CASE PRESENTATION

Patient’s identity
Nama :
AHM Father’s name : H
Gender :
Girl Father’s age : 51years old
Age :
3 years old Education : Senior high school
Date of birth :
October 10th, 2019 Job : Entrepreneur
Address :
Jorong Padang, Durian Mother’s name : E
Hijau
Medical records : 01.08.36.77 Mother’s age : 42 years old
Date of admission : September 22nd, 2022 Education : Senior high school

I. ANAMNESIS (Alloanamnesis was taken from parents)

A 3-year-old girl went to the RSUP Dr. M. Djamil pediatric's gastroenterology
outpatient clinic and admitted on March 5th 2022, with a diagnosis of extrahepatic
cholestasis due to biliary atresia, intrahepatic cholestasis due to CMV infection, and
liver cirrhosis with history of recurrent bleeding due to ruptured esophageal varices.

Chief complaint :
Looked pale since one week before admission.

Present Illness History :

Patient looked yellow since aged 1 month. Defecation looked pale or light yellow since
aged 1 month. Abdoment looked bulging since aged 1 month. Recurrent anemia since
aged 1 year. Recurrent bloody vomiting since aged 2 years. Recurrent melena since
aged 2 years. Patient felt itching especially on both of extremities and the patient wants
to rub her skin until she got hurt. Patient will be planned for
esophagogastroduodenoscopy (EGD). Patient well-known with intrahepatic
cholestasis due to CMV infection and the treatment was over and extrahepatic
cholestasis due to biliary atresia, liver cirrhosis with esophageal varices as well. First,
her parents took the patient to a pediatrician in Pasaman when she was 1 month old
and got advice to sunbathe every morning, but the patient still looked yellow. The
parents took the patient again at age 2 months and got the same advice. At the age of
four months, the patient was taken to another pediatrician and referred to a
gastroenterology consultant in Padang, where an abdominal ultrasound revealed
biliary atresia. The patient was referred to RSUP M. Djamil for several examinations,
but the patient came at age 8 months because of the COVID-19 pandemic and was
administered BPJS first. Patient was diagnosed with intrahepatic cholestasis due to
CMV infection, got ganciclovir therapy at age 8 months and complete valganciclovir
therapy on November 2021. Cholangiography was performed in this patient on
November 20, 2021, and the result was biliary atresia and liver cirrhosis. The patient
had varices ligation 2 times before, on August and December 2021. Patient had
screening for ophthalmology and otorhinolaryngology with normal result.

1
 Past Illness History
Patient never had other symptoms before

Family Illness History

There was no family history of icteric or liver disease before.

History of Delivery, Immunization and Development

The patient is the fourth child of four siblings, born by caesarean section due to
preeclampsia, assisted by a doctor at a hospital; her birth weight was 3300 g and her
birth length was 49 cm; she immediately cried. There was no history of shortness of
breath or cyanosis at birth. Patient only received BCG with scar (+), HB0, and polio 1
immunization. Patient learned to prone at 5 months old, sit independently at 12 months
old, stand up at 17 months old, walk at 24 months old, and talk at 12 months old. Now,
the patient talked fluently.

Family and Social Economy History

The patient’s biological mother is 43 years old, graduated from junior high school,
and has a current status as a housewife. The patient’s biological father is 50 years
old, graduated from senior high school, and worked as an entrepreneur. Patient lives
with her father, mother, two older brothers, aged 12 and 6, and one older sister, aged
17 years old. The average income of the family is IDR 5,000,000/month. Patient and
her family live in a permanent house with good environmental hygiene and
sanitation.

