CaseCase

Report
Report
NEONATUS ATERM WITH
CONGENITAL RUBELLA SYNDROME

Presented by :
dr.Ririn Esterina
 
Supervised by :
dr. Gatot Irawan, Sp.A(K)
Introduction
Congenital Rubella Syndrome (CRS) is an illness in infants that results from maternal infection with
rubella virus during pregnancy. 
A woman is infected with the rubella virus early in pregnancy  she has 90% chance of passing the
virus in to her fetus
During the first 12 weeks of pregnancy, present an 80% probability of fetal infection with serious
residual defect decreasingly 67% probability during weeks 13 and 14  25% by week 26.
Before introduction of rubella vaccination, epidemics of rubella have resulted in rates of CRS of 0.8–
4.0 per 1,000 live births
The classic triad for congenital rubella syndrome is:
1. Sensorineural deafness (58%)
2. Eye abnormalities especially retinopathy, cataract, glaucoma and microphtalmia (43%)
3.Congenital heart disease especially pulmonary artery stenosis and patent ductus arteriosus (50%)
CASE PRESENTATION
 Name : Baby NY
 Age : 8 days old
 Date of birth : March 15th 2018
 Sex : Female
 MR/Reg number : C684xxx/ 946xxx
 Date of admission : March 15th 2018
 Ward : Level 2 neonatal unit
 ANAMNESIS
(from the mother and from medical records at level 2 neonatal unit of Dr Kariadi
General Hospital on Monday, March 23th 2018, at 15.00 pm (9th day of admission).

A baby was born by caesarean section, cry immediately (AS 8-9-10) No

No fever,
fever, nono shortness
shortness of
of breath.
breath. Vital
Vital
from 29 y.o G3P2A1 mother, 38 weeks pregnancy,birth weigt 3000 sign
sign :: HR
HR 148
148 bpm,
bpm, RR
RR 50
50 bpm
bpm tt ::
grams, heigt of 45 cm, head circumference 47 cm, antenatal care 36.8
36.8 C,
oo
C, SpO2
SpO2 98%.
98%. Physical
Physical examination
examination
every month in midwife and obstetrician, bleeding spot and head
head enlargement
enlargement andand bulging
bulging
hyperemesis during pregnancy in first trimester for a month.There fontenells,
fontenells, dilated
dilated and
and distented
distented scalp
scalp
was no other disease . Other medicine include vitamin and iron vein,
vein, setting
setting sun
sun eyes.
eyes. The
The other
other
supplementation. Mother had received a toxoid tetanus examination
examination was was normal
normal
immunization during pregnancy and before married.

Newborn, 15 March 2018 at Kariadi Hospital

th Admission day 1st

• Head ultrasound showed suspect hydranencephaly

• Hearing screening was normal
Babygram
Babygram X-ray CTR 50%,
X-ray CTR 50%, No
No abnormality
abnormality • Consult to neurosurgery  immediately VP Shunt and must
in
in abdomen,
abdomen, neonatal
neonatal pneumonia
pneumonia be transferred to NICU afterthat
 • infusion D10% 8 ml/h (GIR 4.7
mg/kgbw/min) added 3% NaCl (2 meq)
and KCl otsu (2 meq)
• amino acid infusion 2 g/kgbw/day,
lipids infusion 2 g/kgbw/day
• Ampicilin injection was replaced by
• Baby has been operated VP Shunt. She was somnolent cefotaxime injection 150 mg/12 hours iv
on sedation. She was connected to the ventilator by an (50 mg/kgbw) at 6th day
endotracheal tube. Physical examination revealed that • gentamicin injection 12 mg/24 hours iv
baby was tachypnea, grunting, nasal flaring, and (4 mg/kgbw)
retractions. • calcium gluconas injection 1.4 ml/12
hours iv
• paracetamol injection 30 mg/6 hours iv.

