TIMELINE DIAGRAM

15th September 2021 28th September 2021 14th October 2021 21st October 2021 9th November 2021

Patient was admitted to an Patient was admitted to Patient was admitted to

ophthalmologist at L hospital pediatric polyclinic and pediatric ward at W hospital
ophthalmologist ward at W Initial observation by End of observation Report
hospital candidate

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 PATIENT’S IDENTITY
Name : PCV Father : Mr. YV
Gender : Female Father’s age : 31-years old
Age : 1-years-6-months Education : S1
Date of birth : 29th March 2020 Occupation : Swasta
MR : 947XXX Mother : Mrs. YS
Address : East Manggarai, NTT Mother’s age : 28-years old
Admission date : 14th October 2021 Education : S1
Length of stay: 7 days Occupation : Civil Servant

Initial observation by candidate began on 28th October 2021

PATIENT’S HISTORY
Chief complaint (Alloanamnesis from the mother)
Swollen and redness in the left eye in the left eye that have been noticed
since 1 months ago.

History of present illness

1 months prior to hospital admission, the parents took the patient
polyclinic at L hospital with chief complaint of swollen and redness in the left
eye since 2 weeks ago. Parents also noticed that there are white spot (cats
eye reflexes) in the left eye since 2 months. Initially around the age of 7
months, parents noticed their child's eyes like white spots on the left eyeball.
She had no fever, cough, dyspnea, nausea and vomiting. Her appetite was
decreased. Defecation and micturition were unremarkable. No history of gum
bleeding, epistaxis, hematemesis, melena, nor hematuria. No history of
frequent fever and joint pain. She had no history of pale or blood transfusion
before. The patient was then diagnosed with bilateral retinoblastoma and
was referred to the ophthalmology clinic at W hospital.

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CT Scan results date 22/9/2021
Right orbitals: The eyeball is normal in shape, heterogeneous thickening
appears on the posterior wall of the right bulbus with calcification. The
area of the intraconal is reduced in size with decreased optic nerve.
Left orbitals: The eyeball is irregular in shape, heterogeneous lesions
appear and heterogeneous thickening on the posterior wall of the left
bulbus oculi with calcification. The area of the intraconal appears
occluded (28 Hu) in all paranasal sinuses.
Impression: Suggestive of bilateral retinoblastoma oculi with optic nerve
atrophy.

15 days prior to hospital admission, the parents took the patient polyclinic
eye at W hospital with chief complaint of swollen and redness in the left eye
which has been noticed since 1 months ago. Parents also complained about
the cats eye reflexes in the left eye since 2 months. Initially around the age of
7 months, parents noticed their child's eyes like white spots on the left
eyeball. She had no fever, cough, dyspnea, nausea and vomiting. Her
appetite was decreased. Defecation and micturition were unremarkable. No
history of gum bleeding, epistaxis, hematemesis, melena, nor hematuria. No
history of frequent fever and joint pain. She had no history of pale or blood
transfusion before.
The patient was admitted for 3 days for examination of the posterior segment
of the eye under general anesthesia and in collaboration with the pediatric
hematoncology department. Then the patient was diagnosed with bilateral
retinoblastoma oculi and was referred to the pediatric hemato oncology
division for chemotherapy.

3
Results of the Evaluation Under Anesthesia Examination from the eye area
of the W hospital (5/10/21):

Anterior segment of right eye: Palpebra is not edematous, cilia is not

secreted, conjunctiva is not hyperemic, clear cornea measuring 11x11.5 mm,
pharmacologically dilated round pupil, clear lens, fundus reflex is present.
Posterior segment of right eyeball: Positive fundus reflex, partially visualized
optic nerve papillae, CDR difficult to evaluate Peripheral retinal vein 2/3rd
artery, T1 tumor mass seen from posterior temporal penetrating vitreous
cavity but not reaching lens, T2 tumor mass seen in posterior pole superior to
optic nerve but limited in the intra-retinal layer and vitreous seedding around
eye tumors. In the right eye, the visual acuity was within normal limits.
Left eye anterior segment: The eyelids are not edematous, the cilia
have no hyperemic conjunctival secretions, the cornea is cloudy with corneal
blood staining measuring 11.5 x 11.5 mm, other details are difficult to
evaluate, the fundus reflex is absent, so a posterior segment examination
was not performed. In the left eye, the eye's vision only reacts to light.
Complete blood count (06-10-2021) revealed Hb 10,2 g/dl, Hct 30,9%,
MCV 70,2 fL, MCH 23,2 pg, erythrocyte 4.400.000/mm3, reticulocyte 1,29%,
platelet 398.000/mm3, WBC 9.300/mm3, ANC 3.150/µl, ALC 4.340/ µL,
neutrophyl 33,8%, lymphocyte 46,5%, monocyte 6,3%, eosinophil 0,9% dan
basophil 0,4%. Natrium 142 mmol/l, kalium 4,4 mmol/l, chloride 110 mmol/l.
Ureum 16 mg/dl, creatinine 0,3 mg/dl, blood glucose 82 mg/dl, SGOT 31 U/l,
SGPT 10 U/l, albumin 4,4 gr/dl.
Peripheral Blood Smear (06-10-2021) revealed monocytosis cause infection.
Chest X-ray (27-09-2021) revealed no abnormalities on this chest
radiograph.

On the day of admission, patient was moderately ill, she had swollen and
redness in the left eye which has been noticed since 1 months ago. Parents
also complained about the cats eye reflexes in the left eye since 3 months.

4
Initially around the age of 7 months, parents notice their child's eyes like
white spots on both eyeballs. There is decreased in vision in the left eye
which has not been noticed. She had no fever, cough, dyspnea, nausea and
vomiting. Her appetite was decreased. Defecation and micturition were
unremarkable. On physical examination at Oculi sinistra, there is no
proptosis, there is cat eye reflex, there is leukocoria, there is palpebral
edema, there is hyperemic conjunctiva, there is no blood and pus. Oculi
dextra, no proptosis, no cat eye reflex, no secretions, no blood and pus.
There is no lymphadenopathy. No organomegaly. No spontaneous bleeding
manifestations.

History of past illness

- No history of severe illness
Conclusion: The patient seemed to have showed symptoms and signs
at left eyes since 3 months prior to hospitalization. Before that, the
patients have complained of leukocoria in the left eye from the age of 7
months.
History of illness in the family or relatives
Patient’s family pedigree

Note:
: Male
: Female
: Patient

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 - No history of bleeding disorder in the family
- No history of malignancy in the family
Conclusion: unremarkable history of illness in the family

Prenatal and perinatal history

Patient is the second child who was born from a planned pregnancy. Her
parents were married when his mother was 25 years old and his father was
28 years old. Mother was pregnant at the age 26 years. During pregnancy,
the mother routinely visit community health center and midwife for antenatal
care. She also took iron supplementation and vitamins. She never
consumed any herbs or non-prescribed medicines. She also never got sick
or had any trauma during pregnancy. She received 2 tetanus toxoid
injections. She didn't smoke but she frequently exposed to cigarette smoke.
Patient was born at term through spontaneous labor, immediately cried and
weighed 3300 grams and body length cannot remember. She had no history
of breathing difficulty, cyanosis or jaundice. She was exclusively breastfed
until she was 6 months of age.
Conclusion: The mother had no complaint during pregnancy and the
patient was born healthy, but her mother frequently exposed to
cigarette smoke.

