of
platelets and white blood cells. Children are often prevented
from engaging in sporting activities for fear that even slight
trauma to the abdomen may result in splenic rupture and
catastrophic intra-abdominal bleeding, exacerbated by raised
venous pressure and low platelet counts. It is hard to find
documented cases of splenic rupture in which the underlying
portal hypertension contributed to massive bleeding and
death.39 Splenic artery aneurysm is a rare complication in
patients with portal hypertension and spontaneous hemor-
rhage, and suggested treatments have been reported.40
Encephalopathy
Encephalopathy is extremely unusual in the absence of signs
of advanced liver disease such as jaundice and low levels of
liver-dependent coagulation factors or low albumin levels.
Learning disabilities and behavioral abnormalities are mani-
festations of encephalopathy in children, in contrast to the
traditional signs of disorientation, memory loss, and drowsi-
ness commonly seen in adults. Children may also have accom-
panying hyperammonemia.41
Bleeding from Nongut Sites
Severe thrombocytopenia can lead to hematuria, menorrhagia
in adolescent girls, epistaxis, and hematochezia. In severe
cases, spontaneous intracranial bleeding can also occur, with
serious neurologic consequences. Liver-dependent coagula-
tion factor deficiencies also lead to bleeding, typically from
the genitourinary tract or from the nose. In cases of advanced
liver disease, hemorrhagic complications in the lungs may
cause severe respiratory compromise.
Ascites
Children with ascites may have advanced liver disease
with synthetic failure. Ascites may be accompanied by a low
serum albumin level and decreased plasma oncotic pressure.
Dilated lymphatics in the abdomen from increased hydrostatic
pressure in all portal tributaries can lead to transudation of
fluid across capillary membranes and accumulation of fluid
in the abdominal cavity. Additional mechanisms include
increased nitric oxide production in capillaries, causing
vasodilatation, and an increase in renal tubular absorption
of sodium in patients with decompensated cirrhosis. Ascites
can also occur in the setting of Budd-Chiari syndrome
and outflow obstruction. Ascites is an alarming symptom
that may herald the onset of liver decompensation on a
wider scale.
1360 PART VII ABDOMEN

Pulmonary Disorders Jaundice

Pulmonary hypertension can occur in children with both Jaundice and portal hypertension usually do not occur to-
cirrhotic and noncirrhotic forms of portal hypertension.42,43 gether unless there is advanced liver disease. Children with
Pulmonary hypertension may be secondary to an increase in extrahepatic portal vein obstruction may have mild elevation
vasoactive substances that exert a vasoconstrictive or direct of the total bilirubin level, but jaundice is not a clinical feature.
toxic effect on pulmonary vessels.43a An increase in blood If jaundice is a prominent symptom in a child with portal hy-
levels of prostaglandin F2a, thromboxane, and angiotensin I pertension, it may indicate that the liver function is severely
in patients with portal and pulmonary hypertension suggests compromised and consideration should be given to a liver
that these agents play a role in mediating pulmonary hyperten- transplant evaluation.
sion by reaching the pulmonary circulation in blood shunted
Abdominal Examination
around the liver. In experimental studies, portal hypertension
induced by portal vein ligation was associated with increased Patients with portal hypertension often have protuberant ab-
amounts of inducible nitric oxide synthetase and heme oxyge- domens. Causes include splenomegaly, ascites, and enlarge-
nase-1 messenger RNA and protein in the lungs, compared ment of the liver. Distended abdominal veins are a frequent
with animals in the control group.44 This suggests that the finding, and the direction of blood flow away from the abdo-
pulmonary pathology seen with portal hypertension is associ- men or umbilicus is easily demonstrable. In patients with con-
ated with the production of nitric oxide and carbon monoxide genital hepatic fibrosis, the combination of enlarged liver and
in the lung. spleen and often large polycystic kidneys contributes to mak-
Patients with both pulmonary and portal hypertension may ing the abdomen extremely protuberant.
develop pulmonary symptoms before there is any evidence of
Encephalopathy
bleeding from portal hypertension. Symptoms may include
exertional dyspnea or chest pain, or there may be no symp- Clinically evident encephalopathy is a sign of advanced liver
toms other than unheralded syncope or sudden death.45 disease. Signs of encephalopathy may be hard to discern or
Treatment of portal hypertension may be problematic in chil- quantify in young children. Advanced psychometric testing
dren with advanced pulmonary hypertension, and medical may detect subclinical encephalopathy that may be manifest
treatment for both conditions may be limited in scope and as impaired attention span, poor school performance, or be-
duration.46 havioral issues that may be present even in children with
The hepatopulmonary syndrome includes persistent no underlying liver disease or patients with well-compensated
hypoxemia from arteriovenous shunting in the lungs. Both cirrhosis.49 Although advanced encephalopathy may be exac-
pulmonary hypertension and arteriovenous shunting are po- erbated or unmasked by acute bleeding because of the in-
tentially reversible after the portal hypertension and shunting creased protein load in the gut, it usually signifies advanced
are eliminated, although in cases of cirrhosis, a liver transplant hepatocellular disease that may require the patient to be eval-
may be necessary.47,48 uated for liver transplantation.50

Diagnosis
------------------------------------------------------------------------------------------------------------------------------------------------ LABORATORY INVESTIGATIONS
The diagnosis of the underlying cause of portal hypertension Laboratory tests are useful adjuncts in evaluating patients with
depends on the synthesis of the clinical information gathered portal hypertension. Tests may indicate the severity of the
from the parents and the child and the results of imaging tests accompanying liver disease or may indicate the cause of the
and laboratory investigations. underlying liver disease in many instances. In the acute setting
of a GI bleed, a complete blood count and electrolyte levels
will be necessary as a guide to blood and fluid replacement.
CLINICAL INFORMATION A complete metabolic panel including renal function tests is
helpful in guiding fluid resuscitation and to assess the under-
Hepatomegaly lying status of the kidneys. In patients with established cirrho-
An enlarged liver is usually a sign of hepatocellular disease, sis from underlying systemic diseases like cystic fibrosis,
although patients with cirrhosis may present with impalpable blood gases may be necessary. Low blood glucose levels
shrunken livers. If the liver is hard or nodular to palpation, may indicate impending liver decompensation or an underly-
this is further evidence that the cause of the portal hyper- ing glycogen storage disorder.
tension is a diseased liver. Children with congenital hepatic Children with long-standing portal hypertension may have
fibrosis often present with enlarged livers that may extend nothing more than thrombocytopenia and leukopenia, partic-
as far as the iliac crest on palpation. Children with venous out- ularly those with extrahepatic portal vein thrombosis. An in-
flow obstruction may also present with easily palpable liver crease in the direct bilirubin fraction, a low albumin level, and
enlargement. a long prolongation in prothrombin time all indicate signifi-
cant hepatocellular disease and possible cirrhosis as the
Splenomegaly underlying cause of the portal hypertension. A raised serum
Enlargement of the spleen is a common finding in children ammonia level indicates significant portosystemic shunting
with portal hypertension and is often the first abnormality and may occur with all forms of portal hypertension, although
found on a routine physical examination before the onset of severely increased ammonia levels are generally only seen in
bleeding. Splenomegaly occurs in all forms of portal hyper- patients who have decompensated cirrhosis or those in fulmi-
tension and is not helpful in diagnosing the cause. nant hepatic failure.

