SYMPOSIUM: HEPATOLOGY
Portal hypertension in
children
Fang Kuan Chiou
Mona Abdel-Hady
Abstract
Portal hypertension (PH) is an important complication of chronic liver
disease. It can also be caused by a wide range of extrahepatic pathol-
ogies in children, and is often clinically silent. Acute variceal haemor-
rhage (VH) is the most serious consequence of portal hypertension
associated with significant morbidity and mortality. Management of
PH in children consists of medical, endoscopic and surgical ap-
proaches which are mainly focused on acute treatment as well as
reducing the risk of variceal haemorrhage. Current treatment strate-
gies for children with PH are mostly based on extrapolation of data
from adult studies and expert opinion and consensus. A structured
protocol, consisting of surveillance endoscopy with primary and sec-
ondary prophylactic therapy by endoscopic variceal ligation or sclero-
therapy, is increasingly becoming the standard of care. This article
discusses the causes and current treatment options for PH in
childhood.
Keywords hypersplenism; liver cirrhosis; oesophageal and gastric
varices; portal hypertension; splenomegaly
Introduction
Portal hypertension (PH) in children is a major complication
arising from liver cirrhosis and extra-hepatic vascular disorders.
It is associated with significant morbidity and mortality. It
commonly presents catastrophically with variceal haemorrhage
(VH), which may occur for the first time in a child with no
apparent medical history, particularly if PH is due to a non-
hepatic cause. Other common clinical features of PH include
splenomegaly, hypersplenism and ascites. Encephalopathy and
pulmonary manifestations such as hepatopulmonary syndrome
(HPS) and portopulmonary hypertension (PPH) are important
complications but less commonly encountered in children.
The goals of management of PH are directed at treating its
complications. Controversies still surround treatment strategies
for prevention or reducing the risk of variceal bleeding and
evidence-based recommendations remain scarce.
Portal venous system
The liver receives its blood supply from the hepatic artery and
the portal vein (PV). The PV accounts for 75% of the blood
Fang Kuan Chiou MBBS MRCPCH is Clinical Fellow in Paediatric
Hepatology, Liver Unit, Birmingham Children’s Hospital, Birmingham,
UK. Conflict of interest: none declared.
Mona Abdel-Hady MBBch MD MRCPCH is Consultant Paediatric
Hepatologist, Liver Unit, Birmingham Children’s Hospital,
Birmingham, UK. Conflict of interest: none declared.
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supply to the liver and is formed by the union of the splenic vein
(SV) and the superior mesenteric vein (SMV). The SMV drains
mainly the small intestine, proximal colon and head of the
pancreas. The SV drains blood from the spleen and the inferior
mesenteric vein (IMV), which receives blood from the distal large
bowel. Before reaching the liver, the main PV divides into the left
and right portal veins, which in turn divides sequentially into
smaller venules. The portal venules and hepatic arterioles ulti-
mately merge into hepatic sinusoids that drain into hepatic veins.
Three hepatic veins eventually drain blood from the liver into the
inferior vena cava (IVC). A schematic representation of the portal
venous system is shown in Figure 1.
Pathophysiology
PH occurs as a result of increased vascular resistance and/or blood
volume through the portal venous system. The hyperdynamic
circulatory state results from a series of physiologic responses
which include splanchnic vasodilatation and activation of the
sympathetic nervous system and renin-angiotensin-aldosterone
axis, which in turn lead to sodium and water retention, hyper-
volaemia, increased cardiac output and splanchnic blood inflow.
Normal portal venous pressure is 7e10 mmHg, and hepatic
venous pressure gradient (HVPG) ranges from 1 to 4 mmHg.
HVPG is the difference between the free hepatic venous pressure
(FHVP) and wedged hepatic venous pressure (WHVP) which
reflects hepatic sinusoidal pressure. PH is defined as portal
pressure greater than 10 mmHg or a HVPG greater than 4 mmHg.
The most significant pathological consequence of portal hy-
pertension is the formation of collateral vessels between the
portal venous system and the systemic circulation, leading to the
development of varices in the oesophagus, stomach and rectum.
In adults, HVPG above 10 mmHg is associated with oesophageal
variceal formation and a pressure gradient above 12 mmHg is
associated with ascites and variceal bleeding.
Causes
The causes of PH are classified into three categories: prehepatic,
posthepatic and intrahepatic, which can be further subdivided
into presinusoidal, sinusoidal and postsinusoidal. These are
summarized in Table 1.
