The Life-Saving Power of a Squint: When to Investigate a Child’s Turned Eye
Monday September 30, 2024
Evalynn, Gabriel, Abby and John all developed strabismus (squint or turned eye), one of the most common signs of retinoblastoma. This can also occur in healthy eyes or affect multiple family members without eye cancer. Sandra Staffieri, Retinoblastoma Care Co-ordinator at the Royal Children’s Hospital, Melbourne, explains, and answers the key question – when should parents and doctors be concerned?
Can you tell which of these two babies has cancer?
Read on to find out!
Straightforward Squint or Sinister Sign?
Many words can be used to describe a turned eye. “Lazy eye”, “wandering eye”, “cast eye”, “squint”, “crossed-eye”, but the medical term is ‘strabismus’.
The most confusing of all these terms is “lazy eye” as it is used interchangeably to mean different things. It may refer to ‘strabismus’, or to an eye that has ‘poor vision’, or sometimes when the eyelid ‘droops’ or appears ‘lazy’ – probably because it isn’t opened up enough! It’s no wander there is confusion when we talk about strabismus since in lay terms, we don’t have a single, consistent word to describe it.
On top of that, a health practitioner using the word ‘lazy’ might infer that the eye will get ‘stronger’ or ‘better’ by itself. True strabismus does not get better by itself.
“Which Way Can It Go and How Big Does It Need To Be?”
Usually, the two eyes should point in the same direction and move together to follow an object of interest. When strabismus is present, the affected eye will usually wander inwards or outwards but can also go up or down instead. Strabismus may be observed some or all of the time. The turn may be obvious only at certain angles or even only in a photo, which makes it very confusing or difficult for a parent to decide if they should see a health care practitioner.
All eye turns are different and can be caused by different things. Irrespective of the direction of the strabismus, how far in, out, up or down it goes, or even how often you see it, the child’s eyes should be assessed by someone experienced in doing so.
Outward turn (exotropia)
Upward turn (hypertropia)
Inward turn (esotropia)
The three children pictured above were all diagnosed with retinoblastoma. A downward turn (hypotropia) is harder to photograph and often goes unnoticed because the eyelid masks it – eye turns are most noticeable due to an imbalance of visible sclera (white of the eye).
“All Babies Eyes Turn! Won’t They Just Grow Out Of It?”
Strabismus is complicated. It may be an optical illusion, occur as a normal part of early development, or most of the time not be caused by retinoblastoma at all.
For a very short period in a baby’s life, strabismus that comes and goes can be normal. This usually occurs briefly as the baby’s brain develops all the connections needed to use the information from both eyes at the same time.
By the time baby is 4 months old however, their eyes should be straight all the time, and working together. Strabismus in a child beyond this age, even if it comes and goes, should be assessed by someone who is used to examining young children.
The appearance of an eye turn can frequently be an optical illusion caused by the baby’s wide nasal bridge. This wide fold of skin hides the white of the eye and can give the impression the eye is turning in. As their face grows, the wide fold of skin narrows, and the eyes look straight.
This is probably why we often hear the advice: “Don’t worry, he’ll grow out of it”.
“Why Does Retinoblastoma Cause A Turned Eye?”
A turned eye can either CAUSE poor vision or BE CAUSED by poor vision.
For most children, a turned eye will cause poor vision which still makes it very important to diagnose and treat early whilst their vision is still developing.
In children with retinoblastoma, once the tumour in the eye interferes with their vision, this can cause the eye to wander. Often, parents will describe seeing strabismus long before the white pupil, but because we are ‘trained’ or ‘conditioned’ to believe strabismus is normal, it is readily dismissed.
As with the white pupil in photographs, there is no way to distinguish between strabismus that is caused by retinoblastoma and one that is not. As with white pupils, the only way to be sure is for the child to be assessed by someone who is used to examining children’s eyes.
A) Something
B) Nothing
Both these photos show a child with a large inward turning left eye.