Children with extrahepatic portal vein thrombosis may
have laboratory evidence of disordered synthesis of liver-
dependent coagulation factors in both the procoagulant and
plasminolytic pathways, but this is generally not evident
clinically without detailed testing.51
ENDOSCOPY
Endoscopy is indispensable in establishing the cause of GI
bleeding and to confirm the presence of varices in the esoph-
agus and stomach. In cases of acute bleeding and after
stabilization in the intensive care unit, upper GI endoscopy
provides direct confirmation of the bleeding source. Varices,
if present, will be most prominent in the distal third of the
esophagus and in the cardia of the stomach. Endoscopy
should be done only by those who are well trained in the pro-
cedure and familiar with the equipment. Endoscopy provides
an opportunity to intervene therapeutically, as well as to rule
out other sources of bleeding such as peptic ulcers, Mallory-
Weiss tears of the esophagus, or hemorrhagic gastritis.
If endoscopy is done before the onset of bleeding because
of suspected portal hypertension, the appearance of the vari-
ces provides valuable clues about the likelihood of future
bleeding. Variceal diameter greater than 5 mm, the appearance
of red wale markings, and more advanced liver disease based
on Child-Pugh class indicate a greater chance for future
bleeding and may justify the institution of prophylactic treat-
ment.52–54
IMAGING
The first imaging study in any child who presents with hema-
temesis should be abdominal ultrasonography. The status of
the portal vein is one of the most important radiologic con-
siderations in arriving at a diagnosis. Cavernous transforma-
tion of the portal vein and portal vein thrombosis are best
diagnosed by ultrasonography with Doppler interrogation
of the vessels of the liver. A complete evaluation of the
intra-abdominal vasculature including the hepatic veins, the
patency of the splenic and superior mesenteric veins, and
the inferior vena cava is possible. In addition, information
about the size of the spleen can be obtained to confirm the
clinical examination. Liver parenchymal abnormalities such
as nodularity, inhomogeneity, or the presence of cysts can
be seen. The appearance of the kidneys can yield useful diag-
nostic clues because children with congenital hepatic fibrosis
may also have autosomal dominant polycystic kidney disease
and other renal abnormalities.
Computed tomography (CT) and magnetic resonance (MR)
angiography are excellent diagnostic tools and have sup-
planted conventional digital angiography for most purposes.
Both modalities provide excellent information about all the
intra-abdominal vessels and detailed information about the
liver anatomy including the bile ducts. CT-angiography has
several advantages; it can be done more quickly and is less
prone to image degradation from motion artifact than is MR
angiography, unless the MR study is done under general
anesthesia with ventilatory arrest for the duration of the study.
Conventional angiography is an invasive modality that has
few indications as a diagnostic tool in children with portal hy-
pertension. The exceptions are rare cases of unusual vascular
malformations such as arteriovenous communications in the
CHAPTER 108 PORTAL HYPERTENSION 1361
abdomen or liver that may best be delineated by angiography.
Also, angiographic visualization is essential as a prelude
to angiographic embolization or stenting of a vascular
abnormality.
Splenoportography is a technique that was written about
extensively in the past but is almost never used for diagnostic
purposes today.
Transjugular hepatic venography is a test that can be used
to measure free and wedged hepatic vein pressures indicating
the gradient across the hepatic veins. This technique is limited
in small children where the size of the child and the veins
makes the performing of this test more challenging. Wedge he-
patic vein pressure can rule out parenchymal disease and high
sinusoidal pressure as a contributing factor to portal hyperten-
sion. Sinusoidal pressures are normal in cases of prehepatic
portal venous obstruction.
Retrograde transjugular portal venograms can also be used
to obtain high-quality definition of the intrahepatic portal vein
(Fig. 108-5). Hepatic vein angiography can also be used ther-
apeutically for the placement of a transjugular intrahepatic
portosystemic shunt (TIPS) or the dilatation of congenital or
acquired strictures causing venous outflow obstruction.
LIVER BIOPSY
Many children who present with portal hypertension do not
need a liver biopsy to establish a diagnosis. For example, in
children with biliary atresia a biopsy is not necessary as an
adjunctive test after the onset of portal hypertension. In chil-
dren with a known underlying metabolic disease such as
cystic fibrosis or alpha-1 antitrypsin deficiency, a biopsy will
not necessarily add useful information to aid in formulating
a treatment plan.
However, if a biopsy is considered helpful, it can be done
percutaneously in most cases. In children with mild coagulo-
pathies that can be corrected with vitamin K, fresh frozen
plasma, and platelets, the procedure can be performed with
close monitoring, where any bleeding from the biopsy site
can be treated promptly. Children with more profound
FIGURE 108-5 A catheter has been advanced into the left hepatic
vein, and contrast is injected under pressure filling the entire intrahepatic
portal vein.
1362 PART VII ABDOMEN

