Vision

Evidence 

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5806224/ 

P Vijayalakshmi and Clare Gilbert 

Preterm babies, and newborns who are unwell, are now surviving at higher rates globally than ever before. This is the result of expansion and improvement in services for sick and preterm babies. However, preterm birth is associated with a range of complications, including retinopathy of prematurity (ROP), and preterm infants are at a far higher risk of disabilities – including blindness – than healthy, full-term babies. Clinicians, together with low vision and rehabilitation specialists, can play a key role in reducing visual impairment and promoting normal development in this group of children. 

Education 

Children who are born prematurely are more likely to have vision impairment than children who were born at term. They’re more likely to develop mild visual problems like short-sightedness or long-sightedness, a squint, reduced contrast sensitivity or problems with depth perception. 

Most severe eye problems are picked up very early. Very premature babies have regular eye tests while they’re in hospital to check for the most serious causes of vision impairment due to prematurity – Retinopathy of Prematurity - which sometimes requires treatment during the hospital admission. 

Eye and Vision Problems 

The most common eye problems to appear after discharge are: 

• Poor vision due to refractive error (either near sighted or far sighted) 

• Strabismus (inward or outward turning of one or both eyes) 

• Amblyopia (unequal vision) 

• Nystagmus (constant or frequent jerking movements of the eye) 

• Decreased colour vision (some colours look alike, for example purple, blue, green) 

• Smaller field of vision 

Although the above listed problems are more common in infants who have had Retinopathy of Prematurity, they can occur in other premature infants as well. 

Retinopathy of Prematurity (ROP)

Retinopathy of Prematurity (ROP) is an eye disorder that primarily affects premature infants where the risk significantly increases with babies before 32 weeks gestation or with a very low birth weight (less than 1250 grams). The smaller and more premature a baby is at birth, the more likely that baby is to develop ROP. 

ROP occurs when abnormal blood vessels grow and spread throughout part of the retina which is the tissue that lines the back of the eye. These abnormal blood vessels are fragile and can leak, scarring the retina and pulling it out of position. This causes a retinal detachment. Retinal detachment is the main cause of permanent visual impairment and blindness in ROP. 

Scientists believe that ROP occurs because the periphery of the premature retina sends out signals to other areas of the retina for nourishment. As a result, new abnormal vessels begin to grow. These new blood vessels are fragile and weak and can bleed, leading to retinal scarring. When these scars shrink, they pull on the retina, causing it to detach from the back of the eye.  

ROP is classified in five stages, ranging from mild (stage I) to severe (stage V): 

• Stage I — Mildly abnormal blood vessel growth 

• Stage II — Moderately abnormal blood vessel growth 

• Stage III — Severely abnormal blood vessel growth 

• Stage IV — Partially detached retina 

• Stage V — Completely detached retina and the end stage of the disease 

How is ROP diagnosed?  

All babies weighing less than 1250 grams at birth or less than 32 weeks of gestation will have regular eye examinations after their gestational age reaches approximately 30 to 31 weeks. These examinations are undertaken by a medical eye specialist known as an Ophthalmologist. To look at the back of a baby's eyes the pupil (black circle in the middle of the coloured part at the front of the eye) needs to be dilated. Eye drops are used to dilate the pupils. The examination only takes a couple of minutes. Your baby may be minimally upset by these examinations and will settle very quickly once the eyes have been checked. 

ROP Treatment 

An Ophthalmologist who examines a baby for ROP will know when to watch the condition and when to treat it, based on guidelines. Treatment may include: 

• Medications injected into the eye. These medications, called anti-VEGF agents, switch off abnormal blood vessel growth and have been shown to be effective in several forms of ROP. 

• Retinal laser photocoagulation (laser therapy) to stop the growth of blood vessels and keep the retina attached to the back of their eye. 

• Less common, surgery is required. 

• Scleral buckling, where the doctor places a band around your child’s eye. This pushes it inward, which helps keep the retina in place against the walls of the eye. The band will be removed in a few months or years. 

• Vitrectomy, a more involved surgery. The doctor replaces the vitreous fluid inside your child’s eye with a saline solution. Then they remove any scar tissue from inside the eye. This allows the retina to relax in place against the eye wall. 

Early treatment can help preserve central vision, which lets your child see straight ahead, read, see colours, and drive. Some of these procedures can lead to a loss of side vision. 