Admission day 2nd until 6th ( March 17 th

until 22th 2018 ) at Neonatal Intensive Care Unit

The babygram x-ray revealed endotracheal tube

attached with the distal end at the level of the 3-4
cervical vertebrae, suspect of neonatal pneumonia
with reduced infiltrate, normal abdominal structure.
 Laboratory revealed:
blood glucose level 71 mg/dL, urea 45
• The baby’s general condition was improved
mg/dl, creatinine 0.23 mg/dl, calcium 2.87
• The baby look active and shows a compos mentis
mmol/L, sodium 145 mmol/L, potassium
consciousness
4.8 mmol/L, chloride 106 mmol/L,
• Heart pulse was 152 tpm, the respiratory rate 46 tpm,
toxoplasma IgG 0 IU/mL, toxoplasma IgM
the axillary temperature is 36,8°C, the contain and
0.04 IU/mL, Rubella IgG 36 IU/mL,
tension of vein is adequate, the saturation of peripheral
Rubella IgM 36 IU/mL, CMV IgG 53
oxygen 98%. Amino acid infusion was stopped.
IU/mL, CMV IgM 0.19 IU/mL.

Admission day 7th ( March 23th until 25 th

2018 ) at Level 2 Neonatal Unit

D10% 5 ml/h added 3% NaCl (2 meq) and KCl otsu (2 meq),

cefotaxime injection 150 mg/12 hours iv (50 mg/kgbw),
fusidic acid ointment every 8 hours for scar post operation
at baby’s head.
 HISTORY
Diagnosis at level 2
Neonatal Unit
 Hydrocephalus suspect hydranencephaly
 Aterm neonate (38 weeks)
 Normal birth weight (3000 grams)
 Appropriate for gestational ages
 High risk baby
Management
 IV D10% 8ml/h (Gluccose infusion rate 4.4
mg/kgbw/min) with NaCl 3% (2 meq) & KCl
(2 meq)
 Amino acid infusion 2g/kg bw/day
 Lipid infusion 1g/kgbw/day
 IV Ampicillin 150 mg/12 hours
 IV gentamycin 12 mg/24 hours
 Breastmilk 2 ml every 3 hours increased
step by step
 Monitoring at NICU after
Ventriculoperitoneal Shunt Operation
 Past History

• none

Family History

• No family history of prematurity or low birth weight and congenital disease

• Mother had history of spontaneus abortion at 8 weeks pregnancy (2 nd
pregnancy)

Pedigree
Perinatal history

• Baby was a third child, mother was 29 y.o when she got pregnant
• Mother had history of hyperemesis and bleeding spot for month at
first trimester
• Baby born by caesarean section in operation room Kariadi Hospital
and cry immediately with 3000 grams birth weight, heigt of 45 cm,
head circumference 47 cm, AS 8-9-10  The baby was admitted to
level 2 neonatal care unit at Kariadi Hospital
• Mother had history of spontaneous abortion at 2nd pregnancy

Sosioeconomic status

• Father is as a private worker. Mother is a housewife.

• Monthly income was approximately Rp. 3.000.000 , medical
insurance from national health insurance.
• Impression:middle socio-economic status.
Perinatal history of
sibling and patient
IMMUNIZATION HISTORY

The baby had not been immunized since birth

HISTORY OF NUTRITION

• Baby received partial enteral nutrition through orogastric tube since birth
• On the firstday of care, baby received 2-5ml/3 h of breastmilk, inf. D10%
144/6 ml/hour with 2 meq sodium and 2 meq potassium
• Mother was encouraged to express and store breastmilk. Birth weight 3000
grams, body weight on secondy day is 2960 grams.
 
HISTORY OF CHILD’S BASIC NEED

 Caring
initial management of breastfeeding was not performed expressed, breastmilk was given
on the first day through gastric tube
 Loving
The child was planned and desired by both parents. Bonding is not adequate because the
baby has been admitted in level 2 neonatal unit.
 Stimulating
Unstimulated.
GROWTH AND DEVELOPMENT HISTORY

 Appropriate for gestational age according to Lubchenko

curve 
Normo birth weight baby 3000 grams (P50), birth length 45 cm
(P10-25), head circumference 47 cm (>P90). Progression
according to a 38-week pregnancy based on New Ballard score
(score 38 ~ 38 weeks). Infant development shows reaction
with balanced movements, weeping when touched, not yet
showing eye contact, primitive reflexes.

