Why Can’t This Child See?

Addressing Issues of Poor Vision in Children

by Joanna Lee

Apart from trauma, among the broad reasons for poor vision in children include developmental causes and genetic or hereditary diseases. These are some of the topics that pediatric vitreoretinal surgeon Dr. Manoharan Shunmugam shared and discussed with ophthalmologists, opticians and optometrists in a recent webinar organized by OasisEye Specialists Centre in Kuala Lumpur, Malaysia.

Raise the red flags early

For preverbal children, several tests can be done to determine the cause of poor vision. “If the tests are not accessible, you should check for leukocoria — abnormal white reflection from the retina — to make sure the pupils are normal. If you find any reduced red reflex, then you have to refer them up,” shared Dr. Shunmugam, cautioning to check for asymmetrical pupils or relative afferent pupillary defects, all of which are warning signs and must be taken seriously.

“If a child has any exotropia, check that their reflection on the cornea is normal,” he said.

“If a child has any exotropia, check that their reflection on the cornea is normal.”

There are also intraocular malignancies such as retinoblastoma, a rare cancer most commonly found in children, that physicians should look for. “Should you see any lens subluxation, there’s a high likelihood they’ve had trauma or a significant syndrome that needs to be investigated further,” added Dr. Shunmugam. the eyelids or erythema around the periocular area, then you can suspect the child has been rubbing his eye a lot. Not only can it cause keratoconus but it can also lead to lens subluxation,” he warned.

Watch out for pediatric cataract

Pediatric cataract causes 10% of vision loss in children worldwide. It is important to treat it urgently — and if left untreated, visual deprivation can cause permanent amblyopia. About one in 250 children has this autosomal dominant (AD) condition. “Thus, it’s important to check family members to find out more information, especially if you refract the child and can’t get it down to 6/6 or 6/9,” he said.

“You might know this already, but unilateral cataracts tend to be more amblyogenic, and the posteriorly located cataracts are worse and it’s best

to treat them if the children are under two months old,” Dr. Shunmugam added.

Rarely would ophthalmologists need to look at non-accidental trauma like shaken baby syndrome. But if children have been involved in motor vehicle accidents, they can develop cataracts, as well as retinal detachments or hemorrhages.

The importance of pediatric eye screening

The incidence of hearing problems in babies is about 1 in 300; while the incidence of eye problems needing immediate attention is about 1 in 70 for children at birth.

“You hear about hearing tests for babies, but hardly do we hear of children having eye tests at birth. So, it’s far more worthwhile to screen their eyes,” said Dr. Shunmugam. Screenings are vital to detect several abnormalities in infants’ eyes.

During the webinar, Dr. Shunmugam also shared about BabySight, which helps provide pediatric digital examinations — from birth through the first year — which involves non-invasive RetCam Digital Imaging for retinal screening at birth to detect potential eye issues.

The dangers of retinal hemorrhages

“Childbirth is a highly traumatic event; when going through the birth canal, the baby goes through intracerebral pressure which increases pressure on vessels and nerves, sometimes causing bleeding,” said Dr. Shunmugam. Even in Caesarean-section births, there will be manipulations on the baby’s skull, which could also affect pressure.

Nine out of 10 times, these retinal hemorrhages are innocuous. But, if they continue to block the child’s vision, then they should be removed via vitrectomy.

“It’s interesting as we’re doing more studies, we’re coming to find out why some people are amblyopic where there has been no pathology found,” Dr. Shunmugam added. “Well, it could be traced back to childhood. That’s why we need to do screenings and follow-up until adulthood to see if they develop any visual deprivation.”

“If a young adult comes in with myopia, ask them if they’d been born prematurely. Chances are the answer would be positive.”

Retinopathy of prematurity

When a child is born prematurely and the oxygen levels are not maintained, vasculogenesis stops. “That’s where the trouble starts,” Dr. Shunmugam said. Retinopathy of prematurity (ROP) is found in babies born before 30 or 40 weeks of gestation, weighing less than 1,500 grams.

