Clinical proﬁle of congenital rubella syndrome in Yogyakarta,
Elisabeth S. Herini,
and Yati Soenarto
Department of Child Health, Faculty of Medicine and
Pediatric Surgery Division, Department of Surgery, Faculty of
Medicine, Universitas Gadjah Mada/Dr Sardjito Hospital,
Department of Child Health, Faculty of Medicine, Universitas
Gadjah Mada/UGM Academic Hospital, Yogyakarta and
Indonesia World Health Organization, Jakarta, Indonesia
Abstract Background: Congenital rubella syndrome (CRS) has many severe neurological manifestations and other systemic
consequences. Although various studies have been done in Indonesia, there are no conclusive results on CRS inci-
dence. The aim of this study was therefore to investigate the incidence, clinical manifestations and outcomes of
CRS in Yogyakarta, Indonesia.
Methods: A descriptive study involving a review of congenital anomalies associated with CRS was carried out at
Dr Sardjito Hospital, Yogyakarta, Indonesia, from July 2008 to June 2013. CRS was categorized according to the
World Health Organization (WHO) classiﬁcation. This study involved children aged <1 year old, and was
conducted at the outpatient clinic, pediatric and neonatology wards.
Results: A total of 201 children met the criteria for suspected CRS during the 5 year study. Of those patients, 6%
were classiﬁed as having laboratory-conﬁrmed CRS, 21.4% as having clinically compatible CRS, and 72.6% as
having discarded CRS (i.e. a suspected case that does not meet the criteria for CRS). The estimated incidence of
laboratory-conﬁrmed CRS and laboratory-conﬁrmed and clinically compatible CRS in Yogyakarta, Indonesia during
the study period was 0.05:1,000 and 0.25:1,000 live births, respectively. Of the laboratory-conﬁrmed CRS patients,
83.3% of children had congenital heart disease (CHD), 75% had hearing impairment, 66.7% had congenital cataract
and 50% had microcephaly. Furthermore, none of the mothers was vaccinated against rubella.
Conclusions: The incidence of CRS in infants in Yogyakarta Indonesia is considered high, with most clinical mani-
festations being CHD, hearing impairment and congenital cataract. This emphasizes the necessity for epidemiologi-
cal study of CRS in other hospitals and the importance of establishing a national rubella vaccination program in
Key words clinical proﬁle, congenital rubella syndrome, Indonesia, retrospective study, vaccine program.
Congenital rubella syndrome (CRS) is associated with severe
birth defects. The acquisition of rubella infection during the
early fetal period constitutes a risk factor for CRS. In a
previous study, a pregnant woman infected with the rubella
virus in the ﬁrst trimester of pregnancy had a 90% chance of
transmitting it to the fetus. Fetal transmission of the rubella
virus may lead to fetal death or CRS, which is characterized
by neurological, ophthalmic, auditory, cardiac, and craniofa-
cial defects, as well as systemic complications.
Of the vari-
ous neurological defects, deafness is the most common
Congenital rubella syndrome is frequently found in devel-
oping countries, affecting approximately 110 000 infants annu-
ally. In the Western Paciﬁc region, the number of cases of
rubella infection increased 12-fold from 5,475 in 2000 to
73 077 in 2009, leading to a concern that the incidence of
CRS may have also increased in this region.
According to a
survey of the member countries of the World Health Organi-
zation (WHO), the number of countries that have incorporated
rubella vaccines into their routine national immunization
programs increased from 83 (13% of the birth cohort) in 1996
to 130 countries (40% of the birth cohort) in 2010.
October 2010, the WHO Region of the Americas and Euro-
pean Region established rubella elimination goals for the years
2010 and 2015, respectively; the Western Paciﬁc Region
established targets for accelerated rubella control and CRS
prevention (<1 case per 100 000) by 2015; and the Eastern
Mediterranean Region established a goal of CRS prevention
without a target date for countries that have introduced
national rubella vaccination programs.
In addition, in 2011,
WHO recommended to the countries that are administering
two doses of measles vaccine but have yet to introduce a
rubella vaccine, to consider including rubella vaccinations in
their immunization program.
Correspondence: Elisabeth S. Herini, MD PhD, Department of
Child Health, Faculty of Medicine, Universitas Gadjah Mada/Dr
Sardjito Hospital, Jl. Kesehatan No. 1 Yogyakarta 55281, Indonesia.
Received 27 October 2016; revised 9 October 2017; accepted
20 October 2017.
© 2017 Japan Pediatric Society
Pediatrics International (2018) 60, 168–172 doi: 10.1111/ped.13444