In picture A – the child was found to have retinoblastoma in the left eye. However, the child in picture B had an otherwise normal eye. His strabismus was due to a muscle problem that required an operation.
Their strabismus looks exactly the same. Only an eye examination can determine the underlying cause.
Sometimes strabismus runs in families – either parent or other siblings can have strabismus too. Because it is ‘familiar’, it is easy to ignore and ‘wait’, thinking there is no rush for any assessment or treatment. But no two children are the same – even in the same family! Every child with strabismus should promptly have a full eye assessment, including examination inside the eye.
In fact, in 1969 Dr Robert Ellsworth, a leading expert in retinoblastoma, even suggested “it is not a bad idea to consider that every child with strabismus has retinoblastoma until proven otherwise.” I can understand why he would think that, and his wise words still hold today.
One Big Problem
The biggest problem for retinoblastoma early diagnosis is that it is a rare disease whose early signs are not only common, but often mean nothing or nothing serious. Thus, in raising awareness of strabismus and white pupils as signs of retinoblastoma, there is a fine balance to strike between being alert without being alarmed; vigilant without being paranoid.
For Every Problem There Must Be A Solution!
“I wouldn’t be worried about that – it’s just the flash; I’ve never heard about a white pupil. You’d think you would have if it was important?”
“I wouldn’t see a turned eye and think ‘Oh, that is an emergency…It doesn’t seem like an emergency, it seems like a developmental problem that you can get addressed in due time.”
It is clear from parent interviews that there is a lack of knowledge about the importance of the early signs of retinoblastoma. But this lack of knowledge is because we haven’t told them.
“You don’t know, what you don’t know!”
Parents or caregivers are usually the first to notice a problem with their child. It is our responsibility as health practitioners and retinoblastoma advocates to provide relevant and useful information to parents in a way that can be understood, and often enough for it to be remembered. There are many ways we can provide information. In this new age of information overload via the internet and social media, we may feel overwhelmed and start to ignore information that is actually important.
Solving the problem of awareness is neither easy nor straightforward. Indeed, raising awareness among health practitioners is also very important, and perhaps even more so. There are many instances where parents have reported a problem, only for it to be dismissed without an adequate examination or referral.
Empowering parents with information to recognise and respond to strabismus and white pupils, and providing strategies and resources to navigate when these signs are seen, is critical to educating the health practitioner they visit. Combined, we may start to see earlier diagnosis of retinoblastoma and better outcomes for all children and their families.
All you need to remember: “Something looks the same as nothing. If in doubt, check it out!”
When ‘Something’ Looks the Same As ‘Nothing’
This information about turned eye is reproduced from Dr. Sandra Staffieri’s article on our blog: When ‘Something’ Looks the Same As ‘Nothing’: Strabismus, Leukocoria and Childhood Eye Cancer. Read the original article to learn about white pupil as the most common early sign of retinoblastoma.
White reflex & no fundal reflex
White reflex & strabismus
Strabismus
Turning In to Mother’s Intuition
Evalynn’s Story – USA
Evalynn was 10 months old when Vanessa noticed her daughter’s eye drifting slightly when she was sleepy or attempting to focus on distant objects. The “lazy eye” was so subtle, only Vanessa noticed it. Family members assured her it was normal for young children to have a difficult time focusing, but her mother’s intuition nagged.
Vanessa raised her concerns with the paediatrician. Though he believed the problem to be muscular, he referred Evalynn to an ophthalmologist for a thorough exam. That exam led to Evalynn’s diagnosis of retinoblastoma. Mother’s intuition saved her life.
A Simple Squint Becomes a Lifesaver
Gabriel’s Story – Ecuador
A few months after Gabriel’s first birthday, his parents began to notice his left eye was slightly misaligned. They weren’t overly concerned, but being cautious, they took him to see an ophthalmologist, close to their home in Ecuador, South America.