II. PHYSICAL EXAMINATION

General condition : Moderately ill

Consciousness : Alert (GCS E4M6V5 )
Blood pressure : 100/60 mmHg (p95)
Heart rate : 118 times per minute
Respiratory rate : 22 times per minute
Temperature : 37°C
Oxygen saturation : 98% (room air)

Nutritional status and anthropometry

Body weight : 13,6 kg Length for age : -2<Z<0 SD
Body length : 93 cm Weight for length : 0<Z<1 SD
Weight for age : 0<Z<1 SD Nutritional status : Well nourished
Systematic physical examination
Skin : Pale, jaundice skin, skin turgor well, rough skin
Head : Round, symmetric, head circumference normocephal [Nellhaus
standard])
Hair : Black
Eyes : Pale conjunctival, icteric sclera, isochoric (2 mm/2 mm) and
reactive pupils
Ears : No abnormality
Nose : Absence of nasal flare

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 Mouth : Wet mucous membrane of oral cavity, macroglossia, absence of
oral thrush
Neck : Absence of lymph node enlargement
Chest : Symmetrical chest movement, absence of chest retraction
Lungs : Vesicular breath sound, bilateral rales
Heart : Normal heart borders, regular rhythm, absence of murmur
Abdomen : Not distended, supple, liver ½-1/2, hard consistency, rough
surface, sharp edged and spleen enlargement S2-3, abdominal
circumference 54 cm, normal peristaltic sound
Genital : No abnormality
Pubertal status : A1M1P1
Extremities : Warm, capillary refilling time < 2 seconds, clubbing finger (+)
Neurologic : Absence of nuchal rigidity and meningeal signs, positive
physical physiologic reflexes, negative pathological reflexes
examination

III. SUPPORTING EXAMINATION

Hematologic and clinical chemistry laboratory data

Parameters Values Impression
Hemoglobin 7,3 g/dl Anemia
White blood cell 9.520/mm3 Normal
Platelet count 99.000/mm3 Thrombocytopenia
Differential count 0/4/0/46/46/4
MCV/MCH/MCHC 77/27/37 Normocytic
normochromic
Reticulocyte 11,54 % (corrected: 7,56%) Reticulocytosis
Peripheral blood Anisocytosis normochromic,
smear polychromic, fragmentocyte
Sodium 136 mmol/l normal
Potassium 3,8 mmol/l Normal
Calsium 9,1mg/dl normal
albumin 2,8 g/dl Normal
PT 11 detik normal
aPTT 24 detik Normal
ureum 17 mg/dl normal
kreatinin 0,4 mg/dl normal
RBG 114 mg/dl Normal
SGOT 296 U/L Increased liver enzyme
SGPT 212 U/L Increased liver enzyme
Albumin 2,5 g/dl Hypoalbuminemia

3
 Examination
Abdominal sonography

Liver : Shape and measurement

were improved on the regular
surface. Parenchyme echostructure
is hyperechoic and homogenous.
biliary system and intrahepatic
vascular not widening. There was
no nodule or SOL. There was
ascites.
Gall bladder : Shape and
measurement were normal.
Thickened wall. There was no
gallbladder stone or sludge
appearance.
Spleen : Shape and measurement
was normal. Homogenous
echostructure. There was no focal
lesion or SOL
Pancreas : Shape and
measurement were normal, with
clear cortex-medulla
differentiation. Pelvicocalices
system not widened. There was no
stone or focal lesion appearance.
Aorta : Normal caliber, there was
no enlargement of the paraaortic
lymph node.
Impression:
Liver cirrhosis with portal
hypertension.
Histopathology Macroscopic :
A supple piece of tissue-colored
dark chocolate with a cross-section
of green chocolate, measuring 0.8
x 0.6 x 0.4 cm.
Microscopic :
Liver tissue sections consist of
hepatocytes separated by the
sinusoid and create lobules that are
separated by fibrotic connective
tissue. There was bile pigment and
hyperemic capillaries.
Impression :
Extrahepatic cholestasis
Esophagoduodenoscopy Esophagus :

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 There were varices on 1/3 of the
distal esophagus with diameters
greater than 3 mm, which were
ligated with a rubber band in three
places in a good position.

Gaster :

There was no edema or active

bleeding, and the mucosa was not
hyperemic.

Conclusion : Esophageal varices

grade III-IV

Advice : Serial EVBL

Brain sonography The fontanelle narrowed, and

fusion began so the examination
area became smaller. The cortical
frontal sulci widened, and the gyri
looked prominent.

