Vision for the Long Term: Paediatric Eye Health

Harry was just three when his mother noticed his eye was turning in. “Every now and then Harry’s pupil would flick in then out, but it would happen so quickly that I almost thought I was seeing things,” said Lynda Cross, Harry’s mother.

The intermittent ‘flicking’ went on for a week, before one day, while playing in the park with her son, Ms. Cross noticed a significant change.

“It was a Wednesday and Harry’s pupil turned in to his nose, and this time it stayed there for a while. I knew I wasn’t imagining it anymore but I thought maybe he was making his eye do that. He said he wasn’t.”

never assume that what you are looking at is benign. You have to
do whatever is best for the child

By that afternoon Harry’s pupil had turned in and wouldn’t budge.

Ms. Cross took her son to a local optometrist in her home town on the south coast of New South Wales. He was promptly referred to see Dr. Harry Leung, an ophthalmologist who has rooms in south Sydney and Wollongong.

Dr. Leung said the unusual features of Harry’s squint alerted him to the fact that Harry’s condition needed to be quickly assessed. “The squint happened quite quickly and Harry’s right eye was not moving fully. Also he wasn’t particularly long sighted. I suspected a sixth nerve palsy and therefore expedited an MRI scan through a local paediatrician. Then the next I heard he was being sent to Sydney Children’s Hospital for urgent neurosurgical review.”

Managing Anxiety

Managing anxiety is essential when it comes to paediatric eye health and Dr. Leung was careful to allay fears while also keeping expectations realistic. “I told the parents that because of the features presenting we needed to get an MRI scan to exclude anything sinister.”

Ms. Cross said Dr. Leung’s approach, along with her own generally optimistic outlook on life helped her maintain her calm. Plus, Harry was healthy and happy. “I wasn’t concerned and I wasn’t keen for him to have the anaesthetic necessary for an MRI, however when Dr. Leung said to me, ‘if it was my child I would have it done’ I changed my mind. Now looking back, that was the least of my worries. Harry has had so many anaesthetics that I’ve lost count.”

Do what’s best for the child

Indeed, Dr. Leung said the MRI identified a rapidly growing brain tumour, which necessitated three operations over almost four years to remove the tumour, which persisted in coming back. “If we had missed the tumour he may not have survived. His family is forever grateful. It’s a rare thing but it just makes me think that you always have to keep the options open and never assume that what you are looking at is benign. You have to do whatever is best for the child.”

Ms. Cross said Harry has taken treatment in his stride although as each operation has loomed, he has become anxious. “Each surgery has done damage – after the first, Harry lost movement on his left side and developed palsy on his face. After the second surgery he lost his hearing on one side and for a while he couldn’t swallow,” said Ms. Cross.

In between surgeries, Dr. Leung saw Harry regularly, dropping by to visit him at Sydney Children’s Hospital and working with him to maintain a program of patching for the squint and to manage his dry eye, which is the result of the facial palsy.

Dr. Leung has watched Harry grow from a toddler into a young school boy. “Harry is a great kid and he’s now at school and getting on very well,” said Dr. Leung. “He is very dear to my heart, every year I bought a special Christmas gift for him to cheer him on as he battles with the recurrent brain tumour and many times I pray with Linda and Harry, to ask God to take care of him.”

Finally, Harry is over the treatment of his brain tumour and he is ready to have the squint surgery. “As he was heading towards his fourth surgery, I was able to reassure Harry by reminding him that the tumour had gone and this next procedure was just to fix his eye,” Ms. Cross said.

She said Harry was also looking forward to the eye operation. “He couldn’t wait to get into hospital. In the lead up to the day of surgery he woke up every morning and ran to the mirror to check his eye. Then he’d come in and see me and he’d say, ‘it’s still turned in’. He just wanted to be like all the other kids,” she added.

Prior to the final surgery, Dr. Leung felt the need to caution Ms. Cross about what lay ahead. “Dr. Leung drew diagrams of Harry’s eye and the results he expected to achieve. He said it would not be possible to completely correct Harry’s squint and it’s likely that he will need more than one surgery. But I was very optimistic, very positive – I saw this as the final stage in Harry’s journey of ill health,” said Ms. Cross. Harry has recently had the squint surgery and is recovering well.

All About Play

Dr. Leung said working with children is a joy. “I just love to be with children! Most of my patients are happy and healthy. They walk in and walk out (sometimes run in and out) and I can usually do something good for them, which is long lasting.

He said to get kids on side, you have to play with them. “The first thing I say is, ‘little Johnny come in and play games with me’. You need lots of bright and colourful toys to show them. For the younger ones, I sing nursery rhymes or make funny noises to calm them down, sometimes I even sit down on the floor with them. I try to think about how the child would like to be treated and speak their language as much as possible.” When it comes to working with teenagers, Dr. Leung says the challenges step up another notch. “You have to treat teenagers with respect – some don’t like their parents to be there – so you have to address them separately and then talk with the parents – it’s a whole new ball game.”