 Impression: Intrauterine growth is appropriate for

gestational age. Progress according to aterm baby 38
weeks' gestation.
 Lubchenco’s curve
Lubchenco’s curve

14
Physical Examination
a 8th day girl, weight 2840 grams (birth weight 3000 grams) and height 45 cm
General appearance
Baby was lying in the incubator, supine position, the four extremities slightly flexed. The baby
was opening the eyes spontaneously, looks lethargic, no cyanosis, not pale, no yellow looking, no
retraction, umbilical catether venous cord infusion and gastric tube

 HR: 148 bpm

Vital Sign
 RR: 40 bpm
 pulse: fast, regular
 t: 36.9°C
 SpO2 97-98%
 BP 70/50 mmHg (P50th)

• Head circumference:45cm macrocephaly, VP shunt (+)

• Anterior fontanel bulging (+), dilated scalp sign (+)

• Sun set eyes (+), No anemic conjunctiva, no nasal flare, no

nasal discharge, and no perioral cyanosis,
• ENT were normal, no lymph node enlargement
 The heart sound was normal, no murmur, no gallop, no thrill

There were no chest indrawing,, vesicular sound were

normal on both lungs, no rales, crackles, and decreasing

No organomegaly. Normal of lymph nodes, genitalia, and joints

All extremities were not cyanotic with normal capillary refills, no clubbing
fingers

There were normal physiologic reflexes, normal tone, symmetric

movement, and normal muscle strength, no clonuses and pathologic
reflexes
 Primitive reflexes

 Moro reflex : symmetric

 Rooting reflex : absent
 Sucking reflex : present
 Palmar grasp : symmetric
 Plantar grasp : symmetric
 Tonic neck : present
 Babinski : present

17
 LABORATORY AND IMAGING
EXAMINATION
Clinical Reference Unit 15/3 17/3 26/3
chemistry value
  Reference Unit 15/3/18 17/3/18
Glucose 80-160 mg/dl 91 85 71
value
Ureum 15-39 mg/dL 11 9 45
Hemoglobin 13.6- 19.6 gr/dL 16.3 15.9
Creatinin 0,60-1,30 mg/dL 0.5 0.1 0.23
Hematocrit 44 – 62 % 47.7 45.6
Calcium 2,12-2,52 mmol/ 2,4 2.06 2.87
Erythrocyte 3.9- 5.9 10^6/uL 4.17 4.12
L
MCH 24.0- 34.0 Pg 39.1 38.6
Sodium 136-145 mmol/ 139 139 145
MCV 83 – 110 fL 114.4 110.7
L
MCHC 29.0- 36.0 g/dL 34.2 34.9
Potassium 3,5-5,1 mmol/ 3.4 5.9 4.8
Leukocyte 9 – 30 10^3/uL 8.4 6.5
L
Platelets 150 – 400 10^3/uL 196 150
Chloride 98-107 mmol/ 101 104 106
RDW 11.6- 14.8 % 15.7 14.7
L
MPV 4.00 - 11.00 Fl 9.8 10.4
CRP 0 – 0.30 mg/L 0.07 - -
Impression: leukopenia, hypocalcemia
quantitatif
Differential counting of leukocyte and peripheral blood smear

  Reference value Unit 17/3/18

Eosinophil 2–4 % 0
Basophil 0–4 % 0
Rod 2–5 % 0
Segment 45 – 75 % 57
Lymphocyte 20 – 40 % 26
Monocyte 3 – 12 % 14
Others Myelocyte 1%, Metamyelocyte 1%, AMC 1%
Pheripheral blood smear
Impression: netrofil hypergranulation
Mild anisocytosis (macrocytic, normocytic, microcytic),
Erythrocyte mild poikilocytosis (ovalocyte, pear shape cell,
eliptocyte), polychromatic +
Decrease estimation number of platelets, normal
Platelets
shape domination, giant shape (+)
Normal estimation number, lymphocyte atypical,
Leukocyte
Clinical chemistry Reference value unit 26/3/18
Anti Toxoplasma IgG Negative:<4.0; negative IU/mL 0 Immunoserology
equivocal: 4-8; positive: >8.0
Anti Toxoplasma IgM Negative:<0.55 negative IU/mL 0.04
equivocal: 0.55-0.64; positive:
>0.65
Anti Rubella IgG Negative:<13; positive IU/mL 36
equivocal: 13-15; positive: >15
Impression:
Anti Rubella IgM Negative:=<0.90; positive IU/mL 36
equivocal: 0.91-0.99; positive: = Anti Rubella IgG and IgM,
>1.00 Anti CMV IgG was increased
Anti CMV IgG Negative:=<4; positive IU/mL 53
equivocal: >=4 to <6; positive: =
>= 6
Anti CMV IgM Negative:=<0.70; negative IU/mL 0.19
equivocal: 0.70 < = I < 0.90;
positive: > = 0.90
 Secretion of cerebrospinal fluid

