Congenital rubella syndrome—major review

doi:10.1016/j.optm.2008.03.006

Optometry - Journal of the American Optometric Association

Volume 80, Issue 1, January 2009, Pages 36-43

https://doi.org/10.1016/j.optm.2008.03.006 Get rights and content

Abstract

Congenital rubella syndrome is a rare disorder with devastating ocular and systemic consequences. Although efforts to eradicate the disease have been in place for some time, some areas of the world continue to be affected by this disease. The burden of the disease weighs heavily on patients and society; therefore, vaccination and other preventative strategies should continue to be strongly encouraged.

Section snippets

Historical background of rubella and CRS

In 1752 and 1758, respectively, 2 German physicians, De Bergen and Orlow, discovered the modern day German measles, then known as Röthel.¹ It was not until 1866 that the name rubella was given to the disease by the Scottish physician Veale, who further elaborated on its clinical characteristics and significance.² About a century later in 1941, an Australian ophthalmologist, N. McAlister Gregg, observed that there was resounding evidence of pregnant mothers with rubella transmitting congenital

Epidemiology of CRS

The absolute risk of CRS among children born to mothers infected during pregnancy varies greatly depending on the study, ranging from 1.7 per 1,000 live births in Israel, 0.7 in Oman, 2.2 in Panama, 1.5 in Singapore, and 1.7 in Jamaica.²⁵ Rubella is a worldwide disease, with peak age incidence less than 10 years of age, causing 70% to 90% of the adult population to have been introduced to the disease with those individuals now possessing rubella antibodies. However, vaccination is still

Pathogenesis of CRS

The significance of rubella derives from its teratogenic effects on the fetus when rubella affects pregnant women. The rubella virus is a member of the togavirus family, and the genus, Rubivirus. It is closely related to alphaviruses but differs in that it is not transmitted by vectors. The rubella virus is roughly spherical with a diameter of 60 to 70 nm. It is composed of an icosahedral nucleocapsid containing a single-stranded RNA genome; this is surrounded by a complex lipid envelope.27, 40

Systemic manifestations

The term congenital rubella syndrome is used, as defined by South and Sever,¹⁵ to denote any combination of the findings known to result from gestational rubella. The classic CRS is characterized by the combination of cardiac, ocular, and hearing defects, although the active rubella virus in a fetus can infect virtually any organ. As many as 50% of CRS infants will appear normal at birth, but abnormalities of the central nervous system may develop with time.25, 51 The main defects of the

Ocular manifestations

The fetal eye commonly is affected via the bloodstream, although the lymphatic system may also play a minor role in infection transmission.9, 61 Because the fetus does not have cell-mediated or humoral immune defenses until around the 20th week of gestation, the rubella virus can pass virtually unchallenged via the capillary network to every part of the developing eye.⁹ The ocular pathologies most commonly observed with CRS are nuclear cataracts, microphthalmia, and pigmentary retinopathy.

Diagnosis of rubella during pregnancy

In women affected with rubella during pregnancy, a prenatal diagnosis of a rubella-infected fetus may be beneficial. However, counseling of these women may be difficult because the rate of CRS is always lower than that of the maternal infection rate and because not every infection leads to fetal damage.⁷²

There are 2 accepted forms of early diagnosis, amniocentesis and fetal blood testing. Both tests should be performed 6 to 8 weeks after maternal infection but yield the best reliability if the

Economic consequences

The rubella vaccination program in the United States has resulted in a significant reduction of morbidity and mortality and in cost savings over time. In the United States alone, the lifetime cost of treating a patient with CRS is estimated at more than $200,000.⁷⁵ In other countries, the cost varies, from $63,900 in Guyana, about $50,000 in Barbados, and approximately $14,000 in Jamaica.75, 76 It is estimated that even with the current vaccination strategies in place, 1,500 cases of CRS will

Conclusion

Congenital rubella syndrome is a devastating condition that has multiple consequences for any society, both medical and financial. As such, the implications of this disease are imperative for clinicians and politicians alike to comprehend. To date, preventative strategies have made large differences, but there is still much more work to be done.

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The past two decades have seen expansion of childhood vaccination programmes in low-income and middle-income countries (LMICs). We quantify the health impact of these programmes by estimating the deaths and disability-adjusted life-years (DALYs) averted by vaccination against ten pathogens in 98 LMICs between 2000 and 2030.
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Review articleCongenital rubella syndrome—major review

Abstract

Section snippets

Historical background of rubella and CRS

Epidemiology of CRS

Pathogenesis of CRS

Systemic manifestations

Ocular manifestations

Diagnosis of rubella during pregnancy

Economic consequences

Conclusion

Clin Perinatol

J Pediatr

Lancet

Pathology

Lancet

Lancet

Lancet

Adv Virus Res

J Pediar

Am J Ophthalmol

Ophthalmology

History of an epidemic of Rotheln with observations on its pathology

Edin Med J

Congenital cataract following German measles in the mother

Trans Ophthalmol Soc Aust

Current concepts of infections of the fetus and newborn infant

Viral infections of the fetus and neonate

Chronic infections

Infectious disease

Final observations on congenital defects in infants following infectious diseases during pregnancy, with special reference to rubella

Med J Aust

The ocular manifestations of congenital infection: a study of the early effect and long-term outcome of maternally transmitted rubella and toxoplasmosis

Trans Am Ophthalmol Soc

Rubella epidemic, 1964: effect on 6,000 pregnancies

Am J Dis Child

Neonatal thrombocytopenic purpura and other manifestations of rubella contracted in utero

Am J Dis Child

Rubella syndrome

Teratogen update: the congenital rubella syndrome

Teratology

Transplacental rubella infection in newly born infants

JAMA

Studies on the experimental transmission, clinical course, epidemiology and prevention of rubella

Trans Assoc Am Physicians

The ocular manifestations of congenital rubella

Trans Am Ophthalmol Soc

Rubella and congenital cataract blindness

Med J Aust

Rubella (German measles)

Update: rubella and congenital rubella syndrome, 1980-1990

Epidemiol Rev

Resurgence of congenital rubella syndrome in the 1990s: report on missed opportunities and failed prevention policies among women of childbearing age

JAMA

Maternal rubella and the congenital rubella syndrome

Clin Perinatol

Ocular manifestations of congenital rubella

Curr Opin Ophthal

Rubella in the United Kingdom

Epidemiol Infect

Rubella

Rubella

Rubella reinfection: What risk to the fetus?

Med J Aust

Review article
Congenital rubella syndrome—major review