History of vaccination
The vaccination that has been given is hepatitis B0 (at birth), Polio 0 ( at 2
days), BCG (at 1 month old), pentabio I ( Hepatitis B 2, DPT 1, HiB 1 and
polio 1) at 2 months old, pentabio II ( Hepatitis B 3, DPT 2, HiB 2 and polio 2)
at 3 months, pentabio III ( Hepatitis B 3, DPT 3, HiB 3 and polio 3) at 4
months old, and measles 1 time (at 9-months-old).
Conclusion: basic vaccination was complete.

6
Nutritional history
Patient was exclusively breastfed until he was 6-months-old and was
continued until the patient was 18-months-old. Complementary food was
started after 6 months of age. Family food was started at 1-year-old. The
patient feeds 3 times a day, with food comprise of rice, fish, eggs,
tahu/tempe, vegetables and fruits. His appetite was good until recently.
Conclusion: intake quality and quantity was adequate, the appetite
decreased only recently.

Growth and developmental history

Patient was able to roll over at 3-months-old, sat without support at 6-
months-old, and spoke at 11-months-old. He started to stand at 12-months-
old and was able to walk by 1-year-2-months-old. He gets along well with
children his age.

Patient’s birth weight was 3300 grams, and body length cannot remember.
Although her weight and height were always measured at community health
center at every vaccination visit, but they were not recorded at medical
record, and the mother did not remember either. Her mother recalled being
told that the growth was good. Patient doesn’t seem to be shorter or thinner,
compared to children his age. On admission patient weighed 10 kg, and her
height was 80 cm.
Conclusion: Patient had normal growth and development.

History of basic needs fulfillment (Love, care, stimulation)

Patient was raised lovingly by her parents. Parents-child relationship seems
close. During hospitalization, both parents always accompany her and taking
care of her during hospital stay. Early stimulation was provided by both
parents by playing together. The mother plays a bigger role, since she has
more time together.

7
Conclusion: both parents are the primary caretaker for the patient.
Parents are able to fulfill clothing, food and housing needs. Medical
expenses are covered by BPJS.

Socio-economic, psychological and environmental history

The father is a 31 years old farmer with monthly income ranged from IDR
3.000.000 to 5.000.000. The mother is a 28 years old civil servant with
monthly income ranged from IDR 3.000.000 to 4.000.000. Both are from
Labuan Bajo and both are Chatolic. Patient lives with both his parent in a
house sized 7x10 m2 with 1 living room, 1 kitchen-dining room, 1 bathroom, 2
bedrooms. The house is located in the rural area. There’re no factories
around the neighborhood. The nearest health facilities are a Posyandu
approximately 500 meters away. Community health center is 1 Kms away,
school (junior high school) is 1 km away, and the church is 50 meters away.
Medical expenses are covered by health insurance (BPJS mandiri). During
his treatment at the hospital, patient and his parents are staying at a shelter
house.
Conclusion: family belong to a medium socio-economic class.

PATIENT’S HOSPITAL ADMISSION SUMMARY

Prior to candidate’s initial observation (28th October 2021)
A 1-year-6-month old girl was referred to W hospital with chief complaint of
swollen and redness in the left eye which has been noticed since 1 month
ago. Parents complained about swollen and redness in the left eye which has
been noticed since 1 months ago. Parents also complained about the cats
eye reflexes in the left eye since 3 months. Initially around the age of 7
months, parents noticed their child's eyes like white spots on the left eyeball.
There is decreased in vision in the right eye which has not been noticed. She
had no fever, cough, dyspnea, nausea and vomiting. Her appetite was
decreased. Defecation and micturition were unremarkable. No history of gum
bleeding, epistaxis, hematemesis, melena, nor hematuria. No history of

8
frequent fever and joint pain. She had no history of pale or blood transfusion
before. No history of frequent fever and joint pain.

PHYSICAL EXAMINATION (pediatric ward, 14th October 2021)

General condition : Moderate illness
Consciousness : conscious (GCS 15 E4M6V5)

Vital signs
Blood pressure : 90/60 mmHg
Pulse : 100 times/minute, regular
Respiratory rate : 24 times/minute, regular
Temperature : 36,7 oC
FLACC pain scale :0

Anthropometric and nutritional status

Body weight : 10 kg
Body height : 80 cm
Head circumference : 48 cm (normal: 44,5-49,5 cm)
Mid upper arm circ. : 14 cm
Weight for height : between median and -2SD (good nourished,
based on WHO z-score curve)
Height for age : between median and -2SD (Normal stature)
Weight for age : between median and -2SD (Normal)
MUAC/A : 15/15,9 x 100% = 94,3% (Normal)
Father’s body height 170 cm, mother’s body height 156 cm, midparental
height 156,5 cm
Genetic potential height : 148 -165 cm.
Conclusion: good nourished, normal stature

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Table 1. General examination
Organ Description
Head Normocephal
Hair Black
Face Symmetric , no paralysis of cranialis nerves
Eye Oculi sinistra, there is no proptosis, there is cat eye
reflex, there is leukocoria, there is palpebral edema,
there is hyperemic conjunctiva, there is no blood and
pus. Oculi dextra, no proptosis, no cat eye reflex, no
secretions, no blood and pus.
Nose Nasal septum in the middle, no epistaxis nor secretions
Ear No bleeding nor secretion, intact tympanic membrane.
Mouth No gum bleeding. No stomatitis nor moniliasis. Tonsil
size T1-T1, not hyperemic. Pharynx not hyperemic.
Teeth No dental cavities.
Neck No neck stiffness.
Chest Symmetrical shape and movement.
Lung Vocal fremitus symmetrical, percussion sonor, vesicular
breath sound, no additional breath sound (wheezing and
rales).
Heart Heart sound I-II normal, no murmur nor gallop.
Abdominal No abdominal distention, normal peristaltic, liver and
spleen no palpable.
Genital Normal.
Skin BCG scar is present. No ptechie
Lymph node No lymphadenopathy
Pubertal status A1M1P1

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Extremities Warm on palpation, CRT <2 seconds, no wasting nor
edema, Mid upperarm circumference 14 cm.
Physiological BPR, TPR, KPR and APR appear normal reflexes
Pathological Babinsky, Gordon, Openheim, and Chaddock negative

