Mysterious illness from China leads to childhood blindness: what we know so far.

Chinese researchers have identified a new retinal disease in children that is associated with illnesses, one of the symptoms of which is fever. This condition, known as hyperacute external retinal dysfunction (HORD), results in sudden vision loss in both eyes, impairment of photoreceptor function, and varying degrees of recovery, writes Medical Xpress.

Scientists from the State Key Laboratory of Ophthalmology in China examined eight pediatric cases involving children aged 3 to 7 who experienced severe vision impairment approximately two weeks after a febrile illness.

Despite initially low visual acuity, most patients showed improvement in central vision within a year. However, retinal imaging revealed abnormalities in the ellipsoid zone and external limiting membrane, while electroretinography (ERG) demonstrated a complete loss of photoreceptor responses, even in cases of visual recovery.

The study included a comprehensive examination: the children underwent extensive ophthalmological and systemic evaluations, excluding conditions such as hereditary retinal diseases, uveitis, and white dot syndromes.

Treatment approaches varied: some patients received corticosteroids, intravenous immunoglobulin (IVIG), or methotrexate, although the effectiveness of these treatments remains uncertain.

Symptoms of HORD initially included severe bilateral vision loss, difficulties seeing in low light conditions, reduced visual fields, and color perception issues. Early fundus imaging showed minimal deviations, but optical coherence tomography (OCT) scanning revealed significant damage to the ellipsoid zone and external limiting membrane.

Further research into the origins of HORD suggests a possible inflammatory mechanism. Experts from Iowa State University noted similarities between HORD and autoimmune encephalitis, proposing an antibody-mediated process as a potential cause. Early OCT results support the notion that acute inflammation contributes to retinal damage.

Although HORD shares some characteristics with other retinal diseases, such as non-paraneoplastic autoimmune retinopathy (np-AIR) and multiple evanescent white dot syndrome (MEWDS), its rapid onset and severity in young children differentiate it from these conditions.

Understanding the root cause of HORD, improving treatment strategies, and identifying specific biomarkers may enhance diagnosis and treatment. Current research on new anti-retinal antibodies could provide further insight into disease mechanisms and potential therapeutic targets.

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