Will be presented at : To the honorable :

CASE REPORT-1

A 14th years old Boy with Esophageal Varices due to Non-

cirrhotic Portal Hypertension

Presented By:
dr. Kirnia Tri Wulandari

Tutors:
Dr. dr. Satrio Wibowo, Sp.A (K), M.Si.Med

PEDIATRIC DEPARTMENT
FACULTY OF MEDICINE BRAWIJAYA UNIVERSITY
DR. SAIFUL ANWAR GENERAL HOSPITAL
Mei 2023
 A 14th years old Boy with Esophageal Varices due to Non-cirrhotic
Portal Hypertension
1 2 2
, ,
1
Pediatric Residency, Faculty of Medicine, Brawijaya University-Saiful Anwar Hospital Malang
2
Division of Nephrology, Pediatric Department, Brawijaya University-Saiful Anwar Hospital Malang

Abstract
Non-cirrhotic portal hypertension (NCPH) comprises a group of diseases that are characterized
by increased portal pressure in the absence of cirrhosis of the liver, includes extrahepatic portal
vein obstruction (EHPVO), idiopathic portal hypertension (IPH), non-cirrhotic portal fibrosis
(NCPF) and congenital hepatic fibrosis (CHF). Esophageal varicose veins (Esophageal varices)
form if the portal pressure is more than 10 mmHg and variceal bleeding is still a lifethreatening
event in childhood. This case is about 14 years old boy who was diagnosed with haematemesis
due to esophageal varices bleeding in non-cirrhotic portal hypertension. Endoscpic showed
esophageal varices with rupture of the distal 1/3 of the main esophagus. The patient had been
treated conservatively without any invasive therapy.

Keywords: pediatric; Esophageal varices, non cirrhotic portal hypertension, variceal bleeding

Introduction Varicose bleeding and ascites can occur

Non-cirrhotic portal hypertension when the PPG (portal pressure gradient)
(NCPH) is characterized by a presinusoidal reaches more than 12 mmHg.6
portal hypertension in the absence of Complications related to portal
cirrhosis of the liver or hepatic venous hypertension dominate the signs and
outfow obstruction. 1,2
NCPH includes symptoms present in patients with NCPF.
amongst these diseases, the most frequent Frequency of variceal bleeding (15.7–55%)
entities are extrahepatic portal vein is lesser in children as compared to adults
obstruction (EHPVO), idiopathic portal (70–95%) with NCPF possibly due to longer
hypertension (IPH), non-cirrhotic portal duration of illness in adults.7 in a Childhood
fibrosis (NCPF) and congenital hepatic Liver Disease Research Network abstract,
fibrosis (CHF).2,3 risk of variceal bleeding in almost 600
Portal hypertension (PH) is a children with portal hypertension was 8% at
common problem in children with liver 5 years with no mortality.8
disease, and its most threatening Laboratory features show cytopenias
complication, variceal bleeding, is a major (hypersplenism) and preserved hepatic
4,5
cause of morbidity. Varicose veins form if synthetic functions. Repeated sessions of
the pressure is more than 10 mmHg. endoscopic variceal ligation or endoscopic