Complications 

Infants with ROP are considered to be at higher risk for developing certain eye problems later in life, such as: 

• Myopia (near-sightedness) 

• Strabismus (misalignment of the eyes) 

• Amblyopia (lazy eye) 

• Glaucoma 

• Retinal detachment 

Your child will need regular eye exams by a specialist as often as they recommend. If the doctor catches them early on, they can treat most of these conditions without any vision loss. 

Read more about ROP here: 

https://www.rch.org.au/ophthal/patient_information/what_is_rop/ 

https://www.nei.nih.gov/learn-about-eye-health/eye-conditions-and-diseases/retinopathy-prematurity 

https://www.miraclebabies.org.au/content/retinopathy-of-prematurity-rop/gk6cag 

Myopia 

Short-sightedness (also known as myopia) is a common eye condition that causes distant objects to appear blurred. Someone with myopia can achieve good vision with prescription glasses, contact lenses or in adulthood, laser surgery may be appropriate. Short-sightedness usually first appears in childhood.  

Read more about Myopia here: 

https://www.lei.org.au/services/eye-health-information/myopia/ 

Amblyopia 

Amblyopia or "lazy eye" is reduced vision in one eye caused by abnormal visual development early in life. The weaker or lazy eye often wanders inward or outward. 

Amblyopia generally develops from birth up to age 7 years. It is the leading cause of decreased vision among children. Rarely, lazy eye affects both eyes. 

Early diagnosis and treatment can help prevent long-term problems with your child's vision. The eye with poorer vision can usually be corrected with glasses or contact lenses, or patching therapy. 

Read more about Amblyopia here: 

https://www.lei.org.au/services/eye-health-information/amblyopia-lazy-eye/ 

Glaucoma 

Glaucoma rarely affects infants. It is estimated that 1 in 5,000 to 10,000 children under 2 years of age will develop glaucoma. Glaucoma in children under 2 years of age is called infantile (or congenital) glaucoma. Prompt recognition and timely treatment will improve the chance of a good outcome.  

Infantile glaucoma is the result of failure of fluid to escape from the anterior chamber of the eye resulting in increased pressure within the eye. The young eye responds to this increased pressure differently to an adult eye. 

Initial treatment may be eye drops or medication by mouth to lower the pressure in the eye. Over the long-term medications have a significant risk of complication in young children and compliance with medical therapy is an even greater problem with young patients than it is with older ones. Surgery is usually required and has a very high success rate. 

Read more about Glaucoma here: 

https://www.rch.org.au/ophthal/patient_information/Glaucoma/#:~:text=Glaucoma%20in%20Infants,chance%20of%20a%20good%20outcome. 

Retinal detachment 

Retinal detachment is a condition of the eye in which the retina (a thin membrane consisting of rods and cones and nerve fibres at the back of the eye) separates from the underlying tissue. 

Paediatric retinal detachment (PRD) is rare and challenging. Early diagnosis can be difficult as children may not necessarily recognise or appreciate the symptoms such as a sudden increase in floaters, flashes of light, or changes in their field of vision. As a result, they may not raise the alarm that something in their vision has changed.  

It is very important that if your child has been diagnosed with an eye condition especially ROP to have regular check-ups with your optometrist. 

Read more about Retinal detachment here: 

https://www.healthdirect.gov.au/retinal-detachment 

Strabismus 

A small number of children will get strabismus -- eyes that don’t line up like they should. Infantile esotropia (one eye turning inward) is a type that affects premature babies. When the muscles that surround and control the eye don't work together, your child’s eyes will point in different directions. Doctors think it happens to premature babies, because the baby's brain hasn't developed enough to control their eye muscles. Other factors play into it as well: 

• Brain or nerve problems like water on the brain, bleeding in the brain, seizure disorders, cerebral palsy, and other conditions 

• Retinal damage from ROP 

• Brain or eye tumours 

• A cataract or eye injury 

• Developmental delay 

• Genetic disorders 

Read more about Strabismus here: 

https://www.childrens.health.qld.gov.au/fact-sheet-strabismus/#:~:text=It%20is%20normal%20for%20a,serious%20eye%20and%20health%20conditions.

Conjunctivitis 

Conjunctivitis is a disease that occurs mainly in babies and toddlers due to viral and bacterial infections.  