coagulopathies, as manifested by a prothrombin time in MEDICAL MANAGEMENT

excess of 20 seconds, should have a transjugular biopsy in OF BLEEDING VARICES
the interventional radiology suite or an open biopsy in the
operating room. In general, the limiting factor in the radiolo- Patients admitted with acute variceal hemorrhage require in-
gist’s ability to do a transjugular biopsy is the patient’s size. tense resuscitation with blood and crystalloids, replacement of
The difficulty and technical limitations are greatest in infants coagulation factor deficiencies with fresh frozen plasma, and
and babies. control of the bleeding. Bleeding from varices may be exacer-
bated by disseminated intravascular coagulation, fibrinolysis,
and a decrease in circulating platelet counts. The availability of
Treatment exogenous recombinant factor VII to treat those with compro-
------------------------------------------------------------------------------------------------------------------------------------------------
mised liver synthetic function has enhanced the care of these
The treatment of a child with portal hypertension is predi- patients. Factor VII replacement decreases the amount of
cated first on the underlying pathophysiology of the disease sodium and fluid necessary to correct the liver-dependent fac-
and then on the severity of the symptoms. tor deficiencies, although its use is limited to some extent by
Children with liver disease and portal hypertension do not its cost.58
tolerate bleeding as well as those with essentially normal livers Patient care is best delivered in the intensive care unit,
whose portal hypertension is caused by primary vascular mal- where vital signs including central venous and arterial pres-
formations. Acute hemorrhage adversely affects liver function, sures, hourly urine output, and oxygen saturation can be
which may already be abnormal. The prognosis of a child with monitored. Monitoring of mental status is also essential in
liver disease and portal hypertension depends entirely on the those with cirrhosis because bleeding into the GI tract may ex-
hepatic reserve. Children with relatively stable cirrhosis and acerbate or unmask encephalopathy.
preserved synthetic function require a completely different Cessation of bleeding is the most important therapeutic
therapeutic approach from those with hypoalbuminemia, goal, along with the replacement of extracellular fluid and
growth failure, and coagulopathy. The Child-Pugh score55 blood. In the past, Sengstaken-Blakemore tube tamponade
was devised in an attempt to categorize patients according of varices was the most effective method of short-term control
to degree of hepatic reserve and thus arrive at a prognosis of blood loss. Pharmacologic therapy has proved to be just as
for survival if left untreated or with surgery.56 More recently, effective in adults,59 rendering the balloon method almost ob-
a pediatric-specific score (pediatric end-stage liver disease solete. Pharmacologic control of variceal bleeding has im-
[PELD]) has been developed to stratify children on liver trans- proved greatly with the use of intravenous octreotide, the
plant waiting lists in a way that accurately reflects the severity octapeptide analogue of somatostatin. It has a longer half-life
of the underlying liver disease.57 Children with age-appropriate than somatostatin and fewer side effects. Octreotide has
growth, normal coagulation, and no jaundice have low PELD largely replaced vasopressin in the management of acute var-
scores; their treatment for the complications of portal hyper- iceal bleeding because of the severe vascular side effects of the
tension is different from that of children with high scores, who latter drug.
would be better served by liver transplantation. Octreotide and other somatostatin analogues such as
In children with Child-Pugh scores of 5 or 6 or PELD vapreotide reduce hepatic blood flow and wedged hepatic
scores lower than 10 who have a predicted 1-year survival vein pressure and constrict splanchnic arterioles by a direct
of more than 90%, it is justified to consider complex surgi- effect on arteriolar smooth muscle. Somatostatin analogues
cal therapy to alleviate the symptoms of portal hypertension. are effective in reducing and temporarily stopping the bleed-
The expectation is that these children will survive the sur- ing from portal hypertension and can be used in the initial 24
gery and have a good long-term prognosis without the need to 72 hours while awaiting direct endoscopic management of
for liver transplantation in the short term. For children with varices.60–63 More recently, terlipressin, a vasopressin deriva-
portal hypertensive bleeding from varices and who also have tive, has also been proven effective in the acute bleeding situ-
ascites, advanced coagulopathy or poor synthetic function ation, although experience in children is limited.62,64,65
as indicated by hypoalbuminemia, endoscopic banding or Octreotide is started as a continuous infusion at 1 to 2 mg/kg/hr,
sclerosis of esophageal varices in addition to aggressive up to a maximum of 100 mg/hr, and continued for as long as
medical therapy of portal hypertension is indicated as they symptoms of bleeding persist. Antibiotics and acid suppression
await transplantation. Operation is not indicated in children are also recommended.62
with advanced liver disease because it would be poorly tol- The long-term medical management of children with portal
erated and not address the underlying issue of poor hepatic hypertension includes the use of nonselective beta blockers
reserve. such as propranolol or nadolol, either alone or in combination
Pediatricians and pediatric gastroenterologists closely fol- with nitrate vasodilators such as isosorbide-5-mononitrate.
low all patients with portal hypertension and liver disease. Some authors, however, have shown that local control of var-
When portal hypertension becomes symptomatic, it is rarely ices with banding is equivalent to long-term pharmacologic
a surprise and the parents are usually well versed in the med- management. In a majority of patients, surveillance endoscopy
ical issues. Under ideal circumstances, surgery can be consid- and intermittent banding may be sufficient,37 with equivalent
ered for the control of symptoms when bleeding is imminent survival and rebleeding rates in endoscopically managed and
or likely to recur. Selective shunting is generally the procedure pharmacologically managed groups. The role of beta blockers
of choice, as discussed later. in reducing splanchnic blood flow and wedge hepatic vein
In contrast, patients with noncirrhotic forms of portal hy- pressure is well documented; their use may decrease the inci-
pertension can develop symptoms quite unexpectedly and as dence of recurrent bleeding by as much as 50% and lessen the
the first manifestation of a serious underlying condition. need for liver transplantation in patients with liver disease.66