LABORATORY EXAMINATION
Table 1. Urinalysis
Hospital W
14-10-2021
Color Yellow
Clarity Clear
Glucose Negative
Billirubin Negative
Ketones Negative
Ph 6,5
Specific gravity 1.015
Protein urine Negative
Nitrites Negative
Blood Negative
Leukocyte esterase Negative

SUMMARY
PCV, A 1-year-6-month old girl was referred to W hospital with chief
complaint of bilateral retinoblastoma chemotherapy plan. Parents complained
about swollen and redness in the left eye which has been noticed since 1
months ago. Parents also complained about the cats eye reflexes in the left
eye since 3 months. Initially around the age of 7 months, parents noticed
their child's eyes like white spots on the left eyeball. There is decreased in
vision in the left eye which has not been noticed. She had no fever, cough,
dyspnea, nausea and vomiting. Her appetite was decreased. Defecation and
micturition were unremarkable. No history of gum bleeding, epistaxis,
hematemesis, melena, nor hematuria. No history of frequent fever and joint

11
pain. She had no history of pale or blood transfusion before. No history of
frequent fever and joint pain.
She was go to polyclinic in L hospital with chief, left eye swelling and
redness and diagnosed with bilateral retinoblastoma and referred to
ophthalmology polyclinic at W hospital. At W hospital, the patient was
hospitalized for 3 days in the ophthalmology department in collaboration with
the pediatric division of haematology and was diagnosed with bilateral grade
IV retinoblastoma and then planned to undergo chemotherapy.
Physical examination on the observation by candidate revealed a
moderately ill, conscious, good nourished girl, weighed 10 kg, and height 80
cm. Vital signs were normal. Oculi sinistra, there is no proptosis, there is cat
eye reflex, there is leukocoria, there is palpebral edema, there is hyperemic
conjunctiva, there is no blood and pus. Oculi dextra, no proptosis, no cat eye
reflex, no secretions, no blood and pus. No organomegaly and no bleeding
manifestation.
Laboratory examination revealed anemia. Peripheral blood smear
reveal monocytosis cause infection. Head CT scan revealed suggestive of
bilateral retinoblastoma oculi with optic nerve atrophy. Chest X-ray within
normal limits.
The management include maintaining fluid and nutritional intake,
monitoring of vital signs, anoxia sign, bleeding manifestation and any signs of
infections.

LIST OF PROBLEMS
- Retinoblastoma bilateral grade IV
- Anemia

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DIAGNOSIS
- Retinoblastoma oculi bilateral grade IV
- Iron deficiency anemia differntial diagnose anemia of chronic disease

TREATMENT
1. Retinoblastoma oculi bilateral grade IV
Diagnostic:
- Head MSCT scan
- Bone marrow aspiration
Therapeutic:
- Chemotherapy plan after bone marrow aspiration
analysis results
Education:
- The course of illness, further examination for
diagnostic, evaluation and prognosis
- Treatment plan including long term chemotherapy
- Importance to monitor vital signs and any signs of
infection.
- Importance to prevent infection
- Importance of patient’s compliance in taking the
medication
- Evaluation of adverse effect and complication due to
disease and treatment
- Evaluation of the child’s growth and development
Monitoring:
- Vital sign and fluid balance
2. Anemia
Diagnostic:
- Complete blood count
- Erythrocyte index
- Peripheral blood smear

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 - Reticulocyte
- Ferritine

Therapeutic:
- Observation sign of anoxia
- Packed red cell transfusion
Monitoring :
- Vital sign and transfusion reactions
Education:
- Cause of anemia and possible complications

3. Nutritional intake
Evaluation:
- Diet analysis
- Routine anthropometric measurement

Therapeutic:
- Diet as recommended daily allowance
Calorie requirement: ideal body weight x RDA based on height age =
10,2 x 100 = 1020 kcal/day, protein 15% total caloric/day = 38 gram, fat
30% total calori = 34 gram.
- Intake per oral
- Family food 3x/day, snacks 2x/day.
- Monitoring and evaluation: evaluation of weight and
height
Education:
- Inform the parents that their son’s weight for height is below standard
curve. Therefore, daily food intake should be able to meet a balanced
nutritional requirement to ensure optimal growth and development.

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OBSERVATION (Pediatric ward)
Observation day 2-3 (15-16th October 2021)
S Swollen and redness in the left eye. No fever, dyspnea, cough or
vomiting. Micturition and defecation were unremarkable.
O Conscious, GCS 15 (E4M6V5)
Blood pressure: 90/60 mmHg
Pulse: 100 x/minute, regular
Respiratory rate : 24 x/minute, regular
Temperature: 36,7OC
FLACC pain scale: 0
Weight : 10 kg
Physical examination:
- No pale. No lymphadenopathy.
- Breath sound vesicular, no additional sound (no rales or
wheezing).
- Oculi sinistra, there is no proptosis, there is cat eye reflex, there
is leukocoria, there is palpebral edema, there is hyperemic
conjunctiva, there is no blood and pus. Oculi dextra, no
proptosis, no cat eye reflex, no secretions, no blood and pus. No
organomegaly. No spontaneous bleeding manifestation.
Complete Blood Count
Hb 10,2 gr/dL, MCV 72 fl, MCH 23 pg, WBC 9300/µL, Thrombocyte
398.000/uL. Neutrophil 42,3%, Lymphocyte 41%. Natrium 135
mmol/l, kalium 4,3 mmol/l, chloride 109 mmol/l. Ureum 14 mg/dl,
creatinine 0,2 mg/dl, blood glucose 68 mg/dl, SGOT 35 U/l, SGPT
35 U/l, albumin 4,9 gr/dl. Ferritine 8,43 ng/ml.
A - Retinoblastoma oculi bilateral grade IV
- Iron deficiency anemia
P - IVFD dextrose 5% = 14 drops/minute
- Iron elemental 3 mg/kgBW/ days= 30 mg/24 hours/oral

15
 - Regular food 1020 Kcal, protein 38 grams
- Bone marrow aspiration
- Explain to the parents about bone marrow aspiration of patient
explain about its benefit and side effects to her parents.