Babies and Toddlers with conjunctivitis need a gentle massage with warm water to gently clean the eyelids and eyelashes. Use cooled boiled water to gently wipe your baby's eyelids, 2-3 times a day if affected. If condition does not improve it is important to see a doctor as they may need to prescribe antibiotics to treat the infection. 

Read more about Conjunctivitis here: 

https://raisingchildren.net.au/guides/a-z-health-reference/conjunctivitis?gclid=CjwKCAjwxOymBhAFEiwAnodBLI_ogUrndUmSGUXbAkOGMFe9tyfeQDdIurx4vZx2c6-1n8FL1XQrxBoCMsIQAvD_BwE 

Obstruction of tear ducts 

Blockage of the tear duct (nasolacrimal duct) is a very common eye disease in infants. Children with blocked tear ducts will have a lot of tears, because there is an obstruction in the drain that removes tears from the eye. In the early days after birth, it is often difficult to detect whether a child has a blocked tear duct or not, and after more than a month of age, clearer signs begin to appear. 

Caregivers can often apply pressure over the lacrimal sac, which is at the point where the upper and lower eyelid come together next to the bridge of the nose. In the case the child has a severe blocked tear duct, it is necessary to take the child to an ophthalmologist for appropriate examination and treatment. 

Read more about blocked tear ducts here: 

https://raisingchildren.net.au/guides/a-z-health-reference/blocked-tear-duct?gclid=CjwKCAjwxOymBhAFEiwAnodBLCrzbvAd0uIuePdc0fO1DJC5m0hVdmhd3Utara51e4JhPoXyzi4BERoC6jwQAvD_BwE 

Cataracts 

A cataract is when the lens of the eye is cloudy instead of clear, making it hard to see. The lens is the tissue inside your eye that helps focus the light coming into your eye. Cataracts can happen in one or both eyes. If cataracts aren’t treated early, they can cause serious vision problems or blindness. 

 There are two kinds of cataracts: 

• Syndromic. This is when congenital cataracts happen along with other birth defects or health conditions. For example, some babies with congenital cataracts also have Down syndrome. Down syndrome is a genetic condition that includes a combination of birth defects, such as intellectual and developmental disabilities, heart defects, certain facial features, and hearing and vision problems. 

• Non-syndromic. This is when congenital cataracts are the only condition a baby has. 

Babies with cataracts need early and regular care from a paediatric ophthalmologist. Early treatment is important, because it can prevent vision loss and help your baby’s eyes develop properly. Treatment depends on whether cataracts affect a baby’s vision. A baby with small cataracts that don’t affect their vision may just need regular checkups with a paediatric ophthalmologist, especially if the cataracts are in both eyes. But most babies with cataracts need special treatment, which may include: 

Surgery. Most babies with congenital cataracts need surgery. During surgery, a paediatric ophthalmologist makes a small cut in your baby’s eye and removes the cloudy lens. Some babies with cataracts may need additional surgery. 

A new type of lens. After cataract surgery, your baby needs a new type of lens to help them see. This may include: 

Contact lenses. Special contact lenses can be used to help your baby see after cataracts are removed from one or both eyes. 

Intraocular lens. This is a special plastic lens that a surgeon places in your baby’s eye during surgery. It replaces the original lens. This surgery usually is done at age 1 or later. 

Many babies need to wear glasses together with a contact lens or an intraocular lens. Some babies need glasses only, especially if they had cataracts removed from both eyes and they have trouble wearing contact lenses. 

Patch therapy - Your baby may need patch therapy if their eyesight is better in one eye than in the other. In patch therapy, you place an eye patch over your baby’s healthier eye to strengthen the weaker eye. Your baby’s provider recommends how many hours your baby wears the patch each day. Patch therapy trains your baby’s brain to send signals to the weaker eye to improve his eyesight. This can prevent or treat amblyopia (also called lazy eye). Babies with cataracts may have patch therapy until their eyes are fully developed, which happens around the age of eight. 

Early intervention services - Babies with cataracts who have vision problems may need special services to help them learn and develop. Getting early intervention services as soon as possible can help improve your baby’s development as they grow. These services can help children from birth through to 3 years old, learn important skills. Services include therapy to help a child talk, walk, learn self-help skills, and interact with others. Babies and children with vision problems also may need orientation and mobility (also called O&M) training as part of early intervention services. O&M training helps children use their other senses to learn about their environment and learn how to move around safely.  

