Sudden Vision Loss in Children Linked to Cold-Related Fever: JAMA

The case series, published in JAMA Ophthalmology, has brought attention to a previously underrecognized retinal condition in pediatric patients, termed hyperacute outer retinal dysfunction (HORD). This condition is characterized by sudden vision loss following fever, accompanied by distinct retinal abnormalities observed in imaging studies. The findings emphasize further research to understand its pathophysiology and optimal treatment strategies.

Recognizing patterns in pediatric retinal diseases is crucial in streamlining diagnostic evaluations and guiding prognosis. In this context, Yizhe Cheng, Guangdong Provincial Clinical Research Center for Ocular Diseases, Guangzhou, China, and colleagues aimed to characterize the clinical features, retinal imaging findings, and one-year prognosis of pediatric patients who experienced sudden, severe bilateral vision loss following fever, marked by diffuse disruptions in the ellipsoid zone (EZ) and external limiting membrane (ELM).

For this purpose, the researchers conducted a multicenter case series involving eight pediatric patients (16 eyes) who exhibited an unusual set of symptoms, including sudden, severe bilateral vision loss, nyctalopia, and diffuse disruptions in the ellipsoid zone and external limiting membrane following a febrile illness. These patients either visited or were referred to a pediatric retina service between November 2022 and May 2023. The researchers assessed changes in visual acuity, visual field, and electroretinography (ERG) results while monitoring characteristic retinal imaging signs.

The following were the key findings of the study:

• The study included 16 eyes from 8 children (6 boys and two girls) with a mean age of 5.1 years.
• Sudden bilateral vision loss occurred an average of 16.1 days after fever onset.
• Initial symptoms included sudden vision loss, visual field constriction, nyctalopia, and dyschromatopsia.
• Baseline visual acuity was predominantly below counting fingers.
• All eyes exhibited a sudden, diffuse loss of the ellipsoid zone and external limiting membrane, with gradual recovery beginning around the fourth week.
• After one year, visual acuity significantly improved in most cases, with 7 of 8 patients (88%) achieving 20/40 or better, including 4 patients (50%) reaching 20/25 or better.
• The macular EZ and ELM were intact in 12 eyes (75%) and 14 eyes (88%), respectively, while extrafoveal EZ and ELM remained absent.
• Electroretinography (ERG) showed extinguished cone and rod responses in all eight patients (100%).
• Despite visual acuity recovery, multifocal ERG remained extinguished in all eight patients (100%).

"The case series highlights a potentially underrecognized condition in pediatric patients following fever, marked by sudden vision loss, diffuse EZ and ELM disruption, and distinct retinal imaging features. The term hyperacute outer retinal dysfunction is proposed to describe this phenomenon, emphasizing the need for further research to better understand its pathophysiology and optimal management," the researchers concluded.

Reference:

Cheng Y, Liu X, Lu J, et al. Hyperacute Outer Retinal Dysfunction. JAMA Ophthalmol. Published online February 13, 2025. doi:10.1001/jamaophthalmol.2024.6372