There was no widening of the

ventricle system.There was no
hyperechiuc lesion
inta/periventricular that was
visualized.Cerebral falx (+)

Conclusion: Suspected cerebral

atrophy

Echocardiography Situs solitus

Intracardiac normal

Good LV and RV function

Left arch

5
 IV. LIST OF PROBLEMS V. WORKING DIAGNOSIS
1. Liver cirrhosis 1. Esophageal varices due to liver
2. Esophageal varices cirrhosis
3. Jaundice 2. Extrahepatic cholestasis due to
4. Organomegaly biliary atresia
5. Anemia 3. Intrahepatic cholestasis due to
6. Thrombocytopenia CMV infection (relieved)
7. Severe itching 4. Anemia and trombocytopenia
8. Liver function disturbance due to chronic disease
9. Hypoalbuminemia 5. Hypoalbuminemia
10. Incomplete immunization

VI. MANAGEMENT PLAN

1. Esophageal varices due to liver cirrhosis
Diagnostic : anamnesis, physical examination, additional examination
e.g esophagoduodenoscopy (EGD)
Treatment : Spironolactone 1x6,25 mg
Planning : Ligation esophageal varices, consult to digestive surgery
department
Education : The parents were given information about the disease,
diagnosis, management, complication, and prognosis
(dubia ad malam)
2. Extrahepatic cholestasis due to biliary atresia
Diagnostic : anamnesis, physical examination, additional examination
e.g abdominal sonography, cholangiography and
histopatology examination
Treatment : Vitamin A 1x6000 IU po
Vitamin D 1x400 IU po
Vitamin E 1x300 IU po
Vitamin K 2,5 mg po (alternating day)
Urdafalk 3x80 mg po
Definitive therapy : liver transplantation
Planning : Palliative care
Education : The parents were given information about the disease,
diagnosis, management, complication, and prognosis.
 Management: emergency situation
 Monitoring: liver function, coagulation factor, CBC
 Prognosis : dubia ad malam
3. Intrahepatic cholestasis due to CMV infection
Diagnostic : anamnesis, physical examination, laboratory examination
(TORCH serology and PCR CMV serum and urine
mother and daughter)
Treatment : Gancyclovir IV for 14 days and continued with
valganciclovir for 6 months (therapy was completed)
Planning : Observation
Education : The parents were given information about the disease,
diagnosis, management, complication, and prognosis

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 4. Hypoalbuminemia
Diagnostic Anamnesis, additional examination
:
Treatment Albumin transfusion
:
Planning Tranfusion albumin 50 cc in 4 hours
:
Education The parents were given information and counselling
:
about nutrition in this patient
5. Anemia normocytic normochrome due to chronic disease
Diagnostic : Anamnesis, physical examination, additional
examination
Treatment : Transfusion PRC target Hb 12 g/dl
Planning : Transfusion PRC 1x100 cc, 1x150 cc
Eduacation : Educate mother the risk of tranfusion
6. Incomplete immunization
Diagnostic : Anamnesis
Treatment : -
Planning : Catch up immunization that did not worsened disease
Education : Educate mother that immunization safe for this patient

Figure 1. Patient at outpatient clinic

VII. MONITORING DAY

Follow Up March 5th, 2022
Subjective : Patient still looked pale, patient has no fever, patient has no bleeding,
good tolerance of oral intake.
Objective : Patient was moderately ill. BP 107/78 mmHg (p95), HR 118x/m, RR
24x/m, T 36,7 C, SpO2 98%. Conjunctiva was anemic, sclera was icetric both pupils
were reactive. Chest movement were symmetrical, air entry were clear on both lungs,
no abdominal distension, liver was palpable ½-1/2 sharp edge, and spleen was palpable
S2-3, bowel sound was normal. Extremities were warm, CRT < 2 seconds, clubbing
finger (+).

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Assessments : Esophageal varices due to liver cirrhosis, extrahepatic cholestasis due
to biliary atresia, intrahepatic cholestasis due to CMV infection (relieved), anemia and
trombocytopenia due to chronic disease, hypoalbuminemia.
Plan : Liquid meal hepatic diet 6x175 cc, PRC transfusion 150cc, endoscopic variceal
band ligation, albumin transfusion 50cc, urdafalk 3x100 mg po, propranolol 3x4 mg
po, phytomenadion 1x2,5 mg po alternating day, vitamin A 1x6000 IU po, vitamin D
1x400 IU po, vitamin E 1x200 IU po.