Prehepatic causes
Portal vein thrombosis (PVT) is the most common cause of
extrahepatic portal vein obstruction (EHPVO) in children.
Neonatal events such as umbilical vein catheterization, ompha-
litis and sepsis are common attributable factors, while pro-
thrombotic disorders such as protein C, protein S and anti-
thrombin III deficiencies and factor V Leiden mutations have
been found to account for up to 35% of children with PVT.
Interestingly, the cause of PVT remains unidentified in about
50% of cases.
Intrahepatic causes
Various intrahepatic presinusoidal, sinusoidal and postsinusoidal
causes give rise to increased portal bed resistance within the liver
and PH. Presinusoidal causes include congenital hepatic fibrosis
and nodular regenerative hyperplasia, which often do not result
in impaired liver function. Sinusoidal obstruction is mainly due
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 SYMPOSIUM: HEPATOLOGY
Figure 1 Schematic representation of the portal venous system. (SMV,
superior mesenteric vein; IMV, inferior mesenteric vein; PV, portal vein;
SV, splenic vein; HV, hepatic veins; IVC, inferior vena cava).
Classification and causes of PH in children
Classification Causes
Prehepatic Portal vein thrombosis
Congenital or acquired stenosis of portal vein
Splenic vein thrombosis
Intrahepatic
Presinusoidal Congenital hepatic fibrosis
Polycystic liver disease
Nodular regenerative hyperplasia
Myeloproliferative diseases (lymphoma,
leukaemia)
Granulomatous diseases (schistosomiasis,
sarcoidosis, tuberculosis)
Non-cirrhotic portal fibrosis/Idiopathic PH
Sinusoidal Liver cirrhosis (independent of cause)
Post-sinusoidal Veno-occlusive disease
Posthepatic Budd-Chiari syndrome
IVC obstruction
Constrictive pericarditis
Right heart failure
Table 1
to liver cirrhosis, independent of the underlying primary liver
disease. The increase in intrahepatic vascular resistance in
cirrhosis is due to two factors: the ‘mechanical factor’ is related
to hepatic architectural derangement caused by fibrosis and
nodule formation, while the ‘dynamic factor’ is related to vaso-
constrictive effect from vasoactive endogenous factors. Post-
sinusoidal obstruction is best represented by veno-occlusive
disease (VOD), or sinusoidal obstruction syndrome, which oc-
curs as a result of conditioning treatment administered prior to
haematopoietic stem cell transplantation (HSCT). VOD is char-
acterised by microthrombosis and sclerosis of hepatic venules,
and presents with hyperbilirubinaemia, hepatomegaly and asci-
tes typically within 3 weeks from HSCT.
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Posthepatic PH
Posthepatic PH can be due to a hepatic venous outflow
obstruction (Budd Chiari syndrome), which is uncommon in
children, or cardiac disorders with increased right atrial pressure.
In posthepatic portal hypertension, chronic venous congestion
results in hepatomegaly and can eventually lead to liver
dysfunction and cirrhosis.
Clinical presentation
The main clinical manifestations of PH in children are gastroin-
testinal haemorrhage, splenomegaly and ascites. Abnormal
abdominal venous patterning (caput medusa) may also provide
an important clue to underlying portal hypertension. Other
complications that are less common include hepatorenal syn-
drome, pulmonary vascular disease, growth failure and en-
cephalopathy. In patients with extrahepatic portal hypertension
or compensated liver disease, there may be no prior symptom
and the first indication of portal hypertension may be gastroin-
testinal bleed or an incidental finding of splenomegaly.
Gastrointestinal bleeding: gastrointestinal haemorrhage is usu-
ally from ruptured oesophageal varices, but may also be sec-
ondary to portal hypertensive gastropathy, gastric antral vascular
ectasia, or gastric, duodenal, peri-stomal or rectal varices. VH
has been reported to occur in 17%e29% of children with biliary
atresia in retrospective cohorts, and nearly half to two-thirds of
children with EHPVO by 16e18 years of age. The age of the first
bleeding episode is related to the underlying aetiology of PH.
Reported median ages at presentation of first VH was 3.8 years in
patients with EHPVO, 17 months e 3 years in patients with
biliary atresia, and 11.5 years in patients with cystic fibrosis-
related liver cirrhosis.