The Biggest Challenge

By far the biggest challenge, of course, is breaking bad news. “What I say can change their lives. When I tell the parents their child will never see normally – perhaps because they have a congenital problem – I have to be professional, but also be compassionate. It breaks my heart when I tell the mother that her child is blind. It’s not just the child who is devastated – in fact they might not realise the consequences – it’s also the impact on the parents and the whole family. But I need to help them to focus on the positives and what can be done to provide the child and the family with as much support as possible,” Dr. Leung said.

Dr. Leung said diagnosing a child with a life-changing congenital illness is personally frustrating. “Sometimes the child is born with a defect and you can’t do anything about it. As a doctor I’ve been trained to fix problems but sometimes this is just not possible. It’s then that I realise how limited I am. The longer I am in this profession, the more humble I become. It is a privilege to walk the journey with the families who have a visually impaired child. They are usually very inspirational. I learn more from them than I can give them.”

New Possibilities

New innovations in technology and approaches to treatment offer hope to surgeons who are looking to find long-term solutions to previously incurable eye conditions. Dr. John Males is a corneal, cataract and refractive surgeon based in Sydney. He said treating children who have keratoconus with collagen cross-linking is a new approach that is able to slow or stop disease progression, and at least buy time so that surgery – such as a corneal transplant – can be performed at a later stage when the best long-term outcomes are more likely to be achieved.

Keratoconus is a thinning of the central zone of the cornea, the front surface of the eye. As a result of this thinning, the normally round shape of the cornea is distorted and a cone-like bulge develops, resulting in significant visual impairment. Cross-linking, a procedure usually performed in rooms, involves soaking the eye in riboflavin drops then using a specific wavelength of UV to induce a chemical reaction that strengthens the collagen fibres, making the cornea stiffer and helping to prevent the bulge from growing further.

“Corneal collagen cross-linking is relatively new – it’s been around for about 13 years – and patients may have not heard about it,” said Dr. Males. “The scientific literature and evidence – particularly surrounding treating children with cross-linking – is behind the reality of what is happening in practice, which can make it more challenging to discuss as an option with other treating eye health professionals and with parents. So it’s a matter of taking the time to do so.”

When mivision spoke to Dr. Males he was preparing to perform cross-linking on an eight-year old child with keratoconus, who lives in Wagga Wagga.

“The boy’s optometrist diagnosed him with allergic eye disease, having a combination of red eyes and light sensitivity,” said Dr. Males. “However, when the allergic eye disease didn’t seem to abate, and when he noticed the boy’s visual acuity was down, he referred him on to a local ophthalmologist who noticed keratoconus and sent him to see me.”

Dr. Males said the patient was at the younger end of the spectrum and had quite advanced keratoconus in both eyes. “The disease progresses as you get older and so, given his young age, it was extremely likely to progress, leading to schooling and social problems. Right now his vision is still correctable with glasses and it will be good if we can stabilise his vision so that it doesn’t deteriorate – then we can buy him some time before surgery is required.”

He said corneal transplants are not performed on children unless absolutely necessary. “The results of corneal transplantation are much poorer in children, and they are more prone to transplant rejection.

“The reasons for doing a corneal transplant on a child are usually fairly severe – so when the treatment is a corneal transplant, it’s usually a serious condition and that comes with a lower chance of success.”

Additionally he said performing even simple procedures, such as removing stitches post-operation, becomes more difficult when the patient is a child, and achieving treatment compliance over the long-term is not easy at all.

Dr. Males said one of the biggest challenges when communicating with the child and parents about keratoconus is managing expectations and explaining the plan for treatment, which often takes place under stressful circumstances. He said this is a matter of taking things slowly and gauging reactions and levels of understanding before moving into detail.

“The concern you have when giving anyone a diagnosis of something that affects vision like keratoconus is that it’s almost like telling a person they have cancer. Once you’ve said the word, you know they won’t hear anything else you say – it’s a lost cause. So the first thing I say is ‘there is an issue but this is something that is manageable, your child’s vision will be a nuisance for a while but it is something we can manage together – it’s a fixable problem’, then I go into what the issues are. I get my model of the eye and we look at the cornea. I explain the problem, and what can be done to prevent their vision from getting worse or to treat their vision if it deteriorates.

“I talk about what they can do at home to minimise risks of deteriorating vision – to stop eye rubbing; to use appropriate medication to reduce allergies; and then I talk about the cross-linking procedure to slow or stop keratoconus. Depending on how they have taken everything I might talk about ongoing treatment with contact lenses or corneal surgery.”

Dr. Males says he supports his discussion by providing patients with literature from Keratoconus Australia and his own, that can be referred to at their leisure. “I work with a fantastic team and I tell patients they are welcome to call with any questions, which either my team or I will do our best to answer.”

While ‘Dr. Google’ can be helpful, it can also be a hindrance when it comes to navigating complex disease processes. “Nothing makes your heart sink more than when a patient comes in with a ream of paper, because often they’ll find all this information and then get themselves in a twist. So my job is to provide the most appropriate information and to help them filter what they have heard or read.”