Follow Up March 6-10th, 2022

Subjective : Patient was not pale, patient has no fever, patient has no bleeding, good
tolerance of oral intake.
Objective : Patient was moderately ill. BP 100/70 mmHg (p95), HR 115x/m, RR
24x/m, T 36,7 C, SpO2 98%. Conjunctiva was anemic, sclera was icetric both pupils
were reactive. Chest movement were symmetrical, air entry were clear on both lungs,
no abdominal distension, liver was palpable ½-1/2 sharp edge, and spleen was palpable
S2-3, bowel sound was normal. Extremities were warm, CRT < 2 seconds, clubbing
finger (+). Hb 11,9 g/dL, leucocyte 9170/mm3, platelet 173.000/mm3, differential
count 0/4/2/51/40/3, SGOT 296 U/L, SGPT 212 U/L, sodium 136 mmol/L, potassium
3,8 mmol/L, calcium 9,1 mg/dL, ureum 17 mg/dL, creatinine 0,4 mg/dl, PT 11,2
second, aPTT 25,5 second.
Assessments : Esophageal varices due to liver cirrhosis, extrahepatic cholestasis due
to biliary atresia, intrahepatic cholestasis due to CMV infection (relieved), anemia and
trombocytopenia due to chronic disease, hypoalbuminemia.
Plan : Endoscopic variceal band ligation (EVBL), liquid meal hepatic diet 6x175 cc,
urdafalk 3x100 mg po, propranolol 3x4 mg po, phytomenadion 1x2,5 mg po
alternating day, vitamin A 1x6000 IU po, vitamin D 1x400 IU po, vitamin E 1x200 IU
po.

Follow Up March 11-14th, 2022

Subjective : EVBL was done, patient was not pale, patient has no fever, patient has
no bleeding, patient was fasting temporary after surgery.
Objective : Patient was moderately ill. BP 100/70 mmHg (p95), HR 115x/m, RR
24x/m, T 36,7 C, SpO2 98%. Conjunctiva was anemic, sclera was icetric both pupils
were reactive. Chest movement were symmetrical, air entry were clear on both lungs,
no abdominal distension, liver was palpable ½-1/2 sharp edge, and spleen was palpable
S2-3, bowel sound was normal. Extremities were warm, CRT < 2 seconds, clubbing
finger(+). Hb 9,6 g/dl, leucocyte 10.960/mm3, platelet 145.000/mm3, hematocrit 33%,
differential count 0/5/3/42/45/5.
Assessments : Esophageal varices due to liver cirrhosis, extrahepatic cholestasis due
to biliary atresia, intrahepatic cholestasis due to CMV infection (relieved), anemia and
trombocytopenia due to chronic disease.
Plan : PRC transfusion 150cc, IVFD D12,5% + e 1150 cc/24 hr, urdafalk 3x100 mg
po, propranolol 3x4 mg po, phytomenadion 1x2,5 mg po alternating day, vitamin A
1x6000 IU po, vitamin D 1x400 IU po, vitamin E 1x200 IU po, patient planned to
discharge after good tolerance of intake and stable condition.

8
 Figure 3. EVBL Arsylla

Admission on November 15-21st 2022

Subjective: Patient looked pale 2 days before admission. There was no gastro
intestinal bleeding.
Objective: Patient was moderately ill. BP 100/70 mmHg (p95), HR 114x/m, RR
24x/m, T 36,7 C, SpO2 99%. Conjunctiva was anemic, sclera was icetric both pupils
were reactive. Chest movement were symmetrical, air entry were clear on both lungs,
no abdominal distension, liver was palpable 3/4-1/2 sharp edge, and spleen was
palpable S3, bowel sound was normal. Extremities were warm, CRT < 2 seconds,
clubbing finger(+). Hb 9,6 g/dl, leucocyte 10.960/mm3, platelet 145.000/mm3,
hematocrit 33%, differential count 0/5/3/42/45/5, PT 10,7 second, aPTT 20,9 second,
albumin 3 g/dL.
Assessments: Esophageal varices due to liver cirrhosis, extrahepatic cholestasis due
to biliary atresia, intrahepatic cholestasis due to CMV infection (relieved), portal
hypertension, anemia and trombocytopenia due to chronic disease.
Plan: Soft meal hepatic diet 1300 kkal, PRC transfusion 2x150 cc, urdafalk 3x100 mg,
propranolol 3x6 mg po, vitamin A 1x5000 IU po, vitamin D 1x400 IU po, vitamin E
1x100 IU po, phytomenadion 1x2,5 mg po AD, spironolactone 1x12,5 mg po.
Discharged after condition better.

