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Romantic Hole Syndrome of the Retina

On this day, Valentine’s Day, romance and hearts are all around us – even in our eyes – literally. These photos are a submission of a 59-year-old female from Greece, who has Stargardt’s disease. Photographs of her retina reveal heart shaped areas in her eyes, where cells are deteriorating .

Stargardt’s disease is characterised by central vision loss early in life and generally refers to a group of inherited diseases causing light-sensistive cells in the retina to deteriorate – particularly in the area of the macula where fine focusing occurs.

Stargardt’s is an inherited disease passed along to children, when both parents carry mutations of a gene associated with vitamin A processing in the eye. Researchers have found that about 5 percent of people carry gene mutations that cause inherited retinal diseases such as Stargardt’s disease.

Vision loss from Stargardt’s generally begins to appear within the first 20 years of a young person’s life, particularly in early childhood, but there is a great risk of reaching middle age before vision problems are noticed.

  • Blurry or distorted vison
  • Inability to see in low lighting
  • Taking longer time to adjust the eyes when moving from light to dark environments
  • Eyes may be more sensitive to bright light
  • Difficulty recognising familiar faces
  • In the late stages, colour vison also may be lost

Unfortunately, the romance of the disease starts and ends with the heart shape in the retina. Currently, no treatment is available. But much research is being done in the field, and visual aids are available for people suffering from the disease to carry out daily activities and maintain their independence.

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