2
sclerotherapy eradicate esophageal varices
in almost all cases. After
eradication, there is an increased risk of
other complications like secondary gastric
varices, cholangiopathy, colopathy, growth
failure, especially in extra-hepatic portal
vein obstruction (EHPVO). 2
Endoscopy of the
gastrointestinal tract is the gold standard for
diagnosing esophageal
Esophagogastroduodenoscopy
would diagnose clinically
esophageal varices (grade II, III, or red wale
markings/cherry red spots) in children with
portal hypertension.9 The potential value of
screening endoscopy depends in part on
the risk of bleeding, the mortality, and the
effectiveness of interventions to prevent
bleeding.8
There is lack of data on paediatric
Non-cirrhotic portal hypertension (NCPF).
Therefore, the case that will be described
below is expected to be able to increase the
variety of cases of esophageal varieal
bleeding due to non-cirrhotic
hypertension.
Case report
A 14 year old boy presented with
haematemesis accompanied by black stools
since 4 hours before admitting to the
hospital. Vomited 4 times with a volume of
approximately 100cc, accompanied by fever
2 days before. The patient has gone to the
variceal clinic and treated with chloramphenicol,
domperidone, and ibuprofen. There was a
history of consuming spicy food every day.
The patient had never complained of
anything like this.
While in the womb, the patient's
mother routinely went to OBGYN for
upper monthly
Fig antenatal
1. Endoscopic care.ofOn
appearance ultrasound
small-, there
medium-, and
largesized oesophageal varices classified by the
is a transverse
reference observer.4 presentation and umbilical
varices. cord twisting so the patient born by sectio
(EGD) caesaria at 39-40 weeks of gestation. There
significant was a history of bleeding during pregnancy
and was given uterine strengthening drugs.
At birth the patient immediately cried, there
was no cyanosis and jaundice with a birth
weight of 2,800 grams. The patient had a
history of nutrition in the form of breast milk
from birth to 1.5 years of age, then
continued with formula milk until 6 years of
age. Complementary food for ASI has been
started since the patient was 6 months old.
Physical examination: Weight 54 kg
(P25-P50), Height 158 cm (P10) ~ 13 years
3 months, and BMI 21.6 (P50-P75). Other
portal physical examinations are within normal
limits.
The patient is being treated in the
PICU. On lab examination, Hb was getting
lower compared to the initial admission, as
well as leukocytes (table 1). Positive anti-
Hbs with reactive HbsAg.
Examination of the Peripheral Blood
Morphological Smear found neutrophilia
3
 A
B

with macroplatelets. Stool culture

examination found 3+ bacterias with a
brownish and mushy texture, without
erythrocytes. The chest X-ray image was
also found to be within normal limits.
On endoscopy, there were
esophageal varices with rupture of the 1/3th
distal of the main esophagus, and be
Fig. 2. The Physical examination of the patient.
treated
A. normalthe dayappearance;
physical after diagnosed.
B. Normal Chest He X-ray
also
of the patient
had a CPC installed afterwards. We were
trying to reduce portal pressure by
administering propranolol and ocreotide.
There was no chronicity with good liver
function.

The patient also underwent abdominal A B

ultrasound examination (figure 4) and

abdominal CT scan with contrast and
obtained the following results (Figure 5)

Fig.4.Patient's Abdomen ultrasnog

(Splenomegaly and Hepatomegaly with dilated and turtous portal
thickening, may still be a sign of esophageal va

Fig.3 Case’s Endoscopy (02/11/2022) : It was found

Grade III esophageal varices with portal hypertension
(dilated veins wothout thrombus), splenomegaly,
hepatomegaly without cirrhotic marks), and chronic
Table 1. gastropathy
Labratorium findings

4
5
 Fig.5.Patient’s Abdomen CT Scan with contrast
(showed Esophageal varices to the esophagogastric junction (at T6-T10) with dilated and turtous portal vein,
splenic vein, left gastric vein with gastroesophageal, gastroepiploic, splenorenal collaterals; impressive
portosystemic collateral pathway; Splenomegaly; No thrombus is seen in the portal vein; and Multiple
lymphadenopathy of superior-inferior mesenteric, right pericollica and paraaortic

From all the anamnesis and the embolization, the risk of sepsis and
examinations, he was diagnosed with postoperative bleeding, the risk of
esophageal varices bleeding due to non- postoperative hepatoma syndrome, and the
cirrhosis pulmonal hypertension. TIPS tool is not yet available.
During hospitalization, the patient
was given conservative therapy including Discussion
platelet TC transfusion 600cc, IV Octreotide Gastrointestinal bleeding in the form
1mcg/kg/hour, IV Omeprazole 2x20mg, IV of oesophageal varices is an important
Ondansetron 3x8mg, IV Ceftriaxone complication of PH in children with cirrhotic
2x1gram, IV Paracetamol 3x500mg, and noncirrhotic PH.1 Non-cirrhotic portal
Sucralfate 3x10cc orally, and Zinc 1x20mg. hypertension consists of a group of
This patient was not planned for surgical diseases characterized by signs and
procedures such as splenorenal shunting, complications of portal hypertension, which
TIPS, and splenectomy due to several differ from cirrhosis through histological
considerations such as the vein being too alterations, hemodynamic characterization
tortuous, the risk of abscess due to and, clinical outcome.3,5 Typically, patients