Assessment of wedge hepatic vein pressure reduction has been
promoted as the best way to determine the impact of medica-
tions on portal pressure and to guide and refine pharmaco-
logic management,67 but these studies are all adult based
and may represent a less practical approach in children.68
PROPHYLACTIC TREATMENT OF VARICES
Intervention of any kind before the onset of bleeding is con-
troversial. However, the benefit to the patient of preventing a
first bleed may be considerable and justifies early prophylactic
management. Studies in both adults and children have dem-
onstrated that prophylactic treatment of varices by pharmaco-
logic means and with endoscopic ligation reduces the
frequency of bleeding but may not diminish mortality in pa-
tients with advanced liver disease without transplanta-
tion.69,70 Primary procedures for the prevention of an initial
bleed are more controversial if there is no other primary goal
such as the treatment of severe hypersplenism.
ENDOSCOPIC SCLEROSIS OR BANDING
OF VARICES
Surgeons were involved in the earliest attempts to control var-
iceal bleeding by injecting sclerosing solutions into the tissue
around a varix or directly into a varix to obliterate the vessel
lumen.71 This method has proved to be effective and safe in
children in the acute phase of bleeding.52–54,72–81 Sclerosing
solutions include sodium morrhuate, ethanolamine, sodium
tetradecyl sulfate, and polidocanol. Long-term follow-up in
children after sclerotherapy for the control of initial bleeding
has demonstrated success in almost 90% of patients. Initial
sclerotherapy should be followed by repeat endoscopy at reg-
ular intervals until all the varices are obliterated or too small to
inject and then at longer (yearly) intervals for at least 4 years to
check for reappearance of varices. Generally, two to three in-
jections at 1 mL per injection are required for each varix, up to
a maximum of 10 to 15 mL per session. The endoscopist
should proceed circumferentially from the distal esophagus
at the gastroesophageal junction to the more proximal
esophagus.
Sclerotherapy is associated with a fairly high incidence of
complications, both major and minor, in at least one third
of patients. Acute complications include chest pain, esopha-
geal ulceration, and mediastinitis; chronic ones include
esophageal strictures from fibrosis after multiple injection
sessions.
Esophageal banding has become an increasingly popular
method of treating varices. In one prospective trial in children
with portal hypertension from extrahepatic portal vein throm-
bosis, banding was found to be a more effective, more rapid,
and safer method of reducing the chance of bleeding from var-
ices.82 The incidence of complications and of long-term
rebleeding was lower with banding. New technology, such
as the multiband ligator scope, has made it possible to apply
bands to multiple varices; this method was first used in adults
but has recently been extended to children.83,84 This tech-
nique increases the speed and safety with which esophageal
varices can be ligated in children as young as 3 months old;
obliteration of varices was accomplished in almost 100% of
children after only two sessions.
CHAPTER 108 PORTAL HYPERTENSION 1363
Esophageal banding in concert with pharmacologic control
has become the procedure of choice in the early therapy of
bleeding esophageal varices. Subsequent sessions are aimed
at ligating residual varices or varices that arise after the larger
ones are tied off. Ligation is effective in most children and is
not associated with many of the adverse side effects of
sclerotherapy.
INJECTION THERAPY FOR GASTRIC VARICES
With the advent of more effective means of controlling esopha-
geal varices, bleeding from varices lower in the GI tract has be-
come more problematic. Injection with N-butyl-2-cyanoacrylate
has had modest success in adults with bleeding from gastric
varices and results in children have been encouraging.85,86
Gastric varices may coexist with esophageal varices in up to
15% to 20% of patients, or they may be isolated. Gastric varices
may emerge after the obliteration of esophageal varices, or they
may persist after esophageal varices have been ligated. Experi-
ence in adults suggests that in patients with gastric varices,
cirrhosis, and advanced liver disease, bleeding can be controlled
with minimal morbidity, but there is a high incidence of rebleed-
ing at 1 year after treatment.87 In adults, injection of gastric
varices results in relatively poorer control of bleeding in patients
with extrahepatic portal vein thrombosis (35%) than in those
with liver disease (75%).88 Given the fact that extrahepatic portal
vein thrombosis is a more important cause of bleeding varices in
children than in adults, injection therapy for gastric varices in
pediatric patients is not justified, except in the setting of a formal
study to determine its safety and efficacy compared with surgery
or other medical therapies.
TRANSJUGULAR INTRAHEPATIC
PORTOSYSTEMIC SHUNTS (TIPS)
The percutaneous insertion of vascular stents to create chan-
nels between the portal vein and hepatic veins within the pa-
renchyma of the liver heralded a new way of treating patients
with advanced liver disease and portal hypertension.89,90
The main indication for TIPS is variceal hemorrhage recalci-
trant to more conservative therapy with endoscopy or octreo-
tide. It is usually reserved for patients with advanced liver
disease and serves as a bridge to transplantation.91 Other in-
dications include refractory ascites, hepatic venous outflow
obstruction in both transplant and nontransplant patients,
and hepatorenal syndrome.
In children, the experience is limited. TIPS may act as a
bridge to transplantation in children with portal hypertensive
gastropathy or in those whose variceal bleeding is difficult to
control by other means. The TIPS method has been used in
children with cystic fibrosis,92 biliary atresia,93 and congenital
hepatic fibrosis94 with apparent success and in infants as
young as 1 year.
Its primary limitation is the high rate of shunt thrombosis.
Vigilance is required to monitor shunt patency and to declot
the shunt when it is thrombosed. The other limitation is the
aggravation of preexisting encephalopathy in approximately
20% of patients and the hastening of liver failure in patients
whose livers are already borderline; TIPS facilitates the
1364 PART VII ABDOMEN
shunting of blood without the benefit of hepatic clearance in
these extremely debilitated patients.95