“If a young adult comes in with myopia, ask them if they’d been born prematurely. Chances are the answer would be positive,” Dr. Shunmugam said, adding that as younger and younger children survive premature births, the incidences of ROP are increasing as well.

“If a child is born early and his vasculature hasn’t fully developed, he is put into a room where there is more oxygen than in utero, so there’s a stimulus for vessels to grow. If you give them enough oxygen, the vessels stop growing,” Dr. Shunmugam explained.

He added: “But what happens is, the retina starts developing and the oxygen requirements of the retinal cells increases. So, as the retina is developing, there isn’t enough blood supply as the blood vessels never grew there. Then, the body tries to grow new blood vessels, which aren’t good because they grow on a sheet of fibrovascular tissue.”

At this stage, he said it can be stopped by using a laser on the ischemic retina or giving them a neonatal anti-VEGF dose. This sometimes allows the vessels to grow normally again. If that doesn’t work, there might be a risk of retinal detachment.

The increased use of digital devices has been correlated to increased incidences of myopia in East Asian children.

Screening and treatments of ROP

These children are not born with any abnormality of the retina, but rather an abnormality of the vasculature in the retina. “The retina still has a 100% potential to work normally. So, what we’re doing is to try to ensure normal vascularization,” explained Dr. Shunmugam.

Even with a 50% chance of success at stage 5 of ROP, it’s worth a try because if the child ends up being able to see some light or hand movements, it would help them with their diurnal rhythm. “If it is detected at stage 3 or 4, there’s an even better chance of helping them to see,” said Dr. Shunmugam.

“What’s more important after the surgery is visual rehabilitation. For children who have had any eye surgery, it’s important to ensure their refraction is checked more frequently. For babies, they need to be shown light. If they’re at stage 5, they’re usually left aphakic, so they’ll need adequate aphakic glasses,” he added

“If you have a child who comes in with 6/9, yet he can’t see as he often bumps into things, you’ve got to think about retinitis pigmentosa. It’s the most common retinal dystrophy and it’s a chronic, progressive, inherited disease with a broad spectrum of severity.”

Digital eye strain in children

Myopia is a common and increasing condition among children today, with a high (73%) prevalence rate in East Asia. 1 This has been associated with the use of digital devices and lack of time spent outdoors. 2 Not surprisingly, children in Africa and South America have a low prevalence (under 10%) of myopia. Eye strains, headaches, blurred vision and dry eye are common symptoms of computer vision syndrome or with the overuse of digital devices. Other characteristics include reduced attention span, poor behavior, and irritability in children aged below 10.

Retinitis pigmentosa and more ailments

“If you have a child who comes in with 6/9, yet he can’t see as he often bumps into things, you’ve got to think about retinitis pigmentosa. It’s the most common retinal dystrophy and it’s a chronic, progressive, inherited disease with a broad spectrum of severity,” Dr. Shunmugam added.

“Its onset could be from childhood right into adulthood, depending on the expression of genes involved. It’s usually in both eyes, and if you find it in only one eye, you have to think of possibilities like drusen or diffused unilateral subacute neuroretinitis (DUSN),” he said.

For treatment, they need to avoid the sunlight and be given orangetinted glasses to help with contrast. This might not always help their vision, but improving their contrast might help significantly.

As prevalent as FEVR

Familial exudative vitreoretinopathy (FEVR) is more prevalent than we thought it was. The signs are very subtle and it has a wide range of severity. Dr. Shunmugam pointed at its fundus fluorescein angiography (FFA), which would look normal but it may be seen at the temporal periphery — similar to ROP.

“For juvenile retinal detachment or in young adults who may not be myopic or have other trauma or syndromes, you should have a high suspicion for FEVR,” he said.