Admission on February 22nd-March 4th 2023

Subjective: Patient looked pale one week before admission. Patient got hematemesis
about 100cc when admitted in ward. The was no melena. Patient on temporary fasting.
Objective: Patient was moderately ill. BP 100/70 mmHg (p95), HR 114x/m, RR
24x/m, T 36,7 C, SpO2 99%. Conjunctiva was anemic, sclera was icetric both pupils
were reactive. Chest movement were symmetrical, air entry were clear on both lungs,
no abdominal distension, liver was palpable 3/4-1/2 sharp edge, and spleen was
palpable S3, bowel sound was normal. Extremities were warm, CRT < 2 seconds,
clubbing finger(+). Hb 8 g/dl, leucocyte 11.070/mm3, platelet 172.000/mm3,
hematocrit 26%, differential count 0/5/0/53/34/8, PT 10,8 second, aPTT 30,5 second,
albumin 2,8 g/dL, total bilirubin 20 mg/dl, direct bilirubin 13, 8 mg/dl, indirect
bilirubin 6,2 mg/dl, alkaline phosphatase 654 U/L, gamma GT 173 U/L, SGOT 269
U/L, SGPT 126 U/L.
Assessments: Hematemesis due to ruptured of esophageal varices, esophageal varices
due to liver cirrhosis, extrahepatic cholestasis due to biliary atresia, intrahepatic
cholestasis due to CMV infection (relieved), portal hypertension, anemia due to
chronic disease and hemorrhage.

9
Plan: EBVL, IVFD D12,5% + e 1200 cc/24 hour, PRC transfusion 2x125 cc, urdafalk
3x100 mg, propranolol 3x6 mg po, vitamin A 1x5000 IU po, vitamin D 1x400 IU po,
vitamin E 1x100 IU po, phytomenadion 1x2,5 mg po AD, spironolactone 1x12,5 mg
po, HP pro 1x1 caps, curcuma 1x1 tab po. Patienr got discharged after bleeding stop
and EVBL done.

Figure 4. EBVL Arsyila on March 1st 2023

VIII. PROGNOSIS
Ad vitam : malam
Ad functionam : malam
Ad sanationam : malam

IX. SCHEME DISEASE HISTORY

June 2020
November 2019
 Patient diagnosed with CMV infection from
Patient looked yellow serology and PCR examination, and biliary
Defecation looked pale atresia from abdominal ultrasound
Abdoment looked  Patient got therapy ganciclovir IV continued
bulging with valganciclovir orally

August 2021
Since November 2020
Got EBVL
Recurrent anemia and
got PRC transfusion

Since November 2021

March, May
 Recurrent gastrointestinal 2022, March
bleeding,hematemesis and melena 20223
 IOC : billary atresia and liver
Got EBVL
cirrhosis, got EBVL

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X. DISEASE ANALYSIS SCHEME

Viral infection including Cytomegalovirus (CMV), Epstein-Barr

Risk
virus (EBV), and Human papillomavirus (HPV), and hepatotoxins

Cholestasis Portal Esophageal GIT

Problems
hypertension varices bleeding

Anemia

Diagnosis Intrahepatic Extrahepatic GIT bleeding and

Cholestasis due to Cholestasis due anemia due to
CMV infection to billiary atresia ruptured
esophageal varices

Gancyclovir Kasai EBVL

Management portoenterostomy Sclerotherapy
Valgancyclovir po Liver transplantation Beta blocker