Hepatic encephalopathy is less common after TIPS in chil-
dren than in adults, but the incidence of shunt occlusion is
higher because of the smaller shunt diameter.
SHUNT SURGICAL PROCEDURES
Early shunt operations diverted all the mesenteric blood flow
into the systemic circulation, as first described by Eck.2 Later
authors reported the application of portacaval shunting in
the setting of severe bleeding from portal hypertension in
adults with advanced liver disease,55,96–99 as well as in
children with prehepatic and intrahepatic causes of portal
hypertension.100–104
Shunts can be described as nonselective or selective. Selec-
tive shunts preserve the majority of portal or mesenteric blood
flow to the liver while shunting blood from high-pressure gas-
troesophageal varices into the low-pressure systemic venous
circulation. In essence, selective shunts divide the portal cir-
culation into two separate entities, although it is impossible
to fully divide all communications. In contrast, nonselective
shunts divert a large proportion of mesenteric blood flow away
from the liver so that the entire GI tract including the spleen
and pancreas is decompressed. Nonselective shunts can be
further subdivided into total or partial portal diversions.
Nonselective Shunts
Total Diversion The end-to-side portacaval shunt as first
described by Eck is the classic example of a total portal diver-
sion. Portal blood is completely redirected into the inferior
vena cava below the liver, and the hepatic end of the portal
vein is oversewn. That operation is almost never done in
children and bears no further discussion. All nonselective
large-caliber shunts deprive the liver of virtually all mesenteric
venous blood flow and increase the danger that the undesir-
able side effects of portosystemic shunting will be produced.
Encephalopathy, pulmonary hypertension, and formation of
regenerative nodules in the liver are some of the long-term
side effects of complete mesenteric venous diversion away
from the liver and can happen even in children with ostensibly
normal liver function and no intrinsic liver disease.
Proximal Splenorenal Shunt The proximal splenorenal
shunt is another example of a shunt that results in total diver-
sion of mesenteric venous blood into the systemic circulation.
The pancreas is mobilized cephalad, exposing the underling
splenic vein. All branches between the pancreas and the
splenic vein must be tied off meticulously. The splenic vein
is divided close to the spleen, and the spleen is removed.
The left renal vein is isolated, and the mesenteric end of the
splenic vein is sewn to the side of the left renal vein so that
all the blood from the superior and inferior mesenteric veins
is shunted into the systemic venous circulation through the
left renal vein.105,106 This procedure almost invariably in-
cludes a direct attempt to ligate the varices of the esophagus
and stomach in the region of the coronary vein.
Although the operation is effective in relieving the symp-
toms of portal hypertension, it should be used sparingly
because it not only exposes the child to the potential draw-
backs of complete mesenteric diversion, as outlined earlier,
but also results in the loss of the spleen.
Mesocaval Shunt Wide-diameter mesocaval shunts also
completely divert mesenteric blood away from the liver. This
procedure can be done directly as a side-to-side anastomosis
between the two veins or with the interposition of a short au-
tologous vein graft or prosthetic graft.107,108 Use of an inter-
position graft is the more common method. Exposure of
both the superior mesenteric vein at the root of the bowel mes-
entery and the inferior vena cava below the duodenum is
required.
Mesocaval shunting has been used in children in a wide va-
riety of settings and diseases, with uniformly acceptable re-
sults.109–111 Some prefer this method because it allows an
anastomosis between two large-diameter vessels and because
the length of the anastomosis can be increased to some extent
to facilitate the creation of a large venous fistula, which is tech-
nically easier to perform than a proximal splenorenal shunt. In
addition, in contrast to the proximal splenorenal shunt, the
spleen is preserved, which is thought to be important in pre-
venting postsplenectomy sepsis.
Side-to-Side Portacaval Shunting The side-to-side porta-
caval shunt allows blood from the intestine and spleen to
flow easily into the vena cava. In addition, and unlike with
the end-to-side portacaval shunt, the hepatic end of the por-
tal vein is changed into an outflow tract. In cases of Budd-
Chiari posthepatic portal hypertension, the liver is decom-
pressed by this operation; this may result in long-term
palliation of the disease, with arrest or delay in the progres-
sion of hepatic fibrosis and ultimate failure.14,112,113 Other
effective operations for Budd-Chiari syndrome are mesocaval
and central splenorenal shunts, which allow portal blood in
the liver to empty in a retrograde fashion through the patent
portal vein.
Partial Diversion Partially diverting shunts depend on a
fixed, narrow communication between a vessel in the portal
circulation and one in the systemic venous circulation. The
Sarfeh shunt can divert enough blood from the portal circula-
tion to drop mesenteric pressure below 12 cm H2O, thus
decompressing the varices and reducing the chance of bleed-
ing while maintaining enough pressure in the portal bed to al-
low hepatopetal flow and hepatic blood flow preservation.114
Although there may be less encephalopathy with the Sarfeh
shunt because of sustained hepatic portal flow, it is associated
with a higher incidence of thrombosis and recurrence of hem-
orrhage and rarely used in children.115
The relationship between deprivation of portal blood to the
liver and encephalopathy has long been noted following porta-
caval shunt surgery. Sarfeh was able to determine the diameter
shunt that would preserve forward flow in the portal vein, de-
compress the portal circulation so that bleeding from varices
decreased, and minimize the incidence of encephalopathy.
Selective Shunts
To diminish the encephalopathy that followed portacaval
shunting, Warren and Zeppa116 described a shunt between
the portal end of the splenic vein and the side of the renal vein
(Fig. 108-6). The splenic confluence with the portal vein was
ligated, and the coronary vein was also interrupted. In this
manner, the gastroesophageal varices were decompressed
across the short gastric vessels and the spleen was decom-
pressed into the renal vein. Long-term studies have shown