Read more about Cataracts here: 

https://www.cataractkids.org.au/cataracts-and-their-treatment.html 

Optic Nerve Hypoplasia 

Optic nerve hypoplasia is a congenital condition that develops during the first trimester of pregnancy. ONH affects about one in 10,000 children. 

Babies born with optic nerve hypoplasia have an underdeveloped optic nerve, the nerve that sends information from the eye to the brain. If the optic nerve is underdeveloped, it can inhibit the proper development of vision. The effect of ONH on vision can range from mild to severe.  

Development of children with ONH may also be affected by issues with the brain, central nervous system and endocrine system.  

In some cases, ONH may be known by two other names: 

• DeMorsier's Syndrome 

• Septo-Optic Dysplasia 

ONH can result in developmental delays and a range of health problems related to deficiencies of key hormones. And, while optic nerve hypoplasia can lead to decreased vision, there are many cases where the presence of ONH has little to no negative effect on vision. 

How does optic nerve hypoplasia affect vision? 

The effects of ONH on vision vary based on the patient and can range from the inability to detect light to fully functioning vision. Other vision problems that may present in patients with ONH include:  

Nystagmus – An involuntary rhythmic shaking of the eyes, nystagmus makes it hard to focus on an object. The presence of nystagmus typically reduces one's vision. Congenital nystagmus typically decreases as the child ages.  

Strabismus – More commonly known as “crossed eyes,” strabismus prevents both eyes from working together properly. Strabismus often develops in an eye with reduced vision. If ONH results in reduced vision, the affected eye may “cross or turn.” 

Optic nerve hypoplasia symptoms 

The detection of optic nerve hypoplasia may vary based on the child. The first signs parents may notice in infants may include: 

• Rapid, involuntary eye movements (nystagmus) 

• Signs of vision impairment in one or both eyes 

• Delays in development of some motor skills  

• Strabismus (crossed eyes) 

Some ONH patients may also have problems with the pituitary gland, which produces and releases hormones. The specific symptoms will depend on the exact hormonal problems. Hormones from the pituitary gland play a major role in blood sugar regulation, growth, metabolism and other important bodily functions. 

An eye doctor may be able to diagnose optic nerve hypoplasia with a comprehensive eye exam. 

During the exam, the eye doctor will use drops to dilate the child's eyes before using a special magnifying lens to evaluate the optic nerve and retina. During this part of the exam, the eye doctor should be able to see a smaller-than-normal optic disc that may look pale or grey in colour. 

Based on the results of this exam, the child may also need further diagnostic tests such as:  

• MRI – Magnetic resonance imaging (MRI) is a type of imaging that can show the diameter of the optic nerve and detailed images of the brain. 

• OCT – Optical coherence tomography (OCT) is a painless type of imaging similar to an ultrasound that allows an eye doctor to get a detailed map of the optic nerve. 

• Blood work – Blood tests may be ordered to determine cortisol and growth hormone levels.  

These procedures can help an eye doctor make a definitive diagnosis and get a better picture of the optic nerve, other structures in the eyes, and how the body as a whole is affected. 

Optic nerve hypoplasia Treatment 

There are no medications or surgical treatments approved for treatment of optic nerve hypoplasia. However, a child with significant vision issues may benefit from working with a low vision specialist to get support and proper accommodations to help them comfortably complete daily tasks. The specialist may recommend tools such as magnifiers and computer software to make daily tasks easier. 

A patient who has optic nerve hypoplasia along with brain and hormone problems may need to get treatment from an experienced team of doctors that includes a paediatrician, an ophthalmologist, an orthoptist, a neurologist and an endocrinologist. Depending on the specific case, treatment may include hormone replacement therapy. 

Read more about Optic Nerve Hypoplasia here: 

https://www.aao.org/eyenet/article/optic-nerve-hypoplasia-9 

Persistent Hyperplastic Primary Vitreous 

Persistent Hyperplastic Primary Vitreous (PHPV) also known as Persistent Fetal Vasculature (PFV), is a condition in which the eye does not fully develop during pregnancy. Specifically, the primary vitreous, a structure that supports the lens of the eye, does not disappear as it should and continues to grow. It is the most common cause of unilateral cataract (cataract affecting only one eye) and in severe forms may be accompanied by a retinal detachment. 