Apart from choroidal hemangioma, one of the interesting cases he mentioned was of a two-year-old boy who came in with bilateral detachment. “Now, this is where it’s important to check on the other family members,” Dr. Shunmugam said. Confirming his hunch, the boy’s older brother had a bilateral scleral buckle two years earlier. Upon further investigation, his 6-month-old brother also has the same condition. It turned out, they all have X-linked retinoschisis (XLRS), which is only seen in boys. It occurs in one out of 15,000 or 30,000, and it’s usually inherited from the mother, a carrier.

“If there’s only one message you can take home from this talk, it’s that it is important to do genetic counselling,” concluded Dr. Shunmugam. “Ensure that the other siblings are checked along with the family and extended family members, as they may be harboring similar conditions. These would be clues that could help detect eye conditions in the family’s future children.”

Contributing Doctor

Dr. Manoharan Shunmugam is a consultant ophthalmologist, adult and pediatric vitreoretinal surgeon who trained in the United Kingdom. Based in Malaysia since 2013, Dr. Shunmugam has a keen interest in research with publications in a wide range of high-impact journals and has been invited to many international conferences as a speaker. He is also a contributing author of two book chapters in vitreoretinal reference textbooks. He graduated in Scotland and subsequently undertook his ophthalmic specialist training and VR Fellowship in London. En route, he further honed his skills with a pediatric VR fellowship at the prestigious L.V. Prasad Eye Institute, Hyderabad, India – making him one of the few pediatric VR surgeons serving in the Asia-Pacific region. He is currently the director of clinical services at the flagship branch of OasisEye Specialists in Kuala Lumpur, a multi-subspeciality ambulatory eye centre in Malaysia. He continues to serve probono at Hospital Kuala Lumpur and is the honorary secretary of the Malaysian Society of Ophthalmology and is a member of the Asia-Pacific Vitreoretinal Society.

mano.shun@oasiseye.my

REFERENCES:

1 Grzybowski A, Kanclerz P, Tsubota K, et al. A review on the epidemiology of myopia in school children worldwide. BMC Ophthalmol. 2020;20(1):27.

2 Zadnik K, Mutti DO. Outdoor Activity Protects Against Childhood Myopia-Let the Sun Shine In. JAMA Pediatr. 2019;173(5):415-416.

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INDUSTRY UPDATE

New Myopia Progression Findings Presented at ARVO

As part of its comprehensive myopia research collaboration with Singapore Eye Research Institute (SERI) and the Singapore National Eye Centre (SNEC), Johnson & Johnson Vision recently presented new myopia progression findings from one of the largest studies with children in AsiaPacific at the recent virtual Association for Research in Vision and Ophthalmology (ARVO) Annual Meeting.

Data from five studies were presented, which focused on new techniques and practices to better predict myopia progression and vision impairment.

On progression, the team reported that in a study of 674 myopic children (aged 7-10) over two years, they found that the child’s prior year myopia progression correlates with the immediate, subsequent year’s progression. For example, children with slow progression in the first year, generally had slow progression in the second year. Meanwhile, those with faster progression in year one, also experienced faster progression in year two.

However, these findings were presented with the caveat that year-over-year progression does not fully predict longterm myopic progression, and that the child’s age and parental myopia are also important variables to consider in choosing whether to treat a child’s progression.

“Globally, for parents of children with myopia, and eye care professionals, an important takeaway is the need for myopic patients to receive regular annual check-ups,” said Study Lead Author Noel Brennan, Ph.D., global lead, Myopia Control, Johnson & Johnson Vision. “While myopia progression rates will naturally be followed from year-toyear, it is also important that eye care professionals consider the age of myopia onset and parental myopia.”

Xiao-Yu Song, M.D., Ph.D., global head of research and development, Johnson & Johnson Vision Care, added: “Through close collaborations with SERI, SNEC, and other aligned public health groups, we are helping to address this crisis by building a better understanding of the science and biology behind myopia and developing comprehensive programs to address and treat the disease.”