Observation day 4-5 (17-18th October 2021)

S Swollen and redness in the left eye. No fever, dyspnea, cough or
vomiting. Micturition and defecation were unremarkable.
O Conscious, GCS 15 (E4M6V5)
Blood pressure : 100/60 mmHg
Pulse : 88 x/minute, regular
Respiratory rate : 22 x/minute, regular
Temperature : 36,8OC
FLACC pain scale : 0
Weight : 10 kg
Physical examination:
- No pale. No lymphadenopathy.
- Breath sound vesicular, no additional sound (no rales or
wheezing).
- Oculi sinistra, there is no proptosis, there is cat eye reflex, there
is leukocoria, there is palpebral edema, there is hyperemic
conjunctiva, there is no blood and pus. Oculi dextra, no
proptosis, no cat eye reflex, no secretions, no blood and pus. No
organomegaly. No spontaneous bleeding manifestation.
Bone marrow aspirate (18-10-2021)
- Cellularity: Normocellular
- Erythrpoeitic: Decreased activity, found erythroid precursors
- Leukopoietic: Decreased activity, found myeloid precursor, found
hematopoietic cells suspicious of retinoblastoma cells.
- Thrombopoietic: Sufficient activity, found megakaryocytes

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 - Plasma cells: found
- Mitosis: found
- ME ratio: 3: 1
Conclusion: slight hypoplastic marrow with normal morphology, no
infiltration of hematopoietic cells.
Echocardiography (18-10-2021)
- Situs solitus,
- AV-VA concordance,
- Heart chamber dimension: balance
- Intact IAS, no PFO nor ASD
- Intact IVS, no VSD
- Aorta to the left, no CoA
- PA confluence, no PDA
- Heart’s valves: normal
- Good RV and LV systolic function
- Good myocardium contractility, EF 77,6 %
Conclusion: Good RV and LV systolic function
Heart anatomy within normal limits
Cerebrispinal liquor analysis (18-10-2021) found no malignant cells
A - Retinoblastoma oculi bilateral grade IV
- Iron deficiency anemia
P - IVFD dextrose 5% = 14 drops/minute
- Iron elemental 3 mg/kgBW/ days= 30 mg/24 hours/oral
- Regular food 1020 Kcal, protein 38 grams
- Explain to the parents about definitive diagnosis of patient
- Chemotherapy preparation : Informed consent to start the
chemotherapy, explain about the benefit and side effects to her
parents.
- Chemotherapy vincristine 0,5mg/intravenous, doxorubicin 6,7
mg/intravenous, Cyclophosphamide 400mg/intravenous.

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Observation day 6 (19th October 2021)
S Swollen and redness in the left eye. No fever, dyspnea, cough or
vomiting. Micturition and defecation were unremarkable
O Conscious, GCS 15 (E4M6V5)
Blood pressure : 100/60 mmHg
Pulse : 100 x/minute, regular
Respiratory rate : 24 x/minute, regular
Temperature : 36,7OC
FLACC pain scale : 0
Weight : 10 kg
Physical examination:
- No pale. No lymphadenopathy.
- Breath sound vesicular, no additional sound (no rales or
wheezing).
- Oculi sinistra, there is no proptosis, there is cat eye reflex, there
is leukocoria, there is palpebral edema, there is hyperemic
conjunctiva, there is no blood and pus. Oculi dextra, no
proptosis, no cat eye reflex, no secretions, no blood and pus. No
organomegaly. No spontaneous bleeding manifestation.
A - Retinoblastoma oculi bilateral grade IV
- Iron deficiency anemia
P - IVFD dextrose 5% = 14 drops/minute
- Iron elemental 3 mg/kgBW/ days= 30 mg/24 hours/oral
- Regular food 1020 Kcal, protein 38 grams
- Chemotherapy doxorubicin 6,7 mg/intravenous.

Observation day 7 (20th October 2021)

S Swollen and redness in the left eye. No fever, dyspnea, cough or
vomiting. Micturition and defecation were unremarkable.
O Conscious, GCS 15 (E4M6V5)

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 Blood pressure: 100/60 mmHg
Pulse: 84 x/minute, regular
Respiratory rate : 24 x/minute, regular
Temperature: 36,9OC
FLACC pain scale: 0
Weight : 12,5 kg
Physical examination:
- No pale. No lymphadenopathy.
- Breath sound vesicular, no additional sound (no rales or
wheezing).
- Oculi sinistra, there is no proptosis, there is cat eye reflex, there
is leukocoria, there is palpebral edema, there is hyperemic
conjunctiva, there is no blood and pus. Oculi dextra, no
proptosis, no cat eye reflex, no secretions, no blood and pus. No
organomegaly. No spontaneous bleeding manifestation.
Complete Blood Count
Hb 8,4 gr/dL, MCV 70 fl, MCH 23 pg, WBC 3000/µL, Thrombocyte
258.000/uL. Natrium 135 mmol/l, kalium 4,1 mmol/l, chloride 113
mmol/l. Ureum 11 mg/dl, creatinine 0,21 mg/dl, blood glucose 82
mg/dl, SGOT 51 U/l, SGPT 38 U/l, albumin 3,5 gr/dl.
A - Retinoblastoma oculi bilateral grade IV
- Iron deficiency anemia
P - IVFD dextrose 5% = 14 drops/minute
- Iron elemental 3 mg/kgBW/ days= 30 mg/24 hours/oral
- Regular food 1020 Kcal, protein 38 grams
- Chemotherapy doxorubicin 6,7 mg/intravenous.

PROGNOSIS
Quo ad vitam : dubious
Quo ad sanationam : dubious
Quo ad functionam : dubious

19
 A. Course of illness Prior to observation by candidate

15-9-21 28-9-21 14-9-21

Admission L Hospital Admission A Hospital Admission W Hospital

Edema palpebra and red conjungtiva + Edema palpebra and red conjungtiva + Edema palpebra and red conjungtiva + and cat-
and cat-eye reflex in the left eye and cat-eye reflex in the left eye eye reflex in the left eye
fever – fever – lymphadenopathy -
lymphadenopathy - lymphadenopathy - Hepatomegaly -
Hepatomegaly - Hepatomegaly - Splenomegaly -
Splenomegaly - Splenomegaly - Bleeding manifestation –
Bleeding manifestation -
Bleeding manifestation -
Head Msct Scan : suggestive for WfH : between median and 2SD (z-score)
bilateral retinoblastoma oculi with Hb 10,2g/dl
optic nerve atrophy. Leukocyte 9.200/mm3
Platelets 398.000/mm3
Granulocyte 33,8%
Lymphocyte 46,2%

Diagnosis : Diagnosis :
Diagnosis : Retinoblastoma oculi bilateral
Retinoblastoma bilateral
Retinoblastoma bilateral grade IV
Anemia

Referred to W Hospital Ophthalmologist to W

Hospital and referred to Pediatric ward
pediatric departement
Plan for bone marrow
aspiration,
echocardiography and
analysis liquor
cerebrospinalis

