Sudden, Severe Vision Loss in Eight Children Linked to Fevers

Researchers have linked eight cases of sudden and severe bilateral vision loss in young children in China to a novel type of retinal dysfunction that seems to follow cold-related fever.

At a mean of 16.1 days after fever onset, patients experienced initial symptoms including sudden vision loss, visual field constriction, nyctalopia, and dyschromatopsia, reported Xiaoyan Ding, MD, PhD, of the Guangdong Provincial Clinical Research Center for Ocular Diseases in China, and colleagues in JAMA Ophthalmology.

“Despite severe initial impairment, most patients achieved substantial central vision recovery, with many reaching visual acuity of 20/40 or better,” they wrote. “These findings suggest this may be a distinct, underrecognized disease, which we propose to name hyperacute outer retinal dysfunction.”

In an accompanying commentary, Timothy M. Boyce, MD, and Ian C. Han, MD, both of the University of Iowa in Iowa City, wrote that “the pathogenesis of [hyperacute outer retinal dysfunction] is unknown, but the viral prodrome in all patients raises suspicion for an inflammatory mechanism, and the delayed onset (about 2 weeks) implicates an antibody-mediated response rather than primary viral retinitis.”

They theorized that “perhaps akin to autoimmune encephalitis, viral infection may ignite an antibody-mediated, hyperinflammatory response … which initially kindles in the outer retina, and once ablaze, results in diffuse photoreceptor destruction.”

The eight patients (six boys and two girls, mean age 5.1 years) had no ocular concerns until they developed sudden vision loss in 2022 and 2023. “All children, or their parents or guardians, reported a sudden, severe, and symmetric decrease in vision affecting both eyes,” Ding and team wrote. “Specifically, symptoms such as difficulty navigating stairs, reading difficulties, stumbling, failure in grasping objects, or falling while walking outside were commonly reported.”

All patients had visual field loss, half had nyctalopia, and 25% had color vision anomalies.

Within 6 to 48 hours after symptom onset, 14 of 16 eyes had visual acuity (VA) of counting fingers or worse, and the other two eyes worsened over the next few days. Soon, VA in all eyes was hand motion (56.3%) or light perception (43.7%).

Intraocular pressure was normal for all patients. However, examinations revealed loss of outer retinal layers, “substantial photoreceptor loss, extensive peripheral field constriction, and extinguished rod and cone responses.”

The patients were all treated with oral or intravenous corticosteroids and, in some cases, methotrexate, intravenous immunoglobulin, and (in one patient) plasma exchange.

“While there was gradual recovery in the macula, the peripheral photoreceptor cells did not recover, ultimately resulting in macular sparing that was associated with maintained central vision,” Ding and colleagues reported.

VA improved in six patients at 3 weeks to 1 month. At 1-year follow-up, seven patients reached a VA of 20/40, and four reached 20/25. All eyes had attenuated vessels, and 62.5% (five children) had peripheral pigmentary abnormalities.

The researchers said that they believe the condition is novel. “A key finding was the initial dramatic loss of the outer retinal layers, followed by a gradual recovery in the macular region. This progression ultimately was associated with a macula-sparing outer layer deficiency in the later stages of the disease, resembling features seen in RP [retinitis pigmentosa] or AIR [autoimmune retinopathy].”

The researchers noted two similar cases, also connected to fevers, that were reported in 2018 in Iran and in 2023 in India.

In regard to cause, Ding and team implicated the fevers that the patients experienced, reaching 100.4 to 104° F, mostly for 2 to 3 days (up to 10 days in one case). All patients took non-steroidal anti-inflammatory drugs, and two also took the antihistamine chlorpheniramine maleate.

They noted that the effectiveness of therapies like steroids is unclear. “We observed that visual recovery began approximately 3 to 4 weeks after onset in most patients, regardless of treatment timing, possibly indicating that recovery may not directly correlate with treatment initiation.”

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