VH has been observed to occur more often in children with
intercurrent upper respiratory infection and febrile illnesses. The
factors postulated to contribute to rupture of varices include
increased abdominal pressure from coughing and sneezing,
increased cardiac output during febrile episode, and use of non-
steroidal anti-inflammatory medication.
Splenomegaly: splenomegaly is a common clinical finding in
children with PH and can be an incidental discovery on routine
physical examination. The haematological consequence of
hypersplenism, including thrombocytopenia and leucopenia,
often misleads clinicians into performing a work-up for haema-
tological causes, resulting in delayed diagnosis of PH. Liver
function test (LFT) and Doppler ultrasonography are therefore
advisable in the evaluation of children with splenomegaly and
hypersplenism. When portal pressure is relieved either with liver
transplantation or porto-systemic shunt surgery, splenomegaly
and hypersplenism are expected to improve over time.
Ascites: ascites develops when hydrostatic pressure exceeds
oncotic pressure within the hepatic and mesenteric capillaries,
and the fluid shift overcomes the drainage capacity of the
lymphatic system. Ascites is usually seen in patients with PH due
to cirrhosis. Increased sodium and fluid retention contributes to
further fluid accumulation in the peritoneal space. Treatment of
ascites includes salt and fluid restriction, and diuretic therapy.
Spironolactone is the first-line diuretic as its property as an
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 SYMPOSIUM: HEPATOLOGY

aldosterone antagonist counteracts the renin-angiotensin-

aldosterone axis that contributes to the hyperdynamic circula- Diagnostic evaluation of a child with suspected PH
tion associated with PH. A loop diuretic such as frusemide may Diagnostic approach Salient features to assess
be added to enhance diuresis. Albumin infusions in tandem with
diuretics can be used to increase intravascular oncotic pressure History Neonatal history (umbilical
and facilitate diuresis, particularly in patients with hypo- catheterisation)
albuminaemia from chronic liver disease. Paracentesis is History of liver disease, jaundice
reserved for significant ascites that is refractory to pharmacologic Haematemesis/malaena
treatment, causing respiratory compromise, or for diagnostic Physical examination Splenomegaly
evaluation. Liver size/consistency
Abnormal venous patterning
Abnormal venous patterning: prominent abdominal venous Ascites
patterning develops in PH due to spontaneous porto-collateral Mental status (Encephalopathy)
shunting through subcutaneous veins. Prominent periumbilical Stigmata of chronic liver disease
veins (caput medusa) are a result of decompression of portal Laboratory tests Liver function test
pressure through umbilical vein recanalisation that leads to Full blood count to check for anaemia
periumbilical collaterals. In children with short bowel syndrome (blood loss) or thrombocytopenia
and intestinal failure-associated liver disease, stomal varices are (hypersplenism)
often present and are common sites for bleeding. Clotting function
Liver ultrasonography Portal vein: patency, direction of flow,
Pulmonary complications: hepatopulmonary syndrome (HPS) and Doppler cavernomatous transformation
and portopulmonary hypertension (PPH) are rare complications Liver parenchyma
in children with PH, and their pathogenesis remain unclear. Patency/flow in hepatic veins and artery
HPS is characterised by arterial oxygenation defect in the Splenomegaly
setting of liver disease and is thought to occur as a result of Ascites
excessive vasoactive mediators which lead to abnormal vasodi- Porto-systemic shunts
latation of pulmonary arterioles and capillaries causing arterio- Renal abnormalities
venous shunting and ventilation-perfusion mismatch. Patients Upper gastrointestinal Oesophageal, gastric varices
with HPS present with dyspnoea, cyanosis and digital clubbing. endoscopy Portal gastropathy
Liver transplantation is the only effective treatment for children Other causes of bleeding: gastritis,
with HPS but outcome is poor if HPS is at an advanced stage with peptic ulcer
significant hypoxaemia. Liver biopsy Assess degree of liver fibrosis/cirrhosis
PPH is defined as an elevation of the mean pulmonary arterial Histological diagnosis of underlying liver
pressure and increased vascular resistance in the setting of PH disorder
and in the absence of cardiopulmonary disease. Symptoms of WHVP, FHVP and HVPG Evaluate degree of PH
PPH include exertional dyspnoea, fatigue, palpitations, syncope measurement Determination of prehepatic, intrahepatic
and chest pains. Liver transplantation is feasible only in the early or posthepatic cause
stages prior to the onset of frank right-sided heart failure. Abdominal CT scan Assess vascular anatomy for the planning
of shunt surgery
Other complications: hepatic encephalopathy occurs in the
context of decompensated liver disease and PH with anatomical Table 2
and functional porto-systemic shunting, and signs may be subtle
particularly in young children. Growth retardation is also a rec- treatment of varices (Figure 2). Endoscopy may also detect portal
ognised complication of PH in children and may be related to gastropathy, characterised by erythema and oedema of the
portal hypertensive enteropathy, underlying liver dysfunction gastric mucosa with ‘snake-skin’ or mosaic pattern, cherry-red
and growth hormone resistance. spots and mucosal friability. Catheter measurement of HVPG,
which is considered the gold standard technique to measure
Diagnosis of PH portal venous pressure in adults, is also feasible in children but
data in paediatrics are limited. Angiography by computer to-
The aims in the clinical evaluation of a child with PH are to
mography (CT) or magnetic resonance (MR) provides detailed
evaluate for the underlying cause, and assess for complications
imaging of both intrahepatic and extrahepatic vasculature which
of PH (Table 2). Laboratory tests should include LFT and clotting
is essential for planning shunt surgery.
