Hyperacute outer retinal dysfunction may be an underrecognised paediatric disorder
Children experienced sudden vision loss, visual field constriction, nyctalopia and dyschromatopsia following febrile illness
Pattern recognition of paediatric retinal diseases can streamline diagnostics and guide a patient's prognosis, the authors said. Image credit: ©Alexis Scholtz/peopleimages.com – stock.adobe.com
Chinese researchers reported that hyperacute outer retinal dysfunction may be an underrecognised disorder in children and emphasised the need for early detection.1 First author Yizhe Cheng, MD, is from the State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Guangdong Provincial Clinical Research Center for Ocular Diseases, Guangzhou, China.
The researchers studied a series of eight children (16 eyes) who presented with sudden bilateral visual loss and outer retinal disruption following an illness characterised by a fever.
The investigators set out to characterise the clinical features, describe the findings on retinal imaging and determine the 1-year prognoses of these children. The examinations showed severe bilateral vision loss with diffuse ellipsoid zone (EZ) and external limiting membrane (ELM) disruptions following a febrile illness.
The primary outcomes were the changes in the visual acuity (VA), visual fields, electroretinography (ERG) results and presence of characteristic retinal imaging signs during follow-up.
The six boys and two girls were a mean age of 5.1 years (range, 3-7 years). The onset of their sudden visual loss was a mean of 16.1 days after they experienced a fever. The authors reported that the initial symptoms included sudden vision loss, visual field constriction, nyctalopia and dyschromatopsia, symptoms that they described as unusual. The baseline VA was mostly below counting fingers.
Dr Cheng and colleagues reported, “A sudden, diffuse loss of the EZ and ELM was observed in all eyes with gradual recovery beginning around the fourth week. After 1 year, the VA improved substantially in most cases, with seven of eight patients (88%) achieving 20/40 or better, including four patients (50%) achieving 20/25 or better.”
In addition, the macular EZ and ELM were intact in 12 (75%) and 14 eyes (88%), respectively, while the EZ and ELM were absent in the extrafoveal regions. The ERG recordings showed extinguished responses of the cones and rods in all patients, and multifocal ERG remained extinguished despite the VA recovery in all patients.
Relevance of these findings
The investigators commented, “This case series identified a potentially underrecognised disease in paediatric patients after a fever characterised by sudden vision loss, diffuse EZ and ELM disruption, and distinct retinal imaging features. The term hyperacute outer retinal dysfunction is recommended as descriptive, while further investigations are recommended to better understand its pathophysiology and optimal management strategies.”
They also pointed out that pattern recognition of paediatric retinal diseases can streamline the workups and guide the patient prognoses.