Ad vitam : malam
Prognosis Ad functionam : malam
Ad sanationam : malam

XI. CASE ANALYSIS

Neonatal/infantile cholestasis, which affects 1 in 2500 children at term, is defined as

an early-life impairment in bile formation or flow that results in the retention of biliary
substances within the liver. It can be caused by biliary or hepatocellular diseases
(genetic and metabolic defects disrupting bile composition and transport) or by a
combination of the two. It is critical to identify the underlying cause as soon as possible
in order to begin appropriate surgical or medical treatment. The most common cause
of neonatal/infantile cholestasis is biliary atresia, which necessitates an early surgical
referral to maximize success rates. In the case of treatable metabolic conditions,
prompt specific treatment and a better outcome are unquestionably warranted.1

11
Cholestasis in infancy is defined as a serum conjugated/direct bilirubin level greater
than 1 mg/dL (17 mol/L) and greater than 20% of total bilirubin.1 Although 2 mg/dL
(34 mol/L) was previously used arbitrarily as a threshold for conjugated/direct
bilirubin, it has recently been reduced to 1 mg/dL to more accurately reflect clinical
experience. Recent studies suggest that lower direct/conjugated bilirubin levels (>0.3-
0.5 mg/dl) and direct/conjugated bilirubin levels >10% of total bilirubin during the
first 5 days of life are abnormal and should raise the suspicion for cholestasis at this
age and warrant further evaluation. Jaundice beyond 2 weeks of age in a breast-fed
infant or at 2 weeks of age in a formula-fed infant should elicit fractionation of the
serum bilirubin to differentiate the much more common breast milk-associated indirect
hyperbilirubinemia from cholestasis. If conjugated/direct hyperbilirubinemia is
identified, further evaluation for hepatobiliary causes should proceed immediately.2
CMV infection is the most common congenital infection, affecting about
0.6% of all newborns in developed countries. Vertical transmission to the fetus can
occur following either primary or secondary CMV infection in the mother during
pregnancy. It is the most common infectious agent that causes sensorineural hearing
loss in young children. (SNHL). Congenital CMV (cCMV) can cause hearing loss at
birth or develop months or years later. We had hearing screening for this patient with
normal result.3
The etiology of billiary atresia (BA) is complicated, with both virus and
hepatotoxin exposure appearing to be important factors. The fact that elevated bilirubin
levels can be detected at birth in children who will develop BA suggests that exposure
occurs during the fetal stage. BA has been linked to a number of viruses, including
Cytomegalovirus (CMV), Epstein-Barr virus (EBV), and Human papillomavirus
(HPV), and hepatotoxins, such as the plant toxin biliatresone, can induce BA-like
pathology in various species. BA is divided into several subcategories. BA is classified
as isolated (70-80%), syndromic (10-15%, including Biliary Atresia Splenic
Malformation (BASM), cystic (5-10%), and CMV IgM+ (approximately 10%) in one
classification scheme.4 Patient diagnosed with biliary atresia from intraoperative
chonagiography and abdominal sonography.
The current standard of care for BA is sequential surgery, beginning with a Kasai
hepato-portoenterostomy (KPE), in which the obstructed bile duct is resected and a
loop of the small bowel is brought to the liver's porta hepatis to restore bile flow,
followed by liver transplantation for those who fail the KP or progress to cirrhosis and
liver failure later in childhood or into adulthood. Without surgical intervention, all
infants with BA will die before the age of three. BA is a global disease that affects
people of all ethnicities. According to a recent comprehensive review, the incidence
of BA varies greatly among countries reporting population-based data, ranging from
approximately 1:5000 newborns in Taiwan to 1:20,000 in Europe, Canada, and parts
of the United States. French Polynesia reported the highest rates (1:3500). 5
Cirrhosis in children is a common complication of a variety of genetic,
infectious, inflammatory, vascular, and cholestatic diseases. It is usually the result of
one of two processes: a hepatotoxic lesion (infectious, metabolic, or fatty liver) or a
cholestatic lesion. The latter is usually caused by bile flow obstruction, specifically
biliary atresia (BA). The majority of chronic liver disease (CLD) cases can progress to
liver fibrosis, cirrhosis, and even hepatocellular carcinoma. 6

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 Figure 3. Patophysiology portal hypertension

