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PEDIATRIC RETINAL DISEASES

Structure of human eye. In side view..jpg

INTRODUCTION: WHAT IS THE RETINA?

The eye functions much like an analog camera, capturing light that passes through the transparent cornea and lens to project an image onto the inner back wall of the eye. This area, known as the retina, is a thin layer of tissue composed of a variety of highly specialized cells. Among these cells, photoreceptors play a crucial role in converting light into electrical signals.

The human retina contains two types of photoreceptors: rods and cones. Cones are responsible for our detailed central color vision, enabling us to see vibrant hues clearly. In contrast, rods are sensitive to low light and allow us to perceive shades of gray, contributing significantly to our peripheral vision.

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When light activates these cells, the information is transformed into electrical signals, which after processing are transmitted to the brain through the optic nerve - a bundle of nerve fibers originating from the retina. Upon reaching the occipital lobe at the back of the brain, these signals are processed, allowing us to perceive and interpret the world through sight.

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The eye is an extraordinarily complex organ, making it vulnerable to changes that can affect the retinal tissue or its supporting structures, such as the blood vessels that deliver essential nutrients. While retinal diseases vary widely, most can lead to visual impairment over time. Some of the most common retinal diseases and conditions include:

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  • Retinal tears and retinal detachment

  • Epiretinal membrane

  • Macular hole

  • Macular degeneration

  • Diabetic retinopathy

  • Retinal vascular occlusion or thrombosis

  • Inherited retinal diseases

 

Although the majority of retinal diseases typically develop in adulthood, several severe conditions can affect children, starting from birth. Among the most common pediatric retinal diseases are:

  • Retinopathy of Prematurity (ROP)

  • Persistent Fetal Vasculature Syndrome (PFVS)

  • Coats' disease

  • Familial Exudative Vitreoretinopathy (FEVR)

  • Incontinentia Pigmenti

  • Norrie disease

  • Congenital X-linked Retinoschisis (CXLRS)

  • Chorioretinal choloboma

  • Optic Nerve Head-related maculopathy

  • Marfan, Stickler and Wagner syndromes

  • Traumatic retinal detachment

  • Pediatric uveitis

 

Continue reading on the next pages if you wish to learn more about pediatric retinal diseases.

Image by Tanaphong Toochinda

COATS DISEASE

Coats is a rare progressive eye disorder that is characterized by abnormal blood vessel in the outer part of the retina. The affliction causes the small blood vessels to leak fluid or blood under, inside or above the retina. As the fluid builds up, the retina swells until it finally detaches from the wall of the eye. Left untreated, the retinal detachment results in loss of vision and eventually blindness. 


It is a disease that affects mostly young males (by a male:female ratio of 3:1). Most are diagnosed between the ages of 4 to 6 years, although up to a third are diagnosed after the age of 30 when symptoms start. Per date, the exact cause of Coats disease remains unknown, with some genes implicated in selected cases. 


Coats disease can be divided into 5 stages of disease progression. In general, the earlier the disease is diagnosed and the correct treatment initiated, the better the outcome will be. Regular check-ups and treatments by an expert in pediatric retina will help maintain good vision.


Coats disease when not treated can cause severe complications, such as cataract (clouding of the lens) and intractable glaucoma (elevated eye pressure), and can end up in some cases in a blind and painful eye that needs to be removed.


Symptoms of Coats disease include in younger children: 

  • Abnormal white or yellow light reflected from the pupils (often spotted in pictures taken with flash – “leukocoria”)

  • Strabismus (misalignment of the eyes - usually with an outward crossing eye)

  • Decreased or blurry vision

  • Redness or eye pain


Older children or adults can also notice: 

  • Decreased depth perception

  • Loss of peripheral vision

  • Seeing floaters, spots or “flies”

  • Seeing double

  • Trouble seeing at night

  • Change in the color of the iris 


How is it diagnosed?

To check the health of the retina (the back-segment of the eye) and look for any changes to the blood vessels, the retinal specialist will perform a dilated eye exam using drops that widen the pupil. The doctor will also check the rest of the eye to control for other eye conditions. The doctor might also use other non-invasive imaging techniques such as ultrasound of the eye and optical coherence tomography (OCT). Based on the results the doctor can assess which stage the disease is at and administer the appropriate treatment. In case of children that cannot be fully examined at the office, an examination under anesthesia (EUA) will be proposed.


Treatment can include: 

  • Laser treatment (aimed at constricting the abnormal blood vessels and stopping fluid leaks) – the mainstay of treatment for Coats disease

  • Anti-VEGF injection therapy (anti-vascular endothelial growth factor medication given by direct injection to the eye to reduce fluid leaks) – an adjunct to laser treatment

  • Surgery (to remove excessive fluid detaching the retina, as well as scar tissue preventing the retina from reattaching to the eye wall)

  • Glasses and eye patches (in the pediatric age group in order to treat amblyopia/lazy eye)

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