They had noticed “the Glow” in Gabriel’s eyes in flash photos, but not knowing of its significance, they dismissed it as a problem with the camera.
When Gabriel was examined, his parents were shocked to learn the misalignment and glow in photos were signs of cancer – in both eyes.
Gabriel’s mum, Yesenia, wants all families to know that strabismus and leukocoria can be silent signs and potential lifesavers. She strongly encourages families to have your children checked if you notice anything off with their eyes or vision.
Averted Gaze Raises Concern
Abby’s Story – USA
Abby was diagnosed with cancer in both eyes aged 18 months, after her parents noticed her eyes weren’t focusing properly. She showed no signs of developmental problems until the tumours invaded her central vision, at which point her mum and dad noticed she often had an averted gaze; she would interact with them directly, while looking off to the left rather than straight on.
Doctors at the local children’s hospital diagnosed Abby’s retinoblastoma two weeks later after a CAT scan and an MRI. Only then did her parents learn that a white glow in photos can be a sign of eye cancer – a sign that began in Abby’s photos 7 months earlier. They had even used red-eye correction tools to remove the glow, thinking it was a camera fault or a trick of the light.
Liz is keen for other parents to know about these early signs and the importance of seeking a prompt eye exam.
Abby’s parents didn’t notice the white reflex in these photos until after she was diagnosed with cancer in both eyes. As often happens, the glow didn’t appear in every picture. This depends on the size, number and location of tumours, and the angle at which the flash enters the eye. Her squint pushed them to seek medical care.
Observation, Intuition, and Life-Saving Action
John’s Story – Kenya
When John was one year old, his mum, Carol, noticed his eyes didn’t follow together properly. She wasn’t concerned as his father also had a squint that didn’t significantly impact his sight.
However, when Carol began to see a white glow in John’s eye a few months later, she knew something was wrong. She first noticed the reflex while breastfeeding, the light shining down into his eyes as he lay in her arms.
Carol took John to Nyahururu General Hospital, near her home in central Kenya. After an eye exam, the doctor referred John to Kenyatta National Hospital (KNH), but didn’t tell Carol his suspicions. Carol headed for the hospital with John, worried about how the glow would be removed. She never imagined the experience that awaited them in Nairobi.
The next day, before heading to KNH, Carol took John to Lion’s Eye Hospital, which provides charitable care for children. The doctors there insisted Carol go urgently to Kenyatta, and in that moment she began to fear for her little boy…
At KNH, tests found that John had advanced eye cancer. Aggressive treatment was needed immediately to save his life. Carol’s keen observation, instinct, and advocacy, and the swift action of the first doctors who saw him had worked together to bring him to the care he needed just in time.
John and his twin brother, Daniel.
About the Authors
Sandra Staffieri is the Retinoblastoma Care Co-ordinator at the Royal Children’s Hospital (RCH) Melbourne, Australia. Working at the RCH and in private clinics, she has over 35 years’ experience in children’s eye health and disease.
As a Research Fellow and Clinical Orthoptist at the Centre for Eye Research Australia, Sandra completed her PhD on delayed diagnosis of retinoblastoma. Her prime focus was to develop and evaluate an information pamphlet for new parents to raise awareness of the important signs of childhood eye disease – particularly strabismus and leukocoria – in the hope this could lead to earlier diagnosis.
Also From Our Blog:
Abby White’s father was diagnosed with bilateral retinoblastoma in Kenya in 1946. Abby was also born with cancer in both eyes. She has an artificial eye and limited vision in her left eye that is now failing due to late effects of radiotherapy in infancy.
Abby studied geography at university, with emphasis on development in sub-Saharan Africa. She co-founded WE C Hope with Brenda Gallie, responding to the needs of one child and the desire to help many in developing countries. After receiving many requests for help from American families and adult survivors, she co-founded the US chapter to bring hope and encourage action across the country.
Abby enjoys listening to audio books, creative writing, open water swimming and long country walks.
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