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Hyperacute Outer Retinal Dysfunction.

Yizhe Cheng1, Xinyu Liu1, Jinglin Lu1

  • 1State 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.

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Summary
This summary is machine-generated.

Pediatric patients experiencing sudden vision loss after fever showed diffuse outer retinal disruptions. Most regained significant visual acuity within a year, though electroretinography remained abnormal, suggesting a distinct condition.

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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.