 
Unit Reference 17/3/18
value
Phisis      
Colour   yellow
Turbidity cloudy
Protein gr/dl 1.5-4.5 137.9
Glucose mg/dl 50-80 45
Leucocytes      
MN /mmk   0
PMN /mmk 2-7 2
Erytrocytes     0
Impression: protein level was increased in CSF
CSF Culture on 17 March 2018  no growth of germs

Blood Culture on 17 March 2018  no growth of germs

 Babygram x-ray result Head Ultrasonography

16 March 2018 finding and measurement :

- Prominent cystic lesions in the right left cerebral
hemisphere
- The mantle cortical structure or normal ventricular structure
are not clearly seen
- Still visible part of the falx cerebri structure
Diagnostic Impression:
Suspect hydranencephaly

B-scan Ultrasonography

27 March 2018

Impression: Findings and measurement :

Posterior segment intraocular calcification
23
CTR 50%, no abnormality in
abdomen, neonatal pneumonia
 PROGRESS NOTE
Day 8-9th in level 2 neonate unit

S: -
O: HR 150 bpm, RR 46, t: 36,8°C, SpO2 100%.
blood glucose level 71 mg/dL, urea 45 mg/dl, creatinine 0.23 mg/dl, calcium 2.87
mmol/L, sodium 145 mmol/L, potassium 4.8 mmol/L, chloride 106 mmol/L, toxoplasma
IgG 0 IU/mL, toxoplasma IgM 0.04 IU/mL, Rubella IgG 36 IU/mL, Rubella IgM 36 IU/mL,
CMV IgG 53 IU/mL, CMV IgM 0.19 IU/mL.
A:
• hydrocephalus post ventriculoperitoneal (VP) shunt placement
• aterm neonate (38 weeks)
• normal birth weight (3000 grams)
• appropriate for gestational age
• congenital rubella syndrome
• high risk baby.
P:
infusion D10% 5 ml/h added 3% NaCl (2 meq) and KCl otsu (2 meq), cefotaxime injection
150 mg/12 hours iv (50 mg/kgbw), fusidic acid ointment every 8 hours for scar post
operation at baby’s head.
Final Diagnosis & Prognosis

• Hydrocephalus post ventriculoperitoneal Quo ad vitam : ad bonam

(VP) shunt placement Quo ad sanam : ad bonam
• Aterm neonate (38 weeks) Quo ad fungsionam : dubia ad sanam
• Normal birth weight (3000 grams)
• Appropriate for gestational age
• Congenital rubella syndrome
• High risk baby.

26
Discussion
Congenital Rubella Syndrome
 Crosses placenta when mother has acute infection

 The earlier the fetus is infected  more serious disease

 May result in serious congenital abnormalities

• Intrauterine growth retardation

• Hepatosplenomegaly
• Cataracts
• Metal retardation
• Sensorineural hearing loss
• Patent ductus arteriosus
• Trombocytopenic purpura
 • At birth, the serum of a newborn affected with
congenital rubella will contain passively acquired
maternal IgG and fetal IgM  Fetal IgM production is
initiated sometime after the 16th week and probably
prior to the 20th to 24th week of gestation.
• After birth, infants continue to synthesize antibody;
the production of IgM continues through early infancy
up to 7 months of age and there is generally several
months delay in the production of IgG