Regular food
Calorie 1020 kcal
Protein 538gr

20
 Observation by candidate

14/9/21 15-16/9/21 17-18/9/21 19/9/21 20/9/21

Edema palpebra and red Edema palpebra and
conjungtiva + and cat-eye red conjungtiva + and
reflex in the left eye cat-eye reflex in the
Pale - left eye
lymphadenopathy - Pale -
Hepatomegaly - lymphadenopathy -
Splenomegaly - Hepatomegaly -
Bleeding manifestation – Splenomegaly -
Bleeding manifestation
– BMA : No signs of
Hb: 10,2 g/dL
WBC 9300/µL
Platelets 398.000/mm3
Ferritine 8,43 ng/ml
Edema palpebra and red Edema palpebra and red Edema palpebra and red
conjungtiva + and cat-eye conjungtiva + and cat-eye conjungtiva + and cat-eye
reflex in the left eye reflex in the left eye reflex in the left eye
Pale - Pale - Pale -
lymphadenopathy - lymphadenopathy - lymphadenopathy -
Hepatomegaly - Hepatomegaly - Hepatomegaly -
Splenomegaly - Splenomegaly - Splenomegaly -
Bleeding manifestation – Bleeding manifestation – Bleeding manifestation –
Hb: 8.4 g/dL
infiltration of WBC 3.000/µL
retinoblastoma cells. Platelets 258.000/mm3
Echocardiography : within
normal limit

Liquor cerebrospinalis :
Do not contain neoplastic
cell

Diagnosis : Diagnosis : Diagnosis : Diagnosis : Diagnosis :

Retinoblastoma oculi Retinoblastoma oculi Retinoblastoma oculi Retinoblastoma oculi Retinoblastoma oculi
bilateral grade IV bilateral grade IV bilateral grade IV bilateral grade IV bilateral grade IV
Iron denficiency anemia Iron denficiency anemia Iron denficiency anemia Iron denficiency anemia Iron denficiency anemia
dd/ anemia of chronic
disease
rombocytopenia

Dextrose 5% Dextrose 5% Dextrose 5% Dextrose 5% Dextrose 5%

Iron elemental 30 mg/24 Iron elemental 30 mg/24 Iron elemental 30 mg/24 Iron elemental 30 mg/24
hours/ oral hours/ oral hours/ oral hours/ oral
BMA - Vincristine 0,5 mg / - Doxorubicin 10 mg / - Doxorubicin 10 mg /
intravenously intravenously (days 1,2 and 3) intravenously (days 1,2
- Doxorubicin 6,7 mg / - and 3)
intravenously (days 1,2 and -
3)
- Cyclophosphamide 400 mg /
intravenously
-
Regular food Regular food Regular food Regular food Regular food
Calorie 1020 kcal Calorie 1020 kcal Calorie 1020 kcal Calorie 1020 kcal Calorie 1020 kcal
Protein 38 gr Protein 38 gr Protein 38 gr Protein 38 gr Protein 38 gr

21
CASE ANALYSIS
PVC, a girl 1-year-6-month-old was admitted to W hospital with chief
complaint palpebra edema and redness in conjungtiva since 1 month with
cats eye reflexes in the left eye that have been noticed since 3 months before
admission prior to hospitalization. Initially around the age of 7 months,
parents noticed their child's eyes like white spots on the left eyeball. There is
decreased in vision in the left eye which has not been noticed since when.
Leukocoria means ‘white pupil’ or ‘cat’s eye pupil’. Leukocoria is an abnormal
pupillary reflex more clearly seen on mydriasis. Leucocoria It is the most
common feature of retinoblastoma and accounts for 60% of cases. Another
manifestation orbital inflammation resembling pre-septal or orbital cellulitis
and decreased of vision can also be a presenting feature. 1
From physical examination there was cats eye reflexes in the left
eye. Therefore, diagnosis retinoblastoma was made. Retinoblastoma is the
most common encountered primary intraocular malignancy of childhood. This
cancer was first described in 1809 by the Scottish surgeon James Wardrop,
as white brain-like intraocular tumors of retinal origin. Retinoblastoma is
composed of retinoblasts (basophilic cells with hyperchromatic nuclei and
scanty cytoplasm). The tumor can be endophytic (in vitreous) and seeding of
tumor cells throughout the eye, or it can be exophytic (in subretinal space), or
it can demonstrate a mixed presentation. It is also the second most common
intraocular malignant tumor. The number of retinoblastoma cases ranges
from 1 in 14000 to 1 in 20000 live births.1,2 Most children are diagnosed
before the age of five years old. In the UK, bilateral cases usually present
within the first year with the average age at diagnosis being 9 months.
Diagnosis of unilateral cases peaks between 24 and 30 months.3
Retinoblastoma is caused by mutational inactivation of both alleles
of the RB1 gene, which maps to chromosome 13q14 and encodes
retinoblastoma protein that acts as a tumor suppressor. This prompted the
proposal that heritable retinoblastomas result from a germline mutation (‘first
hit’, M1) and an acquired somatic mutation (‘second hit’, M2), whereas non-

22
heritable retinoblastomas arise when two somatic mutations are present in
the same transformation suppressor gene in a susceptible cell. Chromosomal
deletions in some patients pointed to a chromosome 13q14 locus. Loss of
heterozygosity of chromosome 13q14 polymorphic markers in 70% of
retinoblastoma tumours implied that the second hit involved the same locus.
Cases with nonheritable retinoblastoma have normal RB1 gene. Some of the
retinoblastoma cases are caused by RB1 gene mutation while others are
caused by somatic amplification of the MYCN gene. Recently, genetic
laboratories have found that retinoblastoma may arise when
the MYCN oncogene is amplified even in the presence of non-
mutated RB1 genes. These cases are relatively rare, occuring in <3% of
unilateral retinoblastoma cases. Only 6% are familial while 94% are sporadic
in newly diagnosed cases of retinoblastoma. All the cases of bilateral
retinoblastoma involve germinal mutations. Almost 15% of unilateral sporadic
retinoblastoma is caused by germinal mutations affecting only one eye while
the 85% are sporadic.4,5
The somatic changes predisposing to unilateral disease occur in
pregnancy or early life, maternal exposures during pregnancy are the most
relevant potential risk factors for unilateral retinoblastoma development.
Passive exposure to environmental tobacco smoke in pregnancy is
additionally of concern as it can induce somatic mutations in infants born to
mothers exposed during pregnancy. Tobacco smoke has a mutagenic effect
which can cause mutations in paternal germ cells before conception; tobacco
metabolites can also pass through the placenta leading to mutations in fetal
cells. A study in Canada reported by Azary in 2016 that there’s the heavier
paternal smoking around the time of conception is a risk factor for childhood
retinoblastoma (Level of evidence IIIA).6 Omidakhsh in 2017 observed that
there’s a positive association between parental smoking status and the
occurrence of retinoblastoma (Level of evidence IIIA).7 In this case, the risk
factor for retinoblastoma is the exposure of cigarette smoking.