Ligated coronary vein
Spleen
Portal vein
Splenic
Ligated stump
vein
of splenic vein
Superior Kidney
mesenteric vein
Renal vein
FIGURE 108-6 Schematic drawing of the distal Warren shunt. Note that
the coronary vein has been ligated. The varices are both decompressed by
allowing them to drain via the short gastric vessels and splenic vein into
the left renal vein and by decreasing blood flow to the varices by tying
off the blood that reaches them through reverse flow in the coronary vein.
that the disconnection between the lower-pressure splenic cir-
culation and the higher-pressure hepatic one is not always
maintained, and the connection may re-form over time. Hepa-
topetal flow may decrease and be redirected to the coronary
circulation around the stomach; as a result, the hepatic flow
decreases, just as in more central shunts.117,118 In this case,
there would be both angiographic and endoscopic evidence
of gradual conversion of a selective shunt to a less selective
shunt over time. A more complete pancreaticosplenic dis-
connection may avoid the reestablishment of communication
between the splenic and hepatic circulations. Long-term
mortality and rebleeding rates in adults are equivalent with
selective and nonselective shunts,119 but the rate of encepha-
lopathy is reduced with selective shunts.120,121
In children, the selective shunt as described by Warren or
with minor modifications has been used successfully for the
treatment of bleeding varices, as well as for the treatment of
hypersplenism.122 Long-term patency rates exceeding 90%
have been reported in most series.
Distal splenorenal shunting is used primarily in children
with extrahepatic portal vein thrombosis, stable Child-Pugh
classification A or B cirrhosis, or less common forms of intra-
hepatic portal hypertension such as congenital hepatic fibrosis
with well-preserved liver function but symptomatic variceal
bleeding.
The preservation of neurocognitive function is particularly
important in children; thus all forms of nonselective shunting
should be avoided whenever possible. Encephalopathy in
children may manifest as learning disorders or behavioral
abnormalities that are not usually attributed to portosystemic
shunting.123
Hypersplenism may be particularly problematic in children
and has led to splenectomy or splenic embolization in some
children100,124–132 with or without a central splenorenal
shunt.
The distal splenorenal shunt has been beneficial in chil-
dren with advanced hypersplenism as a more physiologic
CHAPTER 108 PORTAL HYPERTENSION 1365
alternative to the splenectomy.133 Spleen size regresses, and
the platelet and leukocyte counts return toward normal.
Following splenic decompression by shunting, hematologic
indices may continue to improve for years after the procedure.
Splenectomy and splenic embolization were advocated in
the past for hypersplenism. However, these procedures are
not without complications and may, in the long run, exacer-
bate bleeding from gastroesophageal varices and leave the
child prone to postsplenectomy sepsis. In addition, splenec-
tomy removes the possibility of later distal splenorenal shunt-
ing if the child continues to suffer from variceal bleeding.
Pediatric surgeons should preserve the spleen whenever
possible, leaving splenectomy as the last resort in children
who have no other options.
MESENTERIC-TO-LEFT PORTAL VEIN BYPASS
The treatment of extrahepatic portal vein thrombosis has been
evolving since the advent of mesenteric vein-to-left portal
vein bypass in 1992. The operation was originally described
as a way to revascularize liver transplant grafts after post-
transplant portal vein thrombosis,134,135 but the indications
were later expanded to treat children with idiopathic portal
vein thrombosis.136 A growing number of children have been
reported in the literature from a small group of institutions
worldwide.11,47,137,138
Essentially, the operation relieves portal hypertension by
redirecting blood from the obstructed mesenteric system to
the still patent intrahepatic portal vein. The prerequisites
for a successful bypass operation are threefold: (1) no intrinsic
liver disease, (2) a patent intrahepatic portal tree, and (3) a
suitable vein in the mesenteric circulation to function as a suit-
able inflow tract for mesenteric blood.
In portal vein thrombosis, the extrahepatic portal vein is
replaced by a network of small collaterals that supply the liver
with a small amount of mesenteric blood and keep the intra-
hepatic portal circulation patent. The intrahepatic portal tree
in these children is patent but hypoplastic.
The preoperative workup includes CT-angiography to as-
sess the caliber and patency of the intrahepatic portal vein,
a liver biopsy to rule out intrinsic liver disease, and a coagu-
lation workup to rule out a hereditary hypercoagulable
state.139 It was once thought that hereditary hypercoagulable
states, particularly protein C deficiency, often may have caused
the thrombosis of the portal vein.140–142 However, it has been
reported that deficiencies not only in the plasminolytic path-
way but also the procoagulant pathway may be secondary to
the hepatic deprivation of portal blood and are reversible after
the successful restoration of portal flow.51
The operation starts by dissecting out the intrahepatic por-
tal vein in the recessus of Rex, where the round ligament in-
serts between segments III and IV of the liver. Portions of
segments III and IV are removed. All the branches to segments
II, III, and IV are controlled. If it is suitable, the superior
mesenteric vein is dissected out at the root of the mesentery.
Alternative sources of blood for the bypass including the
splenic, inferior mesenteric, and coronary veins have been
described.143
Once the inflow vein has been isolated, the jugular vein
(either right or left) is removed and sewn first to the intrahe-
patic portal vein and then to the superior mesenteric vein.
The vein graft usually goes through the mesocolon and the
1366 PART VII ABDOMEN
lesser sac, up behind the stomach, through the lesser omen-
tum, and up to the liver. In children with malrotation or
other congenital disorders, the route of the graft may vary,
depending on the child’s individual anatomy. CT or MR scan-
ning in the postoperative period demonstrates the patent
shunt (Fig. 108-7).
Unlike portosystemic shunting, the mesenteric-to-left
portal vein bypass, or Rex shunt, is restorative rather than pal-
liative. It restores portal flow to the liver and relieves the symp-
toms of portal hypertension. With proper patient selection,
patency rates have been in excess of 90%.11 Patency depends
primarily on the quality of the intrahepatic portal vein and the
number of branches that allow for sufficient runoff to permit
blood flow to be sustained. The intrahepatic portal vein may
be difficult to visualize by preoperative imaging, and a final
assessment may be possible only at the time of surgery.
NONSHUNT OPERATIONS
Nonshunt operations for portal hypertension are generally
limited to a direct attack on the varices by operative interrup-
tion and ligation, as well as esophageal transection to interrupt
the intramural varices within the esophagus.144–147 In the ab-
sence of suitable shunt options, these procedures are useful
alternatives.
Sugiura and colleagues147 described an operation in which
all perigastric and periesophageal vessels were separated from
the incisura of the stomach to the midesophagus at the level
of the inferior pulmonary vein. In addition, the esophagus
LPV
G ventional radiologic techniques can study the vessel and the
SV
REX
dSMV
FIGURE 108-7 The postoperative appearance of a meso-Rex bypass. The
blood flows from the superior mesenteric vein (dSMV) and recruits blood
from the splenic vein (SV), the coronary vein (G). The vein graft (Rex) flows
cephalad into the left portal vein (LPV). The white arrows indicate a mild
narrowing near the anastomosis between the jugular vein graft and the
intrahepatic portion of the left portal vein.
was divided and reattached. This radical approach to portal
hypertension presented an alternative to shunting procedures,
but it may have additional morbidity. A modified Sugiura
operation in which the esophagus is transected and reanasto-
mosed by a stapling device and in which the spleen is pre-
served has been described in children. As experience with
shunting and other vascular surgery in children has increased,
the use of these effective but palliative and unphysiologic
operations has decreased.
Complications of Surgery
------------------------------------------------------------------------------------------------------------------------------------------------
SHUNT THROMBOSIS
Any vascular operation, especially on the venous system, has
the potential for thrombosis. Prophylaxis against thrombosis
may be instituted in the immediate postoperative period with
low-dose heparin and antiplatelet agents such as aspirin or
dipyridamole and continued for 3 to 6 months. Once a shunt
is thrombosed, it may not be salvageable, and other solutions
such as devascularization must be sought if bleeding recurs.
Careful follow-up, antithrombotic prophylaxis, and good
operative technique to begin with should result in a long-term
patency rate of greater than 90%.
ANASTOMOTIC STENOSIS
Anastomoses can develop narrowing that can cause sluggish
flow in the vessels, possibly thrombosis, and recurrence of
the original signs and symptoms. Stenoses have characteristic
appearances on Doppler ultrasound examinations that in-
clude a narrow area at the anastomosis, poststenotic dilatation
of the vessel secondary to turbulent flow, and acceleration of
the velocity of blood flow through the stenotic area. If the ste-
nosis is believed to be of hemodynamic significance, then it
must be dilated either by radiologic or surgical means. Inter-
stenotic area, assess the hydrostatic gradient on either side
of the anastomosis, and dilate the narrow area by balloon di-
latation. The effect of the ballooning can also be assessed by
remeasurement of the pressure gradient. Placement of an
endovascular stent is also a possibility in recalcitrant strictures
(Fig. 108-8). We prefer to avoid stents in smaller children be-
cause, once placed, a stent may act as a fixed stenosis as the
child grows and cannot be removed.
ASCITES
Ascites is common after shunt operations because of disrup-
tion of the retroperitoneal lymphatic channels. In most cases
this resolves spontaneously. Oral diuretics may be necessary
for a short time. A reduced-fat diet may also be helpful be-
cause all the ascites is chylous in nature. Rarely, paracentesis
may be necessary if the ascites is tense and persistent. If so,
parenteral nutrition may be used to eliminate any chylous
flow for a short time until the lymphatic leak seals. If ascites
does not resolve with these measures, it may indicate that
the hepatic reserve is small and transplantation may be
necessary.
 CHAPTER 108 PORTAL HYPERTENSION 1367

