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Congenital rubella syndrome: ophthalmic manifestations and associated systemic disorders
1Department of Ophthalmology, Geisinger Medical Center, Danville, PA.
Insights
Congenital rubella syndrome causes significant ophthalmic and systemic issues, with ocular disease being most common. Long-term follow-up reveals persistent health challenges for affected individuals.
Area of Science:
- Ophthalmology
- Pediatrics
- Public Health
Background:
- Congenital rubella syndrome (CRS) results from maternal rubella infection during pregnancy.
- The 1963-1965 rubella epidemic led to thousands of infants born with CRS.
- Long-term sequelae of CRS necessitate ongoing medical and ophthalmic care.
Purpose of the Study:
- To analyze the prevalence of ophthalmic disorders in patients with CRS.
- To investigate associated systemic problems and their correlations with ocular defects.
- To provide a 20-year follow-up on the long-term impact of CRS.
Main Methods:
- Statistical analysis of 125 CRS cases from the Mayo Clinic ophthalmology department over 32 years.
- Evaluation of ophthalmic conditions, sensorineural hearing deficits, psychomotor and mental retardation, and cardiac abnormalities.
- Correlation analysis among various defects and with gestational age at maternal infection.
Main Results:
- Ocular disease (78%) was the most prevalent, followed by hearing deficits (66%) and psychomotor retardation (62%).
- Multiorgan involvement was common (88%). Cataracts and microphthalmia correlated significantly with poor visual acuity.
- Glaucoma showed significant correlation with cataracts and microphthalmia.
Conclusions:
- Congenital rubella syndrome presents with a wide spectrum of chronic ocular and systemic complications.
- While new CRS cases are rare, surviving individuals require lifelong management for diverse health issues.
- Ocular and cardiac conditions were frequently associated, highlighting the systemic nature of CRS.
Abstract:
Congenital rubella syndrome has a wide variety of severe ophthalmic and systemic complications. A worldwide rubella epidemic from 1963 to 1965 affected thousands of infants. This is a 20 year follow up study of patients with congenital rubella syndrome analysing the prevalence of ophthalmic disorders, associated systemic problems, and correlations among these defects. The authors statistically analysed 125 cases of congenital rubella seen in the Mayo clinic ophthalmology department over a 32 year interval. Most patients were young adults. Ocular disease was the most commonly noted disorder (78%), followed by sensorineural hearing deficits (66%), psychomotor retardation (62%), cardiac abnormalities (58%), and mental retardation (42%). Multiorgan disease was typical (88%). Ocular disease and hearing loss were frequently associated (53% had both) but not significantly correlated. A similar association existed between ocular and cardiac disease. Cataracts and microphthalmia were significantly correlated with poor visual acuity (each p < 0.0001). Glaucoma was significantly correlated with cataracts (p = 0.0002) and microphthalmia (p = 0.0024) but not poor visual acuity. Four patients with microphthalmia developed late onset glaucoma. No significant association was found between gestational age at time of maternal infection and the incidence of individual ocular conditions. However, several cardiac disorders were significantly associated with gestational age. Although new cases of congenital rubella are rare, surviving victims continue to challenge the ophthalmic and medical communities with a wide range of ocular and systemic disorders.