23
 Diagnosis of retinoblastoma is usually clear from presenting signs and
clinical examination. The most common sign is leukocoria. When parents
report a strange reflection in the child’s eye, retinoblastoma should be at the
top of the differential diagnosis. The second most common sign is strabismus
(misaligned eyes), when central vision is lost. Advanced disease stage might
present with iris colour change, enlarged cornea and eye due to increased
pressure, or non-infective orbital inflammation. In very late-stage disease, the
eye may bulge from the orbit, a common presentation in settings where
awareness and resources are inadequate.5
Unlike the majority of childhood malignancies, the diagnosis of
retinoblastoma is based upon typical clinical appearances and not biopsy. In
fact, biopsy is contraindicated in view of the substantial risk of promoting
tumour dissemination. Characteristic features include a creamy-white retinal
mass, retinal detachment with associated subretinal fluid, subretinal seeding
or seeds within the vitreous gel. Supporting evidence from ultrasonography
will demonstrate an intraocular mass associated with flecks of calcification in
the majority of cases. Although magnetic resonance imaging (MRI) is more
sensitive than CT scan for picking up extraocular extension, CT scan is more
widely used in developing countries because of the ease of availability and
affordability. Although a CT scan may detect extraocular extension in a
majority of the cases, some cases are likely to be missed out if MRI is not
done routinely. Further staging with bone marrow aspiration, biopsy and
lumbar puncture is undertaken in children with advanced intraocular
disease.8
In 2006, Chantada and colleagues developed The International
Retinoblastoma Staging System. It sub-classifes the disease from stage 0–
IV. Stage 0 is intraocular disease, usually having a good outcome with
treatment, and stage IV is retinoblastoma with metastases, which has a poor
prognosis.9,10,11
For intraocular retinoblastoma, the first classification system was
introduced by Reese and Ellsworth (R-E) in the 1960s to predict the chances

24
of saving the eye following external beam radiotherapy. When intravenous
chemotherapy for intraocular retinoblastoma was introduced in the 1990s, the
R–E classification system was no longer appropriate and a new classification
scheme, the International Intraocular Retinoblastoma Classification (IIRC)
scheme, was developed. The IIRC scheme groups tumours from A-E,
depending on their size, location, and additional features including the
presence of retinoblastoma ‘seeds’ (small colonies of cancerous cells in the
vitreous) and/or retinal detachment.10
Table 2 International Retinoblastoma Staging System (IRSS)10

Shields and colleagues developed a modifed scheme, the Intraocular

Classification of Retinoblastoma (ICRB), which differed from the IIRC mainly
in the defnitions of the advanced groups, D and E. In 2006, the ICRB scheme
was found to successfully predict the outcome of intravenous chemotherapy.
• For eyes in groups A–C: the globe could be salvaged in ≥90% of eyes.
• For eyes in group D: the globe could be salvaged in 47% of eyes.
• Group E eyes underwent primary enucleation and were excluded from
analysis.
This case was diagnosed as retinoblastoma bilateral grade IV based
on history taking, physical examination, and laboratory findings. The patient

25
came with chief complaint is swollen and red conjungtiva with leukocoria in
left eye at 1 years and 3 months of age and decreased of vision. On physical
examination revealed leukocoria in the left eye. Bone marrow puncture
showed no infiltration of non-hematopoietic cells. Liquor cerebrospinal
analysis showed no maligna cells. From imaging (Head MSCT) showed
bilateral retinoblastoma with infiltrating the optic nerve. Patient can also be
classified into very high risk has massive bleeding in the left eye.
The management of a child with retinoblastoma is aimed at achieving
the three sequential goals of life salvage, eye salvage, and optimal vision.
The management involves the identification of the tumor group and stage,
decision-making regarding the appropriate therapeutic measure, and
meticulous follow-up for monitoring the treatment progress and detection of
any recurrence.11,12 There are three possible modalities of treatment13:
1. Surgery
Enucleation is performed when there is no chance of preserving useful
vision in an eye. Primary enucleation is the choice of treatment for
unilateral retinoblastoma, and Cases of intraocular retinoblastoma that
have failed on chemotherapy and conservative treatment. Careful
removal of the eye with a long section of optic nerve to minimize globe
trauma and tumor seeding into the orbit is emphasized. Following
enucleation, an orbital implant is placed to provide socket volume and
allow attachment of the four rectus muscles for motility purposes.
Following enucleation, proper closure of Tenon’s fascia and conjunctiva
is critical to the centration and retention of the implant. A vital corollary to
enucleation is evaluation of globe pathology as Rb with a high risk for
metastasis requires additional chemotherapy. High-risk features include
choroidal invasion, retrolaminar optic nerve invasion, scleral and orbital
invasion, and anterior chamber seeding. Further, it is now possible to
carry out enucleation in most cases of overt orbital disease (stage III)
following two to three cycles of neoadjuvant chemotherapy.14,15.

26
2. Chemotherapy
Current studies completed by The Retinoblastoma Study Group show
the promising use of chemotherapy (carboplatin, vincristine sulfate, and
etoposide phosphate) as a primary mode of treatment in reducing tumor
bulk, followed by various forms of local approaches (radiotherapy
[external beam or plaque], cryotherapy, thermotherapy, and
photocoagulation) that can be used for final tumor control.15
Some reports suggest the addition of cyclosporine in combination with
the chemotherapy regimen of carboplatin, etoposide, and vincristine.
These reports showed that this addition enhances the efficacy of
chemotherapy and eliminates the need for radiation.15
Abramson and colleagues have demonstrated successful salvage of
eyes typically enucleated for advanced disease. Intra-arterial
chemotherapy for advanced retinoblastoma offers another weapon in the
arsenal of therapies for retinoblastoma. However, there are still potential
complications to consider, and, consequently, this procedure should be
performed at tertiary care institutions that specialize in the care of
patients with retinoblastoma. Adverse effects of chemotherapy include
myelosupression, febrile episodes ,neurotoxicities and non specific
gastrointestinal toxicities.16
3. Radiotherapy
Retinoblastoma is a radiosensitive tumor and radiotherapy is used in
various stages of retinoblastoma.15 Historically, external beam radiation
therapy (EBRT) was the preferred vision-preserving mode of therapy with
very good tumor control and vision preservation in patients, based on the
Reese-Ellsworth (RE) staging system, which stratifies patients on the
probability of vision preservation after RT. However, morbidity and
mortality associated with external beam radiation therapy (EBRT) are
significant. EBRT results in cessation of bone growth. Therefore, children
with retinoblastoma who are treated with EBRT have significant midface
hypoplasia. (The younger the child is when EBRT is instituted, the more