INTERMITTENT BLEEDING FROM

ESOPHAGEAL OR GASTRIC VARICES
Today, because of the effectiveness of endoscopy and medica-
tions, few patients with extrahepatic portal vein thrombosis
present with acute, poorly controlled variceal bleeding requir-
ing surgery. Patients are presenting with greater frequency
with advanced hypersplenism and well-controlled varices.
Controlling variceal bleeding does, however, require numer-
ous endoscopies, occasional transfusions, and treatment with
beta blockers and vasodilators.
As surgical options have become more effective and the re-
sults more predictable, definitive surgery for extrahepatic por-
tal hypertension should be offered at an earlier point in the
disease process rather than waiting until all other therapy
has failed.148 This is especially relevant with the advent of
the meso-Rex bypass because this operation restores normal
portal pressure and relieves hypersplenism by redirecting
mesenteric blood back to the liver. The portal vein delivers
FIGURE 108-8 A transhepatic catheter is entering the liver from the right 50% of the oxygen and 80% of the total blood supply
side of the patient. It has deployed a clearly visible endovascular stent used to the liver. After a successful meso-Rex bypass, the flow in
to treat a stricture in the vein graft or at the anastomotic suture line that the vein graft increases over time as evidenced by the
was resistant to simple balloon dilatation. rapid expansion in portal vein branches inside the liver
(Fig. 108-9, A and B). The effects of restoring portal blood
to the liver are not yet apparent and may have far-reaching
metabolic consequences during the child’s early development.
The correction of coagulation parameters back to normal
ESOPHAGEAL STRICTURES after portal flow restoration may be only one of many amelio-
After devascularization procedures accompanied by esopha- rations in hepatic function that take place. The common belief
geal transection, it is not uncommon for the esophagus to nar- that a child with portal vein thrombosis and portal hyper-
row at the site of transection. Overt leaks are an uncommon but tension has “normal” hepatic function may not be correct.
serious complication that requires thoracostomy drainage, pro- As experience with the meso-Rex bypass increases, more
longed antibiotics, and possibly reoperation. Fortunately, most evidence of the metabolic sequelae of the restoration of portal
strictures are manageable by esophageal dilatation, although flow to the liver has become apparent. Both clinical and
some may require repeated sessions to achieve satisfactory experimental data point to improved growth, protein synthe-
patency. sis, and neurocognitive function following a meso-Rex by-
pass.49,51,149–151 The long-term consequences of portal vein
thrombosis on bile and bile ducts are also not well under-
stood, but evidence suggests there is an increased incidence
Outcome of cholelithiasis and biliary cirrhosis from uncorrected portal
vein thrombosis.152,153
------------------------------------------------------------------------------------------------------------------------------------------------

