What does Leber congenital amaurosis look like?

What your baby can and cannot see. In the words of people who live with it, with the frames from our simulator and the sources behind every statement.

Medically reviewed by Stephen Johnston, MD. Reviewed 2026-10-23.

The short answer

Vision in LCA ranges from a little to none. Many children with LCA see light, large shapes and high contrast, especially in bright conditions, but not detail: faces are blurs, and small objects do not exist until they are touched. About one in three see no light at all. Because this is how the child has always seen, there is no "before" for them; the loss is the family's, not the child's. The signs are in behavior: not meeting your eyes, eyes that wander or wobble, staring at windows and lights, and pressing on the eyes.

What the person is experiencing

LCA is a group of inherited retinal diseases that cause severe vision loss from birth or infancy, and most children have very low vision by their first birthday. Sharpness is, with rare exceptions, 20/400 or worse, and about one in three children have no perception of light at all. The patient charities put 20/400 in everyday terms: the child can see at 20 feet what a person with normal vision can see at 400 feet. Many children are sensitive to light, and many are very farsighted. Nystagmus, light sensitivity and slow pupil responses are typical.

Vision is different for each child: "Some people can see a lot, some a little, and some are blind". Those who see usually see better in the distance than up close, are missing detail vision, may see with the sides of their eyes instead of the center, have a hard time in dim light, may not see colors, and may look at things from the side instead of straight on, which adults can misread. One reassuring point: in three studies covering 90 patients, 75% stayed stable over time.

In their words

Hannah Reif's parents describe her vision before gene therapy. Her father: "Best we can describe as she has no peripheral vision and really would look out of larger straws and that's... that was her visual field. And then add on top of that, the need to have to be bright". As an infant she "wasn't making eye contact, and would only look out windows and at bright lights", and her parents noticed her eyes "moved rapidly". She "couldn't see much of anything unless she was in a bright, well-lit room", and in a new house "would walk in the door and put her hands out". Her father lists what she missed: "she never caught fireflies. She couldn't see 'em. We would sit out at night and try to watch the stars; she wouldn't see them". Hannah's form of LCA was treatable, and her mother's account of the night she first saw a star, "I can see it, I can see it!", is in the source. Most forms of LCA do not yet have a treatment, so we quote it for the before, not the after.

"Best we can describe as she has no peripheral vision and really would look out of larger straws and that's... that was her visual field. And then add on top of that, the need to have to be bright." Her father, Children's Hospital of Philadelphia.

Why she presses her eyes and stares at lights

Eye pressing and rubbing with a knuckle or finger are common in babies and children who have very little vision; most children with LCA like to press their eyes. It is thought to produce sensations of light, and it is gently discouraged because over time it can damage the cornea and lens and make the eyes look deep-set. Staring at windows and lamps makes sense once you know that bright light is where the usable vision is: Hannah needed things "to be bright" and her mother describes a desk lamp she always used "to help brighten up whatever is in front of her".

What our simulation shows

Because LCA is present from birth, our two frames show what the remaining vision is like rather than a progression. The progression video for LCA, which runs from normal vision into the LCA frame, is a teaching device for sighted viewers, not a depiction of how the condition develops. Remember too that a child born with this vision has never known anything else; the frame shows you the difference, not their distress.

Around 20/400: The park as a heavily blurred field of light and dark at roughly 20/400 sharpness with a nystagmus smear.

No light perception: This frame is a dark, featureless frame for the one in three children who have no light perception.

Signs you might notice in a baby or child

Not focusing on faces by 4 to 5 weeks or smiling at familiar faces by 6 to 8 weeks; eyes that do not follow a face or object; no eye contact.

Eyes that move quickly side to side, jerk or wander, and eyes that do not react to a bright light being turned on.

Looking only at windows and bright lights.

Pressing or poking the eyes.

Delayed reaching for objects and unusual head positioning in infancy; later, walking in with hands out in new places.

Common questions

Can she see me?

Possibly as a blur or a shape against light, and she will know you by voice, smell and touch. Whether she sees detail depends on her form of LCA.

Will it get worse?

In most children it is stable or very slowly progressive.

Why does the doctor want genetic testing now?

Because one form, caused by two faulty copies of RPE65, has an approved gene therapy, and other gene-specific treatments are in trials.

What helps right now?

Glasses for farsightedness, bright task lighting with sunglasses and a hat outdoors if light is uncomfortable, large high-contrast objects brought close, early intervention services, and learning braille and cane skills alongside any vision the child has.

References

Sources were read on Oct 22-23, 2026.

1. American Academy of Ophthalmology, EyeWiki. Leber Congenital Amaurosis. https://eyewiki.aao.org/Leber_Congenital_Amaurosis

2. Retina UK. Leber congenital amaurosis. https://retinauk.org.uk/information-and-support/about-inherited-sight-loss/types-of-inherited-sight-loss/leber-congenital-amaurosis/

3. California School for the Blind, California Department of Education. Causes of Vision Loss: Leber's Congenital Amaurosis. https://www.csb-cde.ca.gov/resources/causesvl/cvl-lebersca.aspx

4. Children's Hospital of Philadelphia. Gene Therapy for Inherited Blindness: Hannah's Leber Congenital Amaurosis Story (parents Amy and Chris Reif). https://www.chop.edu/stories/gene-therapy-inherited-blindness-hannah-s-leber-congenital-amaurosis-story

5. A Race Against Blindness. How our vision simulations were built: research basis and rendering conventions (Education Hub, Oct 2026). https://araceagainstblindness.org/education/vision-simulations/methodology

6. Raising Children Network (Australia). Low vision and blindness: babies, children and teenagers. https://raisingchildren.net.au/disability/guide-to-disabilities/assessment-diagnosis/low-vision-blindness-babies-children-teens

7. A Race Against Blindness. Children and Families Guide: Early Warning Signs by Age. https://araceagainstblindness.org/education/children-and-families