27
 dramatic the outcome.) More importantly, EBRT has been shown to
increase the risk of developing second cancers almost 6-fold during the
lifetime of these patients. Today, neoadjuvant chemotherapy
(chemoreduction) has superseded EBRT in order to (hopefully)
circumvent these terrible adverse effects of EBRT. Nevertheless, EBRT
is still indicated in selected circumstances, as follows:
1. For eyes with significant vitreous seeding
2. For children who have progression of disease while undergoing
chemoreduction
3. For tumors extending up to or beyond the cut margin of the optic
nerve of an enucleated eye (The best method of treatment is being
debated in such a case).10,17
This patient got chemotherapy due to optic nerve metastases.
Chemotherapy protocol consist of vincristin, citarabin, doxorubicin,
cyclophosphamid, cisplatin, metothrexate, and etoposide. Then the
consideration for surgery is done after the chemotherapy phase is over The
standard protocol is the vincristine-etoposide-carboplatin (VEC) program
used as a combination triple-drug therapy, typically performed for six cycles.
VEC may yield better results in eyes with advanced cancer when combined
with local therapeutic methods. A study in turkey reported by Varan in 2012
that there’s four-drug chemotherapy (cisplatin, etoposide, cyclophosphamide,
and vincristine) might have satisfactory results for childhood retinoblastoma
(Level of evidence IIIA).18
Chemotherapy literally means the use of chemicals in order to inhibit
malignant cell or to the infectious agents of a disease such as micro-
organism without much affecting the host cells. all the anticancer drugs are
cytotoxic in nature to both cancer as well as normal cells, meaning thereby is
that chemotherapy also destroy cells that divide rapidly under normal
circumstances: digestive tract, cell in bone marrow and hair follicles. There is
a most common side effects observed during the period of chemotherapy
treatment: mucositis (inflammation of the lining of the digestive tract),

28
alopecia (hair loss) and myelosuppression (decreased production of blood
cells, hence also immunosuppression).19
Each cytostatic has its own therapeutic and adverse effects.
Vincristine inhibit mitosis and causing leukopenia, thrombocytopenia,
neurotoxicity, alopecia, hypotension, also stomatitis. Doxorubicin inhibit
mitosis, and causing myelosuppression, mucositis, nausea, vomiting, and
cardiotoxic. Methotrexate has a folic acid antagonist effect and causing
myelosuppression, mucositis, rash, liver dysfunction, neurotoxic and damage
of gastrointestinal mucosa. Cytarabine (Ara-C) has been a drug for
malignancy treatment for more than three decades. The regulation of the
progression of cells from G1 to S phase is a complex event that involves a
series of activations of different cyclins by phosphorylation. The inhibition of
DNA replication by cytarabine in leukemic cells at this G1/S phase check
point can lead to a block in the progression of some malignant cells into S
phase. One of the major concerns of this cytarabine was its toxicity,
myelosuppression or severe neurologic toxicity. Etoposide targets DNA
topoisomerase II activities thus leading to the production of DNA breaks and
eliciting a response that affects several aspects of cell metabolisms. Side
effects of etoposide and teniposide are similar and include bone marrow
suppression, nausea, vomiting, abdominal pain, stomatitis, diarrhea, fatigue,
hypotension, allergic reactions, hair loss and peripheral neuropathy. 20
Cisplatin binds to the N7 reactive center on purine residues and as such can
cause deoxyribonucleic acid (DNA) damage in cancer cells, blocking cell
division and resulting in apoptotic cell death. The 1,2-intrastrand cross-links
of purine bases with cisplatin are the most notable among the changes in
DNA). Cisplatin treatment has been associated with several toxic side effects
including nephrotoxicity, hepatotoxicity and Cardiotoxicity.21
Cyclophosphamide is a type of nitrogen mustard drug which exerts its effects
through the alkylation of DNA. The majority of the antineoplastic effects of
cyclophosphamide are due to the phosphoramide mustard formed from the
metabolism of the drug by liver enzymes like cytochrome P-450. The main

29
areas of concern regarding the adverse side effects of cyclophosphamide are
associated with bladder and gonadal toxicity. Common adverse side effects
reported in several studies and clinical trials involving the use of
cyclophosphamide include hemorrhagic cystitis, amenorrhea,
myelosuppression, alopecia, and spells of nausea and vomiting. 22 In these
patients, after the first stage chemotherapy did not show any side effects
from chemotherapy.
This patient was diagnosed with iron deficiency anemia based on
hemoglobin, erythrocyte index, reticulocyte and ferritin levels. This patient
was treated with elemental iron. In children aged 1–3 years, iron deficiency
anemia is defined as a hemoglobin level of less than 11 g/dL along with a
measure of iron deficiency, such as a serum ferritin level of less than 14
μg/L.23 Pallor is the most important clinical sign, but it is not usually visible
unless hemoglobin falls to 7 g/dL to 8 g/dL. A thorough history may reveal
fatigue, a decreased ability to work, shortness of breath, or worsening
congestive heart failure. Laboratory evaluation will identify anemia. The
hemoglobin indices in iron deficiency will demonstrate a low mean
corpuscular hemoglobin and mean corpuscular hemoglobin volume. 24 A level
of 30 μg/L has been identified as the most sensitive (92%) and specific (98%)
cutoff level to indicate iron deficiency, correlating with the absence of iron
stores in the bone marrow regardless of the presence or absence of
anemia.25 Since the 19th century, the use of oral iron formulations, especially
ferrous sulphate, has been reported to cure iron deficiency anemia and it has
become the current standard treatment for this condition. Oral iron
formulations showed positive effects on the hematological markers of
anemia.26 (Level of evidence IA)
Based on WHO z-score growth chart, the patient was categorized as
good nourished; his weight for height plot was located between median and -
2 SD. Cancer-related malnutrition frequently develops, with prevalence
ranging from 30% to 90% depending on tumor site, stage of disease and
treatment. Major causes are cancer-induced metabolic alterations and/or

30
cancer-induced symptoms (e.g. anorexia, nausea, pain) resulting in
decreased food intake. Malnutrition can be exacerbated by the side-effects of
anticancer drugs such as fatigue, anorexia, altered hedonic input and a wide
range of gastrointestinal symptoms, and/or by physical inactivity resulting
from physical and psychosocial distress, which may lead to further loss of
muscle mass. Malnutrition impairs tolerance to anticancer treatments
including chemotherapy and is associated with decreased response to
treatment, decreased quality of life (QoL) and shorter survival. The duration
of nutritional intervention ranged from 4 to 12 weeks in most studies. A
significant impact on clinical outcome may not be achievable in trials where
the intervention is given for a short time or a much shorter duration than that
of the anticancer treatment. For example, in a treatment plan with 4 monthly
cycles of chemotherapy, an average daily deficit of 400 kcal adds up to a
total cumulative deficit of 48 000 kcal which may result in at least 7 kg BW
loss. If, owing to low compliance and/or a short period of intervention, the
majority of this deficit fails to be addressed, clinical benefits cannot be
attained. In addition, a recent retrospective analysis of esophageal cancer
patients receiving chemo(radio)therapy showed that nutritional intervention
may improve survival when initiated before chemotherapy, suggesting the
importance of early assessment and initiation of nutritional support (Level of
evidence IA).27
In Denver II screening, the patient’s development were appropriate for
his age in all aspects; personal social, language, gross motor, and
adaptive/fine motor skills. Even so, the parents must still be informed on the
child’s condition during treatment, and that it is important for them to always
support the child psycho-socially. This is because children with chronic
disease will suffer from physical and emotional stress. Diagnosis
retinoblastoma itself is a heavy stressor, thus not only potentially causing
psychological problem for the patient but also for the family. Treatments are
associated with significant cognitive decline that could be ameliorated with
appropriate early intervention. Willard, et.al. in their study observed that

