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Purpose: To evaluate the clinical characteristics of congenital rubella retinopathy (CRR) with modern fundus imaging. Methods: Single-center case series. Eleven patients (2005–2020) at the Emory Eye Center with known or presumed CRR. Trained image readers reviewed fundus imaging (color fundus photography, widefield pseudocolor imaging, near-infrared reflectance imaging, autofluorescence imaging, and spectral-domain optical coherence tomography) for pre-specified features suggestive of CRR. Results: Eleven patients with confirmed (63.6%) or presumed (36.3%) CRR were identified. All were female with median (range) age of 53 (35–67) years. Six (54.5%) were born during the 1964–1965 United States rubella epidemic. All had congenital hearing loss. Two (18.2%) had a congenital heart defect. Eleven (50.0%) eyes had salt-and-pepper retinal pigmentary changes. Twenty-two eyes (100.0%) had irregularly distributed regions of speckled hypoautofluorescence. One eye (4.5%) had a presumed macular neovascularization. Conclusion: Modern fundus imaging demonstrates characteristic features of CRR, even when pigmentary changes are not readily apparent on examination. Widefield autofluorescence findings of irregularly distributed speckled hypoautofluorescence are particularly revealing. This series of newly diagnosed adults with CRR may represent the milder end of the phenotypic spectrum of this condition, highlighting imaging findings that may aid in diagnostically challenging cases of CRR. [ABSTRACT FROM AUTHOR]

Rubella, also known as German measles, is usually a very mild infection that can have devastating effects in certain instances. It is a pleomorphic RNA virus in the Toga-viridae family of the genus Rubivirus. It typically causes a scarletiniform rash, cervical lymphadenopathy, and mild constitutional symptoms, but in older children and adults, especially women, it may be more severe, with joint involvement and purpuric rash. Infection during the first 12 weeks of pregnancy results in congenital infection and/or miscarriage in 80-90% of cases. The congenital rubella syndrome (CRS) involves multiple organ systems and has a long period of active infection and virus shedding in the postnatal period. For these reasons, the rubella vaccine program was instituted in 1969, and the incidence of rubella infection in the United States has since declined by 99%. Rubella has been recognized as a disease for approximately 200 years, and it has since been found that humans are the only natural reservoir for the rubella virus. Virus is present in nasopharyngeal secretions, blood, feces, and urine during the clinical illness, although patients with subclinical disease are also infectious. The virus is spread via oral droplets and is shed in the nasopharynx for approximately 7 days before and after the rash is visible. CRS includes a configuration of anomalies, including nerve deafness, cataracts, cardiac anomalies (usually pulmonary artery and valvular stenosis, and patent ductus arteriosis), and mental retardation, with late complications including diabetes, thyroid disease, growth hormone deficiency, and progressive panencephalitis. In 1969, the first rubella vaccine was licensed for use, and the Centers for Disease Control and Prevention (CDC) began its National Congenital Rubella Syndrome Registry. As required under the National Childhood Injury Act, all healthcare providers in the United States who administer any vaccine shall, prior to administration of the... [ABSTRACT FROM AUTHOR]