function to assess for liver disease, and full blood count (FBC) for
evaluation of hypersplenism. Doppler ultrasonography allows
Management
visualisation of the size, patency and flow of the portal vein, and
detection of cavernomatous transformation, porto-systemic Therapy of PH is mainly directed at prevention and treatment of
shunts, splenomegaly and ascites. It is also a useful tool for VH. The management can be divided into primary prophylaxis of
evaluation of liver disease and patency of hepatic veins. Upper the first episode of bleeding, management of acute VH (see
gastrointestinal endoscopy is performed for diagnosis and Table 3), and secondary prophylaxis of subsequent bleeding

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 SYMPOSIUM: HEPATOLOGY

Figure 2 Endoscopic appearance of oesophageal varices and portal gastropathy. (a) Large, tortuous oesophageal varix (Grade 3) at 5 o’clock
position. (b) Extension and prolapse of varices at gastro-oesophageal junction visualised on retroflexion of endoscope in the stomach. (c) Band
applied on an oesophageal varix. (D) Patchy erythema and “snake-skin” mucosal appearance indicative of portal gastropathy.

episodes. Most treatment strategies are derived and extrapolated
from adult studies and paediatric data remain lacking at present.
A recent publication summarises expert opinion on paediatric
PH, by reviewing and adapting the recommendations from the
Baveno V Consensus Workshop on the Methodology of Diag-
nosis and Therapy in Portal Hypertension.
Primary prophylaxis
Avoiding the morbidity and mortality associated with the first VH
is the rationale behind primary prophylaxis. Practice among
paediatric hepatologists varies and decisions on primary pro-
phylaxis may be influenced by individual patient factors. For
instance, primary prophylaxis may be valuable in patients who
live in remote areas far from emergency medical care. In paedi-
atrics, non-specific beta-blockers (NSBB) and endoscopic
variceal ligation (EVL) are options considered for primary
prophylaxis.
NSBB reduce portal pressure by decreasing cardiac output and
inducing splanchnic vasoconstriction via b1 and b2-receptor
blockade. Studies in adults have shown that reducing resting
heart rate by 25% or HVPG by 20% decreases bleeding rate in
cirrhosis. There are no randomised trials assessing the efficacy of
propranolol as prophylaxis of VH in children, and data from
cohort studies did not include effect of treatment on HVPG. There
are also concerns that young children receiving NSBB may
experience worse outcomes as a result of hypovolaemic shock
because of their physiologic reliance on their tachycardic
response to shock and poor ability to increase stroke volume. In
general, NSBB should be avoided as first-line primary prophy-
lactic therapy in children till further evidence on appropriate
dosing, efficacy and safety is established.
Prophylactic EVL is performed on high-risk varices seen
during surveillance endoscopy in children with liver disease and
PH. EVL as primary prophylaxis in children is well-tolerated,
with low subsequent bleeding rate and no reports of major
complication. It has been shown to be superior to sclerotherapy
in terms of efficacy and safety in both adults and children. In
small children in whom banding devices cannot be used in
small paediatric endoscopes, sclerotherapy may be the only
practical option for management of large varices, but further
safety data are required before routine use can be
recommended.