31
patients with retinoblastoma have a higher risk of fine motor, gross motor and
language disorders (Level of evidence IIA).28
During the first treatment days at hospital, patient seemed anxious and
quite un-cooperative, especially when interacting with doctors in charge and
paramedics. But, with patience and hospitality from all medics team in
treating him, together with family’s support, by the end of the second week
the patient has started to feel comfortable with hospital condition and started
to befriend with other patients in the wards. The presence of social institution
from society which provides entertainment especially for malignancy patients
is really helpful in pleasing the children. Psychosocial support not only from
the parents but also from family is needed since it will greatly assist in the
treatment. The patient’s psychosocial as well as growth and developmental
aspects need a long term observation/ follow up since retinoblastoma
required long term management using chemotherapy with adverse effect that
can interfere with the patient’s growth and development. Many parents
initially refuse but eventually return for the treatment once the tumor
progresses and symptoms such as pain and proptosis become difficult to
bear. Treatment decisions for retinoblastoma impact the family psycho-
socially and economically. It is to be predicted that pre-existing psychosocial
and financial issues for families at the onset of treatment, are likely to be
exacerbated along the treatment trajectory. The child’s life and welfare, as
well as that of the parents, must be placed ahead of ocular salvage of a
potentially blind eye especially in unilateral retinoblastoma at presentation. 29

Both the IIRC and ICRB classification systems (see Table 3, overleaf)
are now used as the main classification schemes for intraocular
retinoblastoma and serve clinicians and researchers across the world.9,10

32
 Table 3. Classification systems for Intraocular Retinoblastoma

This patient has a relatively poor prognosis since he has criteria for
very high risk due to massive bleeding in the left eye. Delayed presentation is
related to extraocular retinoblastoma which is a risk factor for poor outcome.
Treatment abandonment, clinical stage (extraocular), and high risk
pathological features are also risk factors for death (Level of Evidence II A).
30

33
 CASE ANALYSIS
RISK FACTOR MANAGEMENT
DIAGNOSIS PROGNOSIS

Head MSCT Scan

Leukocoria Staging Histopatology
LCS analysis
Chemotherapy
Decreased vision BMA (cisplatin, etoposide,
Metastasis
cyclophosphamide, and
vincristine)
Parental smoking
Omidakhsh, 2018 Varan, 2012 (III A)
(IIIA), Azary, 2016
Retinoblastoma grade IV
(IIIA)
CBC and ferritine Prognosis
Quo ad vitam dubia
Quo ad sanationam dubia
Qua ad functionam : malam
Iron deficiency Fe elemental
Gao, 2016 (II,A)
anemia Mohd Rosli 2021 (I A)

Clinical Adequate Nutrition

de van der Schueren,
Education Nutritional Optimal of growth
2018 (I A)
and intake State and developmental
Clinical

Antropometri Family
Education
support

34
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2. Stenfelt, S., Blixt, M. K. E., All-Ericsson, C., Hallböök, F., & Boije, H.
(2017). Heterogeneity in retinoblastoma: a tale of molecules and
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3. Yun, J., Li, Y., Xu, C. T., & Pan, B. R. (2011). Epidemiology and Rb1
gene of retinoblastoma. International Journal of Ophthalmology, 4(1),
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4. Singh, L., & Kashyap, S. (2018). Update on pathology of
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5. Dimaras, H., Corson, T. W., Cobrinik, D., White, A., Zhao, J., Munier,
F. L., … Gallie, B. L. (2015). Retinoblastoma. Nature Reviews Disease
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15. Meel R, Radhakrishnan V, Bakhshi S. Current therapy and recent
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38
Appendix 1. Acronym
AMP : Axilla Mamae Pubis
ANC : Absolute neutrophil count
APR : achiles pes reflex
ASD : atrial septal defect
AV : atrioventricular
BCG : Bacillus Calmette Guerin
BPJS : Badan Penyelenggara Jaminan Sosial
BPR : Biceps pes reflex
BMP : Bone marrow puncture
BW : body weight
cm : centimetre
CoA : Coarctation Aorta
CRT : capillary refill time
dL : desilitre
DNA : deoxyribonucleic acid
DPT : Dyphteri Pertusis Tetanus
EF : ejection fraction
EFS : event free survival
fl : femtoliter
FLACC : Face, Leg, Activity, Cry, Consolability
g : gram
GCS : Glasgow Coma Scale
Hb : Hemoglobin
Hct : Hematokrit
IAS : intra-atrial septum
IVFD : intravenous fluid drips
IVS : intraventrikel septum
Kg : kilogram
kcal : kilocalorie
km : kilometer

38
KPR : Knee pes reflex
LV : Left ventricle
MUAC : Mid upper arm circumfference
m2 : meter square
mg : miligram
mm3 : milimeter cubic
MCH : Mean Corpuscular Hemoglobin
MCV : Mean Corpuscular Volume
mmHg : milimeter mercury
OSR : overall survival rate
PA : pulmonary artery
PDA : Persistent ductus arteriosus
PFO : Persistent foramen ovale
pg : pikogram
PMN : Polimorfonuklear
PRC : Packed red cells
RDA : Recommended daily allowance
RV : right ventricle
SD : standard deviation
SGOT : Serum Glutamic Oxaloacetic Transaminase
SGPT : Serum Glutamic Pyruvit Transaminase
TPR : Tricep pes reflex
TT : Tetanus toksoid
VSD : ventricle septal defect
WBC : white blood cell
WHO : world health organization
uL : mikroliter

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Appendix 2. Head Circumference (Nellhaus Curve)
Name : PCV
Age : 1 Year 6 Month
Head circumference: 48 cm (normal: 44,5-49,5 cm)

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Appendix 3. Genetic potential height
Name : PCV
Age : 1 years 6 month
Weight : 10 kg
Height : 80 cm
Weight for height : between median and -2SD (good nourished,
based on WHO z-score curve)
Height for age : between median and -2SD (Normal stature)
Weight for age : between median and -2SD (Normal)
Father’s body height 170 cm, mother’s body height 156 cm, midparental height
156,5 cm
Genetic potential height : 148 -165 cm.

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Appendix 4. MUAC/A

Name : PCV
Age : 1 year 7 Month
MUAC/A : 15/15,9 x 100% = 94,3% (Normal)

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Appendix 5. Denver II

Gabi/ 23-10-2021 PCV / 29-03-2020

P
P
P

P
P
P

F
P
P
P F
P
P
P

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 Appendix 6. WHO weight for age z-score

Appendix 7. WHO Height for age z-score

44
Appendix 8. WHO weight for height z-score

45