6
present with features of clinically significant and nitrous oxide dilates the capillary
portal hypertension (PHT) such as upper network in the liver, modulating portal
gastrointestinal (GI) bleed and pressure. In addition to portal pressures,
splenomegaly with preserved liver functions. coagulation factor levels and platelet counts
Unlike liver cirrhosis, the disease course is also affect the risk of bleeding. Physiologic
generally indolent and complications such differences between children and adults
as hepatic decompensation in the form of may affect the extrapolation of conclusions
10
jaundice and ascites are rare. on bleeding risk in adults to children. 13
As many as 70-90% of pediatric
patients with portal hypertension have
esophageal varices, with the incidence of
gastrointestinal bleeding due to varicose
vein rupture ranges from 17-42%. The risk
of bleeding can increase to 38% within five
years after varicose veins are diagnosed.12
In the present cohort, the presence of
esophageal varices, primary gastric varices,
and portal hypertensive gastropathy were in
96%, 56%, and 89%, respectively.1 Fig.6. Pathophysiological scheme of occurrence of
varicose vein bleeding
In the pediatric population, EHPVO
is the most frequent cause of non-cirrhotic
Non-cirrhotic portal hypertension
portal hypertension. It is caused mostly by
can be caused by any disease that
phlebitis after umbilical catheterization or
interferes with the blood flow of the portal
omphalitis, previous surgery, dehydration or
venous system, can be pre-hepatic,
a prothrombotic state. Rarely, it is caused
intrahepatic or posthepatic (table 2).6 If
by non-cirrhotic portal fibrosis which
there is obstruction to the portal vein blood
accounts for 4.6% of all causes of non-
flow caused by anything, it can result in an
cirrhotic PH in children.3
increase in portal vein pressure. The high
Many physiologic factors contribute
pressure causes the presence of collateral
to the development of variceal bleeding.
portosystemic in the form of dilatation and
Pressure in the portal system is a product of
opening of the vascular flow that connects
flow and resistance. Variceal wall tension is
the superior-inferior vena cava system with
an additional factor, as is the extent of
the portal vein. Portosystemic collaterals
collateral circulation. Endothelins constrict
also drain blood into the esophageal

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collaterals so that a flow relationship can be can be useful in further defining the vascular
formed between the esophageal varices connections.11
with the vena cava and portal vein.3 The endoscopic outcome of non-
The most frequent clinical symptoms cirrhotic portal fibrosis is favorable. One-
are ariceal bleeding, splenomegaly, and third of patients will have recurrence of
anemia. In 10-34% of patients there is esophageal varices and only a small
ascites. The liver can be normal, proportion of bleeders will have poor
hepatomegaly, or shrunken. Jaundice and outcome. Endoscopic findings followed a
hepatic encephalopathy are very rare (2%).6 well-balanced distribution making statistics
NCPH may present with signs of portal reliable for this cohort.2,4 The classification
hypertension (thrombocytopenia, anemia, of the degree of esophageal varicose veins
splenomegaly, gastrointestinal bleeding), today varies. However, the classification
portal biliopathy (jaundice, elevated alkaline used in general in children is degree zero
phosphatase, elevated gamma-glutamyl (without varicose veins), degree one (small,
transpeptidase), growth failure, or minimal non-winding, and horizontal when
hepatic encephalopathy. With prolonged insufflated), two (winding and occupying
duration of disease, liver dysfunction, nearly one-third of the radius of the distal
including hypoalbuminemia and ascites, esophagus but not flattening when
may develop. 11
Liver structure and function insufflating), and three (large, winding,
remain preserved until late in the course of occupying more than one-third of the radius
the disease, and the most important clinical of the distal esophagus, and confluent). On
presentation is recurrent episodes of the other hand, pediatric endoscopy is an
gastroesophageal variceal bleeding, which invasive or traumatic examination, high
are often well controlled. 3 cost, and requires expertise and is
EHPVO of NCPH can be diagnosed performed in a tertiary facility.14,15
by Doppler ultrasound with 95% sensitivity The present guideline from the
and specificity. The portal vein is Baveno V Consensus Workshop on
transformed into a cavernoma, an irregular diagnosis and therapy in PH proposes a 3-
vascular structure of thin veins visualized size classification and recommend that
near the hepatic hilum which can result in patients with small varices without red signs
elevated portal resistance and compression who have not bled do not require
of the biliary system. Often, a computed prophylaxis, whereas those with medium-to-
tomography scan (CT) or magnetic large oesophageal varices and those with
resonance (MR) angiography/portography