Management of acute VH
Acute VH is the most severe complication of PH, with an asso-
ciated mortality of up to 20% in patients with chronic liver dis-
ease. Initial management of variceal bleeding is aimed at
stabilizing the patient. Vital signs must be monitored and intra-
venous access should be established promptly. Tachycardia and
hypotension are signs of significant blood loss, however patients

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 SYMPOSIUM: HEPATOLOGY
Initial management steps in acute VH
Resuscitation and general management
C Intravenous (IV) fluid resuscitation
 IV crystalloid fluids
 Red blood cell transfusion: target haemoglobin of 70e80 g/L
C Nil by mouth, nasogastric tube on free drainage
C Correct coagulopathy (Vitamin K, fresh frozen plasma) and
thrombocytopenia (if less than 20  109/L)
C Empiric broad-spectrum antibiotics
C Monitor vital signs, urine output, conscious level, blood sugar
and haemoglobin
Pharmacotherapy
C Octreotide (IV): 1e5 mcg/kg/hour continuous infusion
C Omeprazole (IV): 1 mg/kg/dose od, or Ranitidine (IV) 1e3 mg/kg/
dose tds
C Sucralfate (NG/PO): 250 mge1000 mg qds
C Lactulose (PO) start with 0.5 ml/kg/dose tds and titrate to achieve
2e4 soft stools
Endoscopy
C Endoscopic variceal ligation (EVL)
C Endoscopic sclerotherapy (EST)
Early referral to/discussion with paediatric hepatologist/
gastroenterologist
Table 3
on beta-blocker therapy may not manifest the expected
compensatory tachycardia and are at higher risk of haemorrhagic
shock. Volume restoration with crystalloids and red blood cell
transfusion is usually required but caution must be exercised to
avoid overfilling the intravascular space and increasing portal
pressure. Red blood cell transfusion should be administered
conservatively to achieve a general target haemoglobin level
between 70 and 80 g/L. Nasogastric (NG) tube placement is safe
and is useful in detecting ongoing bleeding and removing blood
from the stomach, which can precipitate encephalopathy and
aggravate further bleeding. Vitamin K deficiency, particularly in
cholestatic liver disease, should be corrected. Transfusion of
clotting factors and platelets are reserved for cases of profound
coagulopathy or thrombocytopenia (less than 20  109/L), and
must be balanced against the risk of fluid overload, especially in
liver disease with its associated complications of cerebral
oedema and recurrent variceal bleeding. Antibiotic prophylaxis
has been shown to decrease mortality in adults. A high index of
suspicion for bacterial infection is vital in acute VH and intra-
venous antibiotics should be started promptly if sepsis is
suspected.
Pharmacotherapy: in suspected VH, vasoactive drugs should be
started early before endoscopy is performed, and continued for 2
e5 days. These vasoactive drugs include vasopressin, somato-
statin or their analogues. Two drugs, octreotide and terlipressin,
are suitable for paediatric use.
Octreotide is a synthetic analogue of somatostatin that has
been shown to decrease splanchnic blood flow and its efficacy in
managing variceal bleed in children has been reported in
observational studies. An initial bolus of 1 g/kg and continuous
infusion of 1e3 mcg/kg per hour appear to be safe and effective
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in controlling active VH in children. Newer longer-acting so-
matostatin analogues are currently under investigation.
Terlipressin is a long-acting synthetic analogue of vasopressin
which also has a vasoconstrictive effect on the splanchnic system
leading to reduced portal blood flow. It does not require
continuous infusion. However there are no prospective data in
children and no specific dose recommendations in paediatrics at
present.
Endoscopy: endoscopic therapy is recommended in any patient
who presents with upper gastrointestinal bleeding and in whom
varices are known or suspected cause of bleeding. Ideally, pa-
tients should undergo endoscopy as soon as possible within 24
hours after adequate resuscitation. EVL is the recommended
modality of endoscopic therapy for acute oesophageal VH, with
lower rates of complications compared to endoscopic sclero-
therapy (EST). Nevertheless, EST remains an option in small
infants and young children, in whom EVL is technically chal-
lenging as passing the banding apparatus may not be possible
due to its size.
After endoscopic therapy, patients should continue to fast for
at least 2 hours before liquid and soft solid feeding is introduced
gradually as tolerated. Treatment with sucralfate is also recom-
mended as it appears to decrease the risk of early re-bleeding.
