Ebstein anomaly associated with retinal venular dilatation, migraine, and visual snow syndrome: a case report

P. T. V. M. de Jong, E. F. Thee, B. Straver

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Background: To present a case with Ebstein anomaly, a rare congenital heart disorder, with ophthalmological and neurophthalmological signs and symptoms. To date, retinal venous dilatation and visual snow syndrome have not been previously been published in this anomaly. Case presentation: A 10-year-old white girl was diagnosed with Ebstein anomaly. From age 12, she regularly suffered from migraines with auras and photophobia. At age 16 she started to see short-term bouts of white snow, that after a year were present all day. At age 20, she was found to have a decreased retinal arteriovenous ratio. Conclusions: Retinal arterial tortuosity and venular dilatation are common in congenital heart disease but have not been described in Ebstein anomaly, nor has the visual snow syndrome.
Original languageEnglish
Article number75
JournalBMC ophthalmology
Issue number1
Publication statusPublished - 1 Dec 2022


  • Case report
  • Ebstein anomaly
  • Migraine with aura
  • Retinal venular dilatation
  • Visual snow syndrome

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