8
red marks, irrespective of size, should Potential medical therapies for acute
benefit from primary prophylaxis.4 variceal bleeding include vasopressin,
Bleeding due to rupture of somatostatin, terlipressin (not available in
esophageal varices requires United States), and octreotide. Vasopressin
pharmacological and non-pharmacological is given by continuous infusion to control
management, namely endoscopic therapy acute variceal bleeding, although its
such as sclerotherapy and ligation, balloon synthetic analog terlipressin has a longer
tamponade, TIPS (Trans Jugular half-life and few side effects. Somatostatin
Intrahepatic Portosistmic Shunt), and and its synthetic analog octreotide are
surgery.16 Endotherapy (EVL/EST) causes similarly effective. These drugs decrease
eradication of esophageal varices in 90%- portal pressure by decreasing splanchnic
95% EHPVO cases. A randomized trial blood flow, although the exact mechanism is
comparing endotherapy and shunt surgery not known. Vasopressin can cause
showed the risk of rebleeding is significantly disturbing peripheral vasoconstriction, but
higher in the endotherapy group. octreotide is associated only with mild
Emergency devascularization procedure is disturbances in glucose homeostasis and
required when endotherapy fails to control abdominal pain. Vasoactive drugs are
acute variceal bleed, interval bleed, or effective in stopping variceal bleeding in
recurrence of bleed following eradication. 2
more than 75% of patients.13
Band ligation therapy has a higher The surgical option for NCPF is a
efficiency in relation to post interventional matter of considerable debate with paucity
bleeding and has a lower complication rate of literature in adults and children. In
and lower costs with a higher overall comparison with the EHPVO model, where
survival. TIPS (Trans jugular intrahepatic Meso–Rex shunt is favored, NCPF patients
portosystemic shunt) or shunt surgery can with predominant spleenrelated issues
be used in therapy-refractory cases. Liver would require a surgery that ameliorates the
transplantation in children is not an option. same. Non-selective porto-systemic shunts
In non-cirrhotic portal hypertension, most such as central end-to-side spleno-renal
children will develop a spontaneous shunt shunt with splenectomy would be a
over time. In those cases, all therapeutic favorable compromise, but it is fraught with
intentions should aim to bridge the time for limitations. In a study of 41 adults with
nature to take over. The role of beta NCPF who underwent prophylactic
blockade therapy in children is not lienorenal shunt, long-term complications
8,17
established yet. observed were hepatic encephalopathy,

9
glomerulonephritis, pulmonary arterio-
venous fistulae and ascites.1
Conclusion
In summary, 14 years old boy who
was diagnosed with haematemesis due to
esophageal varices bleeding in non-cirrhotic
portal hypertension had been treated
conservatively (including platelet TC
transfusion and octreotide) without any
invasive therapy.
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