Q&A: White pupils in a photo can be a sign of a serious condition, but not always
Key takeaways:
- Leukocoria can appear in photos taken with a flash.
- Sometimes leukocoria appears in children without eye disease.
- Children with leukocoria should always be evaluated by a pediatric ophthalmologist.
Flash photos typically produce a red reflection in people’s eyes, but sometimes that reflection can appear white or gray, which is known as leukocoria.
When leukocoria is seen in photos, ophthalmologists refer to it as “photoleukocoria.” It can be indicative of retinoblastoma or cataracts, or be purely physiologic and not associated with ocular disease.
Evan Silverstein, MD, a pediatric ophthalmologist and assistant professor of ophthalmology at Virginia Commonwealth University School of Medicine, and colleagues published a report in Pediatrics about two children who were referred to their clinic after their parents noticed leukocoria in photos taken with their smartphones. In both cases, the cause of the leukocoria was a reflection from the child’s optic nerve that was visible in the photos.
However, leukocoria should be taken seriously, the researchers wrote, because photoleukocoria was 12 times more likely to be associated with ocular disease than benign physiologic photoleukocoria. Any time leukocoria is detected, Silverstein said the patient should be referred to a pediatric ophthalmologist to rule out more serious diagnoses.
They also pointed out that it is likely that a parent would be the first to notice leukocoria. According to a study they quoted, most retinoblastoma cases that presented with leukocoria were discovered by a family member or nonphysician first.
Healio spoke with Silverstein about what parents should do if they notice leukocoria in their child’s photos and how pediatricians should talk to worried parents about it.
Healio: How common is leukocoria, and what are the most common causes?
Silverstein: Leukocoria is not something we see often. However, it can be very worrisome to parents and to pediatricians.
Every pediatrician is very familiar with the red reflex, taking the direct ophthalmoscope to check for the presence or absence of a red reflex during well-child examinations. When you shine the light, it is reflecting against the retina, causing that red reflex. When there is something between the front part of the eye and the retina, then you get an abnormal red reflex.
Leukocoria is a white reflex that can be a congenital cataract, which is preventing any light from getting into the retina, or it could be a retinal lesion, like a retinoblastoma, which is the big worry. Instead of the light reflecting against the normal retina, it is reflecting against a tumor or other growth in the eye, or a vascular malformation that would be leading to the retina being white instead of red.
Healio: Can leukocoria appear in children without any underlying conditions?
Silverstein: Sometimes you can see leukocoria when the child is looking slightly toward their nose in that eye, which would pick up the optic nerve. This is a physiologic normal cause of leukocoria. When light comes into the eye, it focuses in one spot in the back part of the eye. Usually, that is the macula or the fovea where you want to be focusing, because that is where your highest concentration of cones is, and it is where you can get your highest resolution of vision. All of the light tends to come in and focus on that one spot. If that one spot happens to align with your optic nerve, that is when you can get the physiologic leukocoria. Now, if that one spot aligns with a retinal tumor or retinoblastoma, then that would also show it as a white reflex.
Healio: In your article, you describe a case of two children whose parents saw the white reflex in their child’s eye in smartphone photos. What should parents do if they notice this in their child’s photo?
Silverstein: There are a couple of things that they need to be thinking about. Number one, is this happening with every photo that they take? Number two is the positioning of the eye. But the fact is, if you are picking this up, you do need a full dilated eye examination with a pediatric ophthalmologist. We want to rule out all those scary things, and we want to make sure that everything is okay. The important lesson from our paper is that leukocoria is not always retinoblastoma. It is not always a cataract. Sometimes it can pick up something very normal in your eye — your optic nerve. If the involved eye is looking in slightly toward the nose, then that raises the suspicion for it being the optic nerve.
Healio: How can pediatricians and primary care providers talk to worried parents about this?
Silverstein: Always start with thanking the parents, acknowledging their worry and never brushing it off. You start by saying, “Thank you so much for showing this picture. I understand why you're worried because you may have Googled this and there can be some very significant pathology that that you can detect with these white reflexes.” Then if I was the pediatrician, I would perform the red reflex to see if it is reproducible. If it is a retinoblastoma, the pediatrician should be able to detect it with the white reflex.
Have the patient look in all different directions, trying to recreate the position of the eye in the photos. Looking at the pictures themselves, I think it is helpful for the pediatrician to point out if they see optic nerve details like we saw in our paper, but at the same time say, “We see this white reflex on the photograph. Given that it can be worrisome, we are going to have you see a pediatric ophthalmologist within a week or 2. I don’t want you worrying too much because this could be a normal finding, but we do have to rule out retinoblastoma and other things.”
For more information:
Evan Silverstein, MD, can be reached at evan.silverstein@vcuhealth.org.
Perspective
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Pediatricians follow screening recommendations as outlined in the AAP Bright Futures periodicity guidelines, which recommend assessment of ocular health and vision beginning at the newborn exam and at each subsequent well-child appointment. One of the most important elements of the eye examination that should be performed at each visit is using a direct ophthalmoscope to check for the presence of a bright symmetric fundus light reflex. Although this is commonly called the red reflex, the reflex can appear yellow or even gray in more darkly pigmented children, thus more accurately termed the fundus reflex. Bright symmetric images from each eye indicate that light from the ophthalmoscope can both enter the eye and be reflected back out of the eye.
When the fundus reflex is absent in one or both eyes this can be indicative of conditions such as cataract, vitreous hemorrhage or high refractive errors — conditions that must be addressed in the first weeks of life to prevent lifelong vision impairment due to deprivation amblyopia. When the fundus reflex looks white, or when the pupil looks white with direct illumination, leukocoria is the most accurate description and immediately raises concern for retinoblastoma or complete cataract.
Although leukocoria is a condition detected by physicians, it can also be observed by parents and families, incidentally, discovered when viewing digital photos. Changes in smartphone camera technology purposefully try to eliminate the red fundus reflex from photos, but the reflexes are still commonly seen and the appearance of asymmetric, absent or frankly white reflexes often alarm parents that underlying pathology may be present.
This new article reports two cases of “pseudoleukocoria,” where smartphone images captured images of the optic nerve, leading to a white rather than red fundus light reflex. Neither child had any ocular pathology, but parental concern prompted a full ophthalmologic evaluation.
The condition pseudoleukocoria is not uncommon; sometimes, the pediatrician will see the patient in their office to check the fundus reflex themselves, and if normal, simple reassurance will allay fear, but often referral to ophthalmology will be advised because the consequence of missing a potentially lethal condition warrants a cautious approach with anxious patients. If the child is seen in the office and normal red reflexes are observed with the direct ophthalmoscope, simply explain to the parents how smartphone images are highly dependent on direction of a child’s gaze when the pupil fundus reflex is captured. It is often helpful to ask the parents to use their phone in the office to take a photo — both with the flash on and off — and the highly variable nature of the fundus reflex will be demonstrated. Of course, continued concern by either the caregiver or physician warrants prompt referral to an ophthalmologist, who can easily confirm absence of ocular pathology.
Clinical professor of ophthalmology
University of California, San Francisco
Member, Ocular Surgery News Pediatrics/Strabismus Board
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