Congenital Rubella Syndrome: A Case Report

Ayodhia Pitaloka Pasaribu

Munira Ulfa

, Syahril Pasaribu

, Irma Sari Nasution

1,2

Departement of Child Health, Faculty of Medicine, Universitas Sumatera Utara, Adam Malik Hospital,

Jl. Bunga Lau No.17, Medan, Indonesia

Faculty of Medicine, Universitas Sumatera Utara, Jl. Dr. T. Mansur No.5, Medan, Sumatera Utara, Indonesia

Keywords: Congenital Rubella Syndrome, virus, infection

Abstract: Congenital rubella syndrome (CRS) is the effect of rubella virus infection during 8-12 weeks of gestation.

In Indonesia, the incidence of CRS annually is 0.2/1000 live newborn. The most common defects of CRS

are hearing defects (deafness), eye defects (cataract, congenital glaucoma), and cardiac defects (PDA). We

reported a case of congenital rubella syndrome in a 5-month-old boy. He was first diagnosed with

congenital rubella syndrome 4 months ago (confirmed by positive of anti-rubella IgM with congenital

cataract and congenital heart disease-PDA and ASD). He was admitted to Adam Malik Hospital due to

congestive heart failure Ross III, bronchopneumonia, marasmus, and anemia. We treated the patient with

bed rest, oxygen supplementation, broad-spectrum empiric antibiotics, antipyretic, diuretic, angiotensin

converting enzyme inhibitor, and nutritional management according to pediatric nutrition.

1 INTRODUCTION

Congenital rubella syndrome (CRS) is an illness in

infants that results from maternal infection with

rubella virus during pregnancy. When rubella

infection occurs during pregnancy, serious

consequences such as miscarriages, stillbirths, and a

constellation of severe birth defects in infants may

happen (Lanzieri, 2012).

In Indonesia, the

incidence of CRS annually is 0.2/1000 live

newborn. Surveillance data of 2015 showed off

979, newly diagnosed CRS case from 4.89 million

live newborns

(Kemenkes RI, 2017).

The natural history of congenital rubella

initiated by maternal viremia presents for several

days before the onset of rash. Maternal viremia may

be followed by placental infection and subsequent

fetal viremia, leading to disseminated infection

involving many fetal organs. Timing is crucial, as

the highly susceptible period of intrauterine rubella

is during the first 8 to 12 weeks (Gershon, 2004).

2 CASE REPORT

A 5-month-old male came to the emergency unit at

Adam Malik Hospital with a chief complaint of

shortness of breath. He had experienced the

symptom since 2 weeks prior to admission,

described as fast breathing and chest indrawing. It

got worsened within two days prior to admission and

aggravated by feeding. One week prior to admission,

the patient was noted to have developed an

intermittent fever. Four weeks prior to admission,

the patient was noted to have an intermittent

productive cough. At that period, he was also noted

to be pale, without a history of bluish discoloration

of skin and lips.

At 2 months old, he had the right eye cataract

surgery. He was then noted to have lost weight

gradually. The highest weight noted was 4000 g at 3

months old. Five months prior to admission, the

patient noted to have interrupted feeding. He was

noted to have fast breathing during feeding, hence

the feeding activity was often interrupted.

On physical examination, his initial body weight

was 3000 gr, body length was 56 cm, and mid-upper

arm circumference was 9 cm. Nutrition status was

marasmus (based on the WHO 2006 Growth chart

weight for length z score, boys age 0-5 years). The

patient was alert but looked ill. The blood pressure

was 75/40 mmHg, heart rate of 160 bpm, respiratory

rate of 62 bpm, and body temperature of 37.5 C

(axilla), peripheral oxygen saturation was 90% at

room air and 96% at oxygen 1 lpm via nasal

cannula.

Pasaribu, A., Ulfa, M., Pasaribu, S. and Nasution, I.

Congenital Rubella Syndrome: A Case Report.

DOI: 10.5220/0009861501110114

In Proceedings of the 2nd International Conference on Tropical Medicine and Infectious Disease (ICTROMI 2019), pages 111-114

ISBN: 978-989-758-469-5

111

Head circumference was 36.5 cm

(microcephaly), with open flat anterior fontanel, no

old man face, and palpebral conjunctiva was pale

and sclera was anicteric. There was no palpebral

edema. The right pupil was 3 mm and reactive to

light while the left pupil cannot be assessed since it

was covered with whitish discoloration. Abnormal

repetitive, uncontrolled bilateral eye movement from

side to side was noted. There was no nasal flaring

and cyanosis at lips and tongue. No cervical

lymphadenopathy was noted.