There are two clinical stages of liver cirrhosis: compensated cirrhosis,

which is not clinically significant (asymptomatic, stable patients), and decompensated
cirrhosis, which has complications related to portal hypertension and/or the presence
of liver failure. It can manifest as acute or chronic liver failure as a result of a
precipitating event, such as a viral, bacterial, or toxic infection. In children, there is
usually a history of previous liver damage, which progresses and continues until the
development of liver failure and patient death. 6 Portal hypertension is defined as
hepatic venous pressure gradient (HVPG) greater than 5 mmHg. HVPG greater than
10 mmHg is associated with the development of esophageal varices and greater than
12 mmHg is associated with variceal bleeding and ascites. In children, extrahepatic
portal vein thrombosis (EHVPO) is the most common cause of portal hypertension,
followed by biliary atresia. Gastrointestinal bleeding secondary to esophageal or
gastric varices , presenting as hematemesis or melena, may be the initial manifestation
of portal hypertension. Ascites is the other complication of portal hypertension.
Ascites occurs when osmotic and hydrostatic pressure within hepatic and mesenteric
capillaries exceeds the drainage capacity of lymphatics and excess fluids accumulates
in the peritoneal space. We found gastroeintestinal bleeding like hematemesis and
ascites in this patient.7,8

Figure 4. Classification esophageal varices and gastric varices

13
The risk of bleeding and the incidence of gastroesophageal varices in children are
disease dependent. Large varices, elevated prothrombin time, ascites, increased total
bilirubin, variceal red markings, and the presence of gastric varices all increase the risk
of bleeding. According to one long-term study of children with BA, the risk of bleeding
is 27%. A cross-sectional study of children with BA found that 19% had GI bleeding,
with 7% having multiple episodes. The age at which patients begin bleeding also
depends on the underlying cause of cirrhosis, with patients experiencing surgically
corrected but progressive BA bleeding for the first time at an average age of three
years.7 Patient in this case started bleeding at age 2 years.
The treatment of children with portal hypertension and variceal bleeding
is still debatable. Primary prophylaxis with endoscopic variceal ligation (EVL),
endoscopic variceal sclerotherapy (EVS), or nonselective beta blockers (NSBBs) is
not recommended due to a lack of efficacy and safety evidence to prevent EVB or
improve survival, according to the summary from the Baveno VI Pediatric Satellite
Symposium. Because it is more safety and effective than EVS as a secondary
prophylaxis, EVL is preferred. MesoRex Bypass should be offered to children with
EHPVO for both primary and secondary prevention of variceal bleeding. EVL has
been used for hemostasis in acute EVB as well as for prophylactic banding of medium
to large EVs.9,7,10
Beta blockers decrease portal venous flow by unopposed a-receptor-
mediated splanchnic vasoconstriction, resulting in lower portal pressure. Beta-
blockade also reduces cardiac output and the constriction of intrahepatic
myofibroblasts, activated stellate cells, and vascular smooth muscle cells caused by
norepinephrine. Over a median 2-year follow-up, a meta-analysis of nonselective beta-
blockade (NSBB) as the primary prophylaxis in adults with cirrhosis revealed a
reduction in the rate of first variceal bleed and mortality. The beta-blocker dose is
titrated to achieve a 25% decrease in heart rate or the maximum tolerated dose. 7
Propranolol was prescribed after EGD confirmed EV formation. Propranolol dosage
was gradually increased from 0.5 mg per kilogram per day to 1 mg per kilogram per
day. The maximum daily dose, regardless of body weight, would be 30 mg. If tolerated,
propranolol could be used indefinitely. Following an acute EVB event, EGD
examinations were performed every two weeks to one month, every 1-3 months for
medium to large EVs, and every 6-12 months in stable patients.9 Patient got beta
blocker therapy to decrease portal venous flow.
The treatment of patients with portal hypertension and gastrointestinal
bleeding for the first steps are to protect the airway, ensure that patients are breathing,
and maintain circulation. A nasogastric tube should be inserted to monitor the ongoing
bleed and to remove blood from the GI tract, which can predispose cirrhotic patients
to encephalopathy. In the case of acute esophageal variceal hemorrhage, prophylactic
antibiotics are advised; all patients should begin vasoactive drug therapy, such as
octreotide, as soon as possible. These medications work by decreasing splanchnic
blood flow, portal venous inflow, and portal pressure. Octreotide has been shown to
safely slow the rate of GI bleeding in children with varices when given as a bolus (1
mg/kg) followed by a continuous infusion (1-5 mg/kg/h) or as subcutaneous injections
3 times daily.7
Once patients are stable, an endoscopy should be performed as soon as
possible to determine the source of the bleeding. Recent guidelines recommend giving
erythromycin 3 mg/kg intravenously over 30 minutes before endoscopy to improve