In this case:
 mother had a bad history of obstetric (miscarriage
at 2nd pregnancy and bleeding spot at early 3rd
pregnancy), but mother never had examined
TORCH screening.
 Mother had no symptom of rubella during
pregnancy.
 The baby’s laboratory revealed that anti rubella
IgG and IgM was positive, cmv IgG positive
 Rubella IgM testing positive showed that
susceptible pregnant women who might have been
exposed to rubella to rule out acute or recent
infection.
Clinical Manifestation
In this case, head
enlargement (p>90),
bulging,dilated scalp sign

In this case, sun In this case, hearing

set eyes, screening was passed
Posterior
segment
intraocular
calcification
 In this
case,
Rubella IgG
and IgM
was
positive
Case definition for case classification CRS

1. Suspected
An infant with at least one of the
2. Probable
symptoms clinically consistent with CRS
3. Confirmed listed above and laboratory evidence of
4. Infection only congenital rubella infection demonstrated
by: IgM or PCR
In this case, we had diagnostic for this
baby with confirmed CRS
 Hydrocephalus is one of the most common
congenital anomalies affecting the
nervous system, occurring with an
incidence of 0.3 to 2.5 per 1.000 live
births.

Head Ultrasonography on 16 March 2018 finding and measurement :

- Prominent cystic lesions in the right left cerebral hemisphere
- The mantle cortical structure or normal ventricular structure are not clearly seen
- Still visible part of the falx cerebri structure
Diagnostic Impression:
Suspect hydranencephaly
 Congenital hydrocephalus means the condition
is present at birth, caused by a complex
interaction of genetic and environmental factors
during fetal development  often diagnosed
before birth through routine ultrasound.

Acquired hydrocephalus develops after birth as a

result of neurological conditions such as head trauma,
brain tumor, cyst, intraventricular hemorrhage or
infection of the central nervous system.

The standard treatment for hydrocephalus is a VP shunt. In a shunt

procedure, a catheter (a thin, flexible tube) is placed in the brain to drain
extra fluid down to the abdominal cavity, chambers of the heart, or a
space around the lungs Then the fluid is absorbed by the
bloodstream.

Infections are a significant complication of shunt surgery  Treatment usually

means a hospital stay for surgery to remove the infected shunt, IV antibiotics (given
into a vein)
 Cause congenital hydrocephalus
Ventriculomegaly  a condition where the ventricles of the brain become larger than
normal due to a congenital disability  Large ventricles cause irregularity in the flow of
CSF leading to hydrocephalus.

Aqueductal stenosis   The passageway that connects parts of ventricles, narrows

down, thus preventing the free-flow of cerebrospinal fluid

Arachnoid cysts  self-contained but are still connected to the ventricles thus affecting
the pressure of CSF on the brain

Spina bifida   a birth defect where the bones of the spine do not fuse properly It
causes the spinal cord and the rest of the nervous system to form abnormally

Maternal infections during pregnancy  Severe infections to the mother during

pregnancy increase the risk of a baby being born with hydrocephalus. Diseases such as
rubella and mumps affecting the mother have been linked to the birth of infants with
congenital hydrocephalus 
Treatment
There is no specific antiviral therapy. Patients with congenital rubella require supportive care not only in the
neonatal period but also throughout life for such permanent impairments as deafness and heart defect 

In this case, baby received ampicillin and gentamycin for 5 days. Ampicilin was stopped and replaced with cefotaxime
until for 14 days. Gentamycin was continued together with cefotaxime. Antibiotic was stopped after improvement in
general condition and laboratory result. Based on clinical manifestation, no respiratory distress and good feeding tolerance.
We have used antibiotic for prophylaxis on hydrocephalus shut
 Prevention
• The vaccine is being introduced in place of the only-measles vaccine
to children aged 9 months  a second-dose of measles vaccine is
recommended for children at any time between 15 and 18 months of
age

• MMR vaccines should not be administered to women known to

be pregnant or attempting to become pregnant  the theoretical
risk to the fetus when the mother receives a live virus vaccine,
women should be counseled to avoid becoming pregnant for 28
days after receipt of MMR vaccine
Thank You