He had symmetrical chest expansion, with

intercostal and epigastric retractions, the prominent

appearance of the intercostal area due to thinning of

subcutaneous fat. The respiratory rate was 62 bpm,

regular, coarse rales on both lung fields, with no

wheezing, equal fremitus. Ictus cordis was not

visible and but palpable at IV-V intercostal space on

the left midclavicular line, the thrill was felt. The

heart rate was 160 beats per minute, regular, with

continuous murmur grade 4/6 on the left

infraclavicular area, radiating to the back and the

thrill was palpable.

Abdomen looked globular, soft on palpation,

with normal bowel sound with no palpable liver,

good skin turgor. There was no edema on

extremities, but with muscle hypotrophy and

thinning of subcutaneous fat. The blood pressure

was 70/40 mmHg, peripheral pulses were full and

regular, capillary refill time less than 2 seconds.

There was no abnormality on genitalia and scrotum

examination, both testicles are palpable.

Complete blood count revealed anemia,

leukocytosis, electrolyte imbalance, low serum iron,

normal liver, and kidney function. Blood culture was

drawn, revealed no growth. Chest X-ray revealed

bronchopneumonia and congenital heart disease with

cardiomegaly. Echocardiography revealed secundum

atrial septal defect (ASD) Ø 3.7 mm and PDA Ø 3.9

mm.

The patient was managed with bed rest, oxygen

supplementation, empiric broad-spectrum

antibiotics, antipyretic, diuretic, angiotensin

converting enzyme inhibitor, and nutritional

management according to pediatric nutrition care for

marasmus child.

The admitting diagnosis was congestive heart

failure Ross III due to cyanotic congenital heart

disease (patent ductus arteriosus (PDA) and ASD),

bronchopneumonia, marasmus, anemia, and

congenital rubella syndrome.

Oxygen supplementation 1 lpm was

administered. Antibiotic ampicillin 75 mg/6

hours/IV (25 mg/kg/dose) and gentamycin 15 mg/24

hours/IV (5 mg/kg/day) were started. Paracetamol

40 mg/6 hours (10 mg/kg/dose) per oral was given if

temperature >38°C. Anti-cardiac failure medications

namely furosemide 1x3 mg (1 mg/kgBW/day) and

captopril 2x3.125 mg (1 mg/kgBW/dose) were

continued.

The total caloric requirement was 80-100

kcal/kg/day during the initial phase with a total

calorie of 210 kcal was received, through F75 with

the mineral mix (75 kcal/100 ml) via nasogastric

tube (NGT). The F75 was given in an amount of 35

ml every 3 hours and multivitamins. During the

transitional phase, the total caloric requirement was

100-150 kcal/kg/day, given through oral nutrition

supplement (ONS) with high-calorie infant formula

(100 kcal/100 ml) via NGT-oral. The total volume of

feeding was increased gradually.

Ophthalmology assessment revealed right eye

aphasia and left eye congenital cataract. Subsequent

cataract surgery was suggested when the clinical

condition had been improved. We also planned for a

hearing function test. Unfortunately, the device for

hearing function test was out of order. On growth

and development assessment, we found global

developmental delay.

3 DISCUSSION

In 1941, Norman Gregg, Australian ophthalmologist

has first described a syndrome comprising of

cataracts and congenital heart disease with or

without mental retardation and microcephaly that he

associated with rubella infection in the mothers

during early pregnancy (Mason, 2016). CRS group

classification of clinical signs listed below :

Cataract(s), congenital glaucoma, pigmentary

retinopathy, congenital heart disease (most

commonly peripheral pulmonary artery

stenosis, patent ductus arteriosus or ventricular

septal defects), hearing impairment.

Purpura, splenomegaly, microcephaly,

developmental delay, meningoencephalitis,

radiolucent bone disease, jaundice that begins

within the first 24 hours after birth.