What Causes PHPV? 

Doctors aren’t exactly sure why this happens. Most of the time it occurs by chance, and it’s not something parents did or could have prevented. Sometimes it can be linked to other rare eye conditions or genetic changes, but usually it happens on its own.  

How Does PHPV Affect Vision? 

PHPV can affect vision in different ways depending on the severity of the condition. In some cases, the affected eye may appear cloudy or have a white mass in the pupil. This can cause a reduction in vision or even complete blindness in the affected eye. 

In other cases, the affected eye may have a distorted shape, or the lens may be displaced, causing double vision or blurry vision. The degree of vision impairment depends on how much of the eye is affected and whether the condition affects one or both eyes.  

What are the signs of PHPV? 

The affected eye may appear white or cloudy, as if there is a film over the pupil. This is due to the persistence of a fetal structure called the primary vitreous, which normally disappears before birth. Additionally, the affected eye may have an abnormal shape, or the lens may be displaced. Other signs of PHPV can include a squint or misalignment of the eyes, or an inability to track objects with the eyes. PHPV can affect one or both eyes and is often detected during a routine eye exam or when a child is experiencing vision problems. 

How is PHPV Treated? 

Treatment depends on the severity of the condition and the extent to which it is affecting the child's vision. In some cases, no treatment is required. However, if the vision is significantly impaired, treatment options may include: 

Patching: If the PHPV only occurs in one eye, or one eye is more affected than the other, the brain will rely on the eye with the clearer vision to see. This prevents the visual pathways in the affected eye from developing and can lead to Amblyopia. To treat this, we patch the eye with clearer vision so that the weaker eye is given the opportunity to strengthen.  

Surgery: If the PHPV is causing a significant reduction in vision, surgery may be required to correct the abnormality in the eye. This may involve removing a cataract, or other procedures to restore vision. 

It is important to seek treatment for PHPV as early as possible to prevent further vision loss and to ensure the best possible outcome. Regular follow-up appointments with an ophthalmologist are also important to monitor the child's vision and ensure that any necessary interventions are provided. 

Read more about PHPV here:  

https://www.institut-vision.org/en/health/28-diseases/98-persistent-hyperplastic-primary-vitreous.html?showall=1&limitstart= 

How will I know if my child has an eye problem? 

If your child develops any of the following, bring these to the attention of your child’s doctor. Your infant may need to see an ophthalmologist (eye doctor). 

• Infant frequently pokes at his/her eye

• Lazy eye, slow to move 

• Constant movement of eyes, even when trying to look at something 

• Frequent crossing of eyes, beyond three months of age correcting for prematurity 

• One eye that stays out or in most of the time 

• Frequent or constant jerking of eyes especially when looking straight ahead. A few jerks when looking to the far left or far right is normal. 

• The infant fails to blink to a camera flash just in front of the face 

• The surface of the eye or the pupil (black circle in the centre of the eye) appears cloudy or white 

• In a photo, the centre of one eye appears red while the other eye does not 

• Abnormal head turn or head tilt 

• The infant (beyond 6 weeks of age correcting for the weeks of prematurity) cannot fix his/her eyes on an object or a face, or follow it as it moves 

• The infant’s eyelid droops so much that it completely covers the pupil (black centre) of the eye when he/she is awake 

• Normal lighting seems to hurt the baby’s eyes and make him/her cry or turn away

Empowerment 

Early intervention is the best way to support your child’s development. Early intervention includes therapies, education and other supports that will help your child reach their full potential. 

Early intervention should also include helping you learn how to bond with your child and interact with them in ways that support their development. Children learn the most from the people who care for them and with whom they spend most of their time, so everyday bonding, play and communication with you can help your child a lot. 

There are several specialists who are trained to work specifically with children who have vision problems. These might include paediatric ophthalmologists, orthoptists, physiotherapists, orientation and mobility specialists, occupational therapists, counsellors, and special education teachers. 

Useful links 

Miracle Babies Foundation 

https://www.miraclebabies.org.au/content/eye-health/gk3u0g 

Cataract Kids Australia 

https://www.cataractkids.org.au/glossary.html 

Lions Eye Institute  

https://www.lei.org.au/ 

Vision Australia  

https://www.visionaustralia.org/ 

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