14
stomach emptying and endoscopy visualization. Patients who do not respond to fluids,
vasoactive medications, or coagulopathy correction may require emergent endoscopy
and, in rare cases, the placement of a balloon tamponade device, emergent surgical
intervention, or TIPS. Endoscopic variceal ligation has successfully replaced
endoscopic sclerotherapy for the treatment of esophageal varices in both adults and
children. Sclerotherapy is currently used in pediatrics only in infants and small
children (10 kg) whose band ligation device is too large to pass through the upper
esophageal sphincter. Sclerotherapy injections can be intravariceal, causing thrombus
formation within the vessel, or paravariceal, causing local inflammation and
compressing the vessel. Sclerotherapy is an effective treatment for variceal bleeding,
but it is associated with more complications than EVL, such as aspiration pneumonia
and esophageal stricture. EVL causes varix thrombosis, and the band and varix slough
off together in about 5 to 7 days. To reduce the risk of treatment failure after EVL,
long-term administration of proton pump inhibitors should be considered. 710 Primary
prophylactic endoscopic variceal ligation for high-risk varices had no effect on overall
survival or transplant-free survival in biliary atresia patients. Prophylactic banding for
high-risk EVs, on the other hand, may reduce the possibility of acute EVB and delay
the first occurrence, potentially reducing bleeding-related complications and the
psychological burdens on caregivers and patients.9 Esophageal varices are a leading
cause of massive bleeding. Despite therapeutic advances, Acute Upper
Gastrointestinal Bleeding episodes caused by esophageal varices kill 5%-19% of
children with portal hypertension. A study in Italy suggests EVL 12-24 h after acute
upper gastrointestinal bleeding.11 Secondary prophylaxis was effective in eliminating
esophageal varices and controlling new upper gastrointestinal bleeding episodes
caused by varices rupture. Band ligation appears to have lower rebleeding rates and
required fewer sessions to eradicate varices than sclerotherapy. 12

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 EVIDENCE BASED MEDICINE

A. Clinical Question
How about outcome of endoscopy band variceal ligation for prophylaxis in
pediatric patient with esophageal varices ?

B. Component of foreground question (PICO)

Population/problem : children/adolescent with esophageal varices
Intervention : endoscopy
Comparator :-
Outcome : Band ligation was performed in 34 (40%) patients and
sclerotherapy in 51 (60%) patients. Esophageal varices
were eradicated in 81.2%, after a median of four
endoscopic sessions. Varices relapsed in 38 (55.1%)
patients. Thirty-six (42.3%) patients experienced
rebleeding, and it was more prevalent in the group that
received sclerotherapy. Gastric varices and portal
hypertensive gastropathy developed in 38.7% and
57.9% of patients, respectively. Patients undergoing
band ligation showed lower rebleeding rates (26.5% vs
52.9%) and fewer sessions required for eradication of
esophageal varices (3.5 vs 5) Secondary prophylaxis
was effective in eradicating esophageal varices and
controlling new upper gastrointestinal bleeding
episodes due to the rupture of esophageal varices. Band
ligation seems that resulted in lower rebleeding rates
and fewer sessions required to eradicate varices than
did sclerotherapy

C. Searching Method
Investigation was done based on keywords “esophageal varices, seizure,
endoscopy, children”. By using limitation of the study in human and establish
within the last 5 years, found an article answered the clinical question with title:
Evaluation of secondary endoscopic prophylaxis in children and adolescents with
esophageal varices. Authors: Pimenta JR, Ferreira AR, Fagundes EDT,
Bittencourt PFS, Moura AM, Carvalho SD.

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