Using these clinical signs, the final

classifications of CRS are as follows: (A) Suspect

CRS: infant less than 12 months of age with at least

one sign from group A; (B) Clinical CRS: infant less

than 12 months of age with at least two signs from

group A; or At least one sign from group A and one

sign from group B without any laboratory

confirmation; (C)

Confirmed CRS: suspect CRS

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with laboratory confirmation shows infant less

than 6 months of age with positive Rubella IgM; (D)

Congenital rubella infection (CRI): infant less than

12 months of age without any clinical signs of CRS,

but who meets the laboratory criteria for CRS

(WHO, 2018 and Ministry of Health Republic of

Indonesia, 2018).

This patient was diagnosed as confirmed CRS,

because he had met the criteria of suspect CRS

(congenital cataract, congenital heart disease-PDA,

and ASD) and laboratory confirmation of positive

anti-rubella IgM when he was 4 months old. Other

important feature like hearing impairment may be

the only manifestation of congenital rubella. It is

probably caused by maldevelopment and

degenerative changes in the cochlea and organ of

Corti. Hearing impairment severity is variable, and

may be overlooked unless detected by an

audiometric examination (Gerson, 2004).

PDA occurs if the ductus arteriosus remains

patent leading to the left-to-right shunt of the heart.

The magnitude of the left-to-right shunt is

determined by the resistance offered by the ductus

(diameter, length, and tortuosity) (Mason, 2016).

Patent ductus arteriosus is also associated with

maternal rubella infection during early pregnancy

(Park, 2014).

The patient has continuous murmur grade 5/6 on

the left infraclavicular area, suggestive of murmur

caused by PDA and had developed retardation of

physical growth due to the shunts. Transcatheter

PDA closure was performed to treat a PDA with a

left-to-right shunt that results in any of the

following: congestive heart failure, failure to thrive,

increased pulmonary blood flow, or an enlarged left

atrium or left ventricle, provided the anatomy and

patient size are suitable (AAP, 2011)

The treatment of CHF consists of elimination of

the underlying causes, treatment of the precipitating

or contributing causes, and control of heart failure

state (Mason, 2016). If the cause of CHF is a

congenital heart defect amenable to surgery, medical

treatment is indicated to prepare the patient for

surgery (Masarone, 2017). The heart failure state is

controlled by the use of multiple drugs, including

inotropic agents, diuretics and afterload-reducing

agent, along with general supportive measures (Park,

2014).

The benefits of a diuretic include the reduction

of systemic, pulmonary, and venous congestion.

Angiotensin-converting enzyme inhibitors decrease

the afterload and possibly reverses the

pathophysiological myocardial remodeling.

Inotropes should be reserved for severe reduction of

cardiac output (Masarone, 2017). In this case, the

patient receives a diuretic (furosemide) and an ACE

inhibitor (captopril). However, the inotropes were

not given since the hemodynamic was stable and

echocardiography revealed good contractility. He

was planned for PDA and ASD transcatheter closure

when he reached a minimum weight of 6 kg.

Respiratory complications are frequent in

children presenting with congenital heart disease.

This group of patients is at a greater risk for viral or

bacterial pulmonary infections. Pongiglione (2016)

stated that in Italy the incidence of respiratory

disease was 63.1%. A total of 26.2% underwent

hospitalization with a median length of hospital stay

was 7 days, and the median age of the patients

hospitalized was <1 year old. The most frequent

respiratory diseases associated with hospitalizations

were lower respiratory tract infections.

This patient experience gradual weight loss when

he was 3 months old, without any data regarding the

length. On admission, he was presented with severe

underweight, severe stunted, diagnosed as

marasmus. The etiology of malnutrition in the

children with CHD can be grouped into the

following three categories: (1) inadequate intake, (2)

inefficient absorption and utilization, and (3)

increased energy needs (Rodica, 2013). Viera (2007)

stated that the caloric intake in hospitalized-children

due to congenital heart disease was 50% below the

recommended daily allowance. Fatigue upon feeding

may explain the decreased intake (Vieira, 2007).

4 CONCLUSION

We have treated a 5-month-old boy who came to

Adam Malik Hospital, Medan, Indonesia due to

shortness of breath. He was diagnosed with

congestive heart failure Ross III due to cyanotic

congenital heart disease (PDA and ASD),

bronchopneumonia, marasmus, anemia, and

congenital rubella syndrome. The patient was treated

with adequate therapy. Congenital rubella syndrome

was confirmed when he was 4 months old, both

laboratory and clinically.

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