The outcome of treatment in a child with portal hypertension Recently, the conclusions of a consensus conference held to
depends on the cause of the hypertension and the underlying formulate surgical guidelines in the treatment of children with
state of the liver. In the past decade, the care of these patients portal vein thrombosis were published. These take into ac-
has improved considerably because of better medical and sur- count both the developing comfort in doing this procedure,
gical options and the availability of liver transplantation in as well as the new data that indicate the meso-Rex bypass
children with poor hepatic reserve. not only relieves the symptoms of portal hypertension as well
as other procedures but also restores to normal many of the
metabolic parameters of the liver that have been affected by
the long-standing deprivation of portal blood flow.148
EMERGENCY BLEEDING FROM VARICES
Selective shunting, through either a distal splenorenal
Medical control of portal hypertension has improved to the shunt or a coronary-to-vena cava graft, to correct portal hyper-
point that emergency shunts in children are the exception tension from portal vein thrombosis has been established as a
rather than the rule. Medical therapy and endoscopic control procedure with minimal morbidity and no mortality when
of esophageal varices by ligation is excellent. Emergency done by experienced surgeons. Patency rates exceed 95%,
shunts, when necessary, are effective in controlling hemor- and hospital stays of less than a week are common. In the long
rhage, with good long-term patency rates reported. However, run, selective shunting may be better for children than re-
the patency rate of emergency shunts is lower than the 90% or peated hospital admissions for banding and long-term medi-
greater patency rate reported for elective operations. Emer- cation use to control bleeding. In addition, advanced
gency mesocaval shunting is seldom reported today, owing hypersplenism occurs in many of these children when defin-
to the availability of medical and endoscopic means of control- itive surgical therapy is delayed. Even when variceal banding
ling acute variceal hemorrhage. is instituted prophylactically, bleeding may shift away from the
1368 PART VII ABDOMEN
A treatment with beta blockers. Long treatment may also include
B Almost all shunts have been shown to decrease the severity
FIGURE 108-9 These two images of the liver from the same patient be-
fore (A) and after (B) the meso-Rex bypass illustrate the expansion in the
intrahepatic portal vein size that occurs quickly after restoration in mesen-
teric venous flow (arrows in both images point to the portal vein in the
equivalent area). The change in portal vein size is indicative of an increase
in the volume of blood flowing through the liver.
esophagus and into the stomach in many children over time.
Surgical therapy (Rex shunt or distal splenorenal shunt)
controls bleeding, reduces hospital admissions, decreases
the need for medications, and relieves hypersplenism in a
physiologic way that does not expose the child to the lifelong
risk of overwhelming sepsis after splenectomy.
Shunting for portal hypertension caused by intrinsic liver
disease is a more complex proposition because it has to take
into account the health of the liver. Selective shunting is excel-
lent at decompressing the child’s portal system, with minimal
morbidity in patients with low Child-Pugh or PELD scores. Al-
though the theory behind the distal splenorenal shunt is that it
separates the splenic and gastric venous drainage from the in-
testinal and mesenteric venous flow, careful studies have
shown that separation between the two systems is not always
complete and may disappear over time as the body forms col-
lateral veins to replace the ones that were interrupted. Never-
theless, selective shunts that attempt to preserve hepatopetal
flow result in less encephalopathy and better hepatic function
than do nonselective shunts in both children and adults.
If a child with well-preserved liver function and portal hy-
pertension cannot have a selective shunt because of a previous
failed shunt or because of unfavorable anatomy, it may be bet-
ter to consider a devascularization procedure to minimize
the potential for encephalopathy.154,155 The only role for
nonselective shunts in children may be the rare child with
well-preserved liver function and obstructive venopathy that
cannot be stented or opened by an interventional radiologic
approach. In this case the risk of minimal encephalopathy
may be outweighed by the dangers of recurrent bleeding.156
GASTROPATHY
The treatment includes all measures generally applied to
bleeding from esophageal varices except that endoscopic treat-
ment is not possible. Measures include drug therapy with
somatostatin analogues, acid suppression, antibiotics,62 and
surgical relief of the portal hypertension. This has been shown
to reverse the gastropathy.157 The choice of operation of
course depends on the cause of the portal hypertension and
the venous anatomy.
HYPERSPLENISM
As the treatment for esophageal variceal bleeding has im-
proved, more children are presenting with bleeding from gas-
tric varices, portal hypertensive gastropathy, or complications
of advanced and debilitating hypersplenism. There is no
medical therapy for hypersplenism other than stimulating
the bone marrow with granulocyte colony-stimulating factor
(GCSF).158,159 In fact, GCSF may transiently lower the platelet
count while it helps with the leucocytes.160 Surgery is the only
alternative.
of hypersplenism when done primarily for the indication of
bleeding from symptomatic varices. The same two principles
should govern the choice of surgery done primarily for
hypersplenism: splenic preservation and the avoidance of
encephalopathy. The distal splenorenal shunt has been shown
to ameliorate hypersplenism in children. In children with
extrahepatic portal vein thrombosis, the Rex shunt has had ex-
cellent results in terms of resolving hypersplenism and also
improving the neurocognitive parameters associated with por-
tosystemic shunting.
Summary
------------------------------------------------------------------------------------------------------------------------------------------------
A child with portal hypertension presents a unique challenge
to the surgeon. The number of treatment options has in-
creased over the past 2 decades and includes a wider variety
of effective medications that reduce the pressure in the mes-
enteric tree and diminish the blood flow in the portal circula-
tion. These options have greatly decreased the need for
emergency surgery in children with portal hypertension.
Figure 108-10 summarizes the treatment options discussed
in this chapter on the basis of the underlying cause of portal
hypertension.
Ancillary interventions such as endoscopic ligation of var-
ices and radiologically guided placement of intrahepatic stents
have also reduced the reliance on operative control of bleeding
 CHAPTER 108 PORTAL HYPERTENSION 1369

Portal Hypertension Treatment Process

Portal
Hypertension

Intrinsic Liver Extrahepatic Outflow

Disease Portal Venous Obstruction
Obstruction

Poor hepatic Good hepatic

Poor hepatic
function (Child function (Child Liver biopsy to rule Stable hepatic
function and/or
class C/D, PELD class A/B, PELD out liver disease function
widespread necrosis
>15) <15)

Treat bleeding Treat bleeding Treat bleeding

complications complications complications Treat medically or List for liver
medically and medically and medically and by stenting transplantation
endoscopic ligation endoscopic ligation endoscopic ligation

Rex shunt or DSRS Consider Consider TIPS

List for liver DSRS for gastric varices, for recurrent bleeding, side-to-side
transplantation advanced hypersplenism while waiting for
hypersplenism, or portacaval shunt transplant
or recurrent esophageal gastric varices
varices

Consider TIPS
while waiting for
transplant

FIGURE 108-10 Treatment algorithm according to the underlying cause of portal hypertension. Portal hypertension accompanied by advanced liver
dysfunction from either venous outflow obstruction or intrinsic liver disease is ultimately treated by transplantation, with or without a transjugular intra-
hepatic portosystemic shunt (TIPS) procedure. If the liver dysfunction is acceptable and not rapidly progressive, an appropriate shunt may be a good long-
term solution. For symptomatic portal hypertension from extrahepatic portal hypertension, mesenteric-to-left portal vein bypass is the procedure of choice,
followed by distal splenorenal shunt (DSRS). PELD, Pediatric end-stage liver disease.

in patients with advanced liver disease. Such interventions in-
crease the chance that these patients will survive to receive a
liver transplant.
Although the need for surgical procedures has been re-
duced, surgical results have improved so that both shunt
and nonshunt procedures offer a more permanent solution
to bleeding and hypersplenism in patients with prehepatic
and posthepatic portal hypertension and in those with
well-compensated cirrhosis. With the decreased morbidity
associated with portal hypertension surgery, the excellent
long-term patency rates reported even in infants and small
children after shunt surgery, and the increased number of sur-
gical options available for children, surgery for portal hyper-
tension offers a good alternative to medical treatment, even
early in the course of treatment.
The complete reference list is available online at www.
expertconsult.com.