Hyperacute Outer Retinal Dysfunction

Area of Science:

• Ophthalmology
• Pediatric Retina
• Neuro-ophthalmology

Background:

• Sudden, severe bilateral vision loss in children is rare and requires prompt diagnosis.
• Identifying patterns in pediatric retinal diseases aids in workup and prognosis.

Purpose of the Study:

• To characterize the clinical presentation, retinal imaging findings, and 1-year prognosis of pediatric patients with sudden, severe bilateral vision loss.
• To investigate the association between febrile illness and outer retinal disruptions in children.

Main Methods:

• A multicenter case series of 8 pediatric patients (16 eyes) presenting with sudden vision loss post-fever.
• Clinical evaluation, retinal imaging (including ellipsoid zone and external limiting membrane assessment), and electroretinography (ERG) were performed.
• Follow-up assessments included visual acuity, visual field, and retinal imaging over 1 year.

Main Results:

• All patients experienced sudden bilateral vision loss, nyctalopia, and diffuse ellipsoid zone (EZ) and external limiting membrane (ELM) disruptions after fever.
• Visual acuity improved significantly, with 88% achieving 20/40 or better after 1 year.
• ERG showed extinguished cone and rod responses in all patients, persisting despite visual recovery.

Conclusions:

• A distinct, potentially underrecognized condition in children following fever is characterized by sudden vision loss and outer retinal disruption.
• The term 'hyperacute outer retinal dysfunction' is proposed for this entity.
• Further research is needed to elucidate the pathophysiology and optimal management of this condition.