Balloon tamponade: the Sengstaken-Blackmore tube is designed
to stop VH by physical compression on oesophageal and gastric
varices with balloon tamponade. It is reserved for rare cases of
severe, refractory VH and should only be used in an intensive
care facility by trained medical staff and for a maximum of 24
hours until definitive treatment can be instituted.
Transjugular intrahepatic porto-systemic shunt (TIPS):
rebleeding may be managed with repeat endoscopy. However,
persistent bleeding refractory to pharmacotherapy and endoscopic
treatment is an indication for TIPS. A catheter is introduced into the
hepatic vein (HV) via the jugular vein, a tract is created between
the PV and HV, and a stent is placed to form a permanent porto-
systemic shunt. Main complications are shunt thrombosis lead-
ing to re-bleeding, and encephalopathy as with any porto-systemic
shunting. The experience in paediatrics is limited but TIPS may
serve as an important rescue therapy in refractory bleeding.
Emergency surgery: emergency surgical shunt procedures, or
oesophageal transection and/or devascularisation in the setting
of acute VH are rarely performed because of associated high
mortality and morbidity. Emergency liver transplantation per-
formed for acute VH is rarely necessary and is also associated
with poor outcomes.
Secondary prophylaxis: children who have experienced VH
should be offered secondary prophylaxis to reduce the risk of
recurrent bleeding. EVL is the preferred modality for secondary
prevention because of its better safety profile and less number of
sessions required compared to sclerotherapy. Based on expert
consensus, EVL should be performed every 2e4 weeks after the
first VH, followed by 6e12 monthly. In infants and young chil-
dren where EVL is technically not feasible, EST is the recom-
mended alternative therapy.
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 SYMPOSIUM: HEPATOLOGY
There is currently insufficient evidence to recommend the
routine use of NSBB in secondary prophylaxis for VH in children.
In patients who fail endoscopic treatment for the prevention of
rebleeding, the options are surgical porto-systemic shunting,
TIPS and liver transplantation.
Porto-systemic shunting e the goal of shunt procedures is to
divert portal blood flow and decrease portal venous pressure.
These techniques include mesocaval shunt in which the shunt is
formed between the SMV and IVC, portacaval shunt in which the
shunt is formed between PV and IVC, and distal splenorenal
shunt in which the shunt is formed between SV and left renal
vein. These procedures divert blood from normal hepatic meta-
bolism and are therefore associated with increased risk of hepatic
encephalopathy especially in patients with intrahepatic disease.
Hence, surgical porto-systemic shunting in children with
decompensated liver disease is associated with poor outcomes,
with reported complications of recurrent bleeding, encephalop-
athy and death. Instead, children with decompensated liver dis-
ease should be assessed and considered for liver transplantation.
In contrast, porto-systemic shunting is a feasible option in pa-
tients with pre-sinusoidal PH, such as in congenital hepatic
fibrosis, and EHPVO.
If technical expertise is available, TIPS may be an alternative
mode of therapy in children in whom surgical shunting is not
feasible. TIPS may be used in children with end-stage liver dis-
ease with refractory VH as an effective bridge to liver
transplantation.
Meso-portal bypass e the meso-Rex bypass procedure is
considered for children specifically with extrahepatic portal vein
thrombosis. In this procedure, a graft is placed between the SMV
and the left portal venous system, thereby bypassing the EHPVO
and restoring physiological portal blood flow. The advantage of
this approach is that it minimises the risk of hepatic encephalop-
athy encountered in porto-systemic shunting. With any shunt
surgery, the complication of shunt thrombosis must be considered,
particular in patients with underlying prothrombotic disorders.
Summary
There is a wide range of causes that lead to PH in children,
management is focused mainly on prevention and acute treat-
ment of VH which is its most severe and frequent complication.
Current recommendations in children are based on extrapolation
of data from adult studies and expert paediatric opinion. A
structured protocol with surveillance endoscopy and primary
and secondary prophylactic treatment to prevent VH is gaining
acceptance as standard of care for paediatric patients with PH.A
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Practice points
C Variceal haemorrhage is the most serious complication of
portal hypertension (PH) in children, timely resuscitation
and endoscopic therapy are vital.
C PH is often asymptomatic and may present with life-
threatening bleeding.
C Splenomegaly and hypersplenism in a child should prompt
the clinician to suspect PH.
C Surveillance endoscopy and prophylactic endoscopic vari-
ceal ligation (EVL) are increasingly adopted as standard of
care in paediatric patients with PH.
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