In the case of the young boy, Dr. Males said the parents had been keen to proceed with cross-linking and he was due to attend Sydney Eye Hospital for treatment in the coming weeks. Following cross-linking and a review the following day, he would be cared for by his local ophthalmologist and would continue to see his optometrist as required. With the patient living so far from Sydney, Dr. Males said clear communication between ophthalmologists, optometrists, the patient and his parents pre-and post-operation, was essential. “We must all be communicating the same message to ensure ongoing management and reduce the chance of the boy’s vision deteriorating,” said Dr. Males.

“Children have long lives ahead of them, with strong demands on their vision, so we know that if we can slow or stop this vision degradation we will make a huge difference to their schooling, and their career options. But we also want to minimise the disruption to their life – so if we can do a relatively simple treatment that delays or stops visual deterioration, that is an excellent outcome. The reward comes in making sure these kids can see as well as possible in as quick and simple a way as possible.”

Managing Complications

For many children, once treatment has been undertaken by an ophthalmologist, life will return to normal with little need for further specialist intervention. However, sometimes complications occur and this is when clear patient communication about post-operative expectations can make all the difference.

General and paediatric ophthalmologist Dr. Caroline Catt cites the example of Tom, a little boy who, like Harry, developed a squint at the age of three, which went on to cause further complications.

Tom, who lives five hours south of Sydney in Ulladulla, was diagnosed as mildly hypermetropic with a high accommodative convergence/accommodation ratio by his local optometrist. He was prescribed the full amount of hypermetropia with a bifocal add, however although he adjusted well to his glasses, the squint persisted. Tom was referred on to Dr. Catt for further opinion and management.

“When I saw Tom, he had equal vision in each eye and a squint. He had full eye movements with bilateral inferior oblique over-action and a normal intraocular examination,” said Dr. Catt.

Despite the power of Tom’s glasses being increased slightly, the next review showed that Tom’s squint persisted and he was diagnosed with a partially-accommodative esotropia.

“The angle of the deviation was stable so after discussing the risks and benefits of surgery with Tom’s family, we scheduled him for treatment at the Children’s Hospital at Westmead,” said Dr. Catt.

Tom underwent uncomplicated strabismus surgery (bilateral medial rectus recessions and bilateral inferior oblique myectomies) under general anaesthesia.

His stayed overnight in Sydney with his family and was reviewed by Dr. Catt the following day.

“Tom was doing well. He had a very small residual esotropia and the expected amount of conjunctival swelling and subconjunctival haemorrhage. I started him on post-operative eyedrops and arranged to review him within three to four weeks. Importantly, I advised Tom and his family that they were to seek attention if Tom experienced increased pain, redness, photophobia or reduced vision.”

Several days later, Tom developed increased pain in one eye and his parents took him to see his optometrist who identified a small corneal ulcer, which she suspected was a dellen, secondary to his adjacent conjunctival swelling. “There was no vitritis nor iritis, which excluded endophthalmitis,” said Dr. Catt.

“The optometrist called me and we discussed the findings together,” said Dr. Catt. “We agreed to continue the eyedrops and increase ocular surface lubrication with chlorsig ointment. Tom’s symptoms settled and at the last review I could see the dellen had completely resolved. Additionally, his ocular alignment demonstrated orthophoria when Tom was wearing his glasses.”

Dr. Catt says you have to be fast, creative and playful when examining children as young as Tom. Additionally, she said, it is necessary to be decisive in your clinical decision making, despite the fact that many of the examination techniques used in adults are not available to all children.

“You also need a good knowledge of whether or not it’s OK to go without the information you’d ideally like to have,” said Dr. Catt. “For example, in a child with excellent vision and features of blepharokeratoconjunctivitis in whom you cannot get a good view of the fundus, it may be appropriate to try to repeat the fundus exam on another day when they return to the clinic. In contrast, in a child with poor vision, a red eye and luekocoria in whom you cannot get a good view of the fundus, you can’t rule out something nasty like retinoblastoma so you may end up having to do an urgent examination under anaesthesia. Fortunately, examinations under anaesthesia are rarely required. With skill and effective parental help, most crucial components of the examination can be done in the clinic.”

With a child’s visual system still undergoing development, Dr. Catt said it is important to be aware that they are vulnerable to amblyopia. “This needs to be considered in every paediatric patient, in addition to their other ophthalmic diagnoses. Failing to consider amblyopia or to consider that your patient has a long life ahead of them is to limit their visual potential throughout life. Thoughts of this can weigh heavily on the paediatric ophthalmologist.”

Smiles and Rainbows

While the challenges and responsibilities of paediatric eye care can be weighty, Dr. Catt said the rewards are massive. “The greatest rewards come from the patients and their parents. Seeing the vision improve in a densely amblyopic eye, or seeing great ocular alignment from surgery or prescribing the right pair of glasses, or seeing clear corneas and good pressure control in a child with congenital glaucoma is good, but the best moments come from something special or funny or moving said to you by one of your patients. Plus, we get given lots of drawings. My favourite is a portrait of me standing under rainbow.”