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Retinal Diseases in Children - A Complete Guide

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Certain abnormalities can be present in the retina of some children. Continue reading to learn more about retinal diseases in children.

Written by

Dr. Syed Shafaq

Medically reviewed by

Dr. Veerabhadrudu Kuncham

Published At February 22, 2024
Reviewed AtApril 4, 2024

Introduction

Many proliferation-related abnormalities and exudative retinal diseases are common in pediatric patients. The most commonly encountered abnormalities are FEVR (familial exudative vitreoretinopathy), retinopathy of prematurity, and Cots disease. A complete eye examination is essential and should be done by an experienced physician or ophthalmologist. Early diagnosis and treatment at the proper time are very important as the retina can get detached from larger eye areas with advancing age.

Various imaging technologies also support the diagnosis, such as fundus photography, which has been popular for decades. It gives objective documentation of the fundus and monitors the disease's progression. The Fluorescein angiography is also helpful in making a proper diagnosis. This can help diagnose any vascular leakage, nonperfusion, and abnormality in microvascularization. Traditional methods of imaging the peripheral retina are challenging because of the need for high-level expertise.

Mediator of Pathology: Vascular endothelial growth factor (VEGF) is considered an important mediator for pathological changes in the eyes, affecting neovascularization and the vascular permeability of the retina. The treatment, including intraviteral therapy of the VEGF, has revolutionized the treatment of abnormalities related to the retina.

In cases such as retinopathy of prematurity (ROP), VEGF is considered responsible for the pathological changes, and treatment with anti-VEGF during the development of phase 2 of ROP gives amazing results. This targeting of the VEGF for treating retinal diseases, mostly the posterior part, has drastically changed the era of retinal diseases in children. The intraviteral injections and suppression of the VEGF for the short term are very beneficial for the treatment of retinal eye diseases in children. The systemic VEGF normally is responsible for the neurodevelopment of an individual in the early stages of life. It is essential to know the long-term effects of using anti-VEGF and how suppressing the vEGF in children can affect their neurodevelopment.

How Can Retinal Detachment Affect a Child's Vision?

The most common retinal detachments in children include rhegmatogenous retinal detachments (RRD), tractional retinal detachment, and exudative retinal detachment.

1. Rhegmatogenous Retinal Detachments: This can be caused due to traumatic or nontraumatic reasons. The nontraumatic include myopia, congenital abnormalities, nonhereditary abnormalities, and any previous intraocular surgeries. Delayed diagnosis is the leading cause. During diagnosis, macular involvement and proliferative vitreoretinopathy are seen during the presentation. Bilateral involvement is also seen in pediatric patients.

2. Retinal Detachments in Congenital Abnormalities: Congenital anomalies are associated with more than 50 % of RRDs, primarily affecting children up to 10 years of age. The most common congenital anomaly causing retinopathies is Stickler syndrome. In these cases, vitreous fluid is abnormal, and peripheral retinal changes are also seen. Stickler syndrome combines facial, ocular, palatal, and skeletal abnormalities. The main features include cleft palate, flat face, and hearing loss.

What Are the Symptoms of Retinal Abnormalities?

In conditions such as Sticler's syndrome, post-anterior and posterior segments are involved. Cataracts set in at a very early stage. The opacity of a lens that is wedge-shaped is usually symmetrical bilaterally. Myopia with thinning of peripheral retina and breaks seen in the retina. A giant retinal tear can also be seen. The vitreous gel is optically empty, and a fibrillar membrane extends to the peripheral retina. RDD is the most common and severe complication. This is usually present early in life. Children below ten years of age are mostly affected. Examining both eyes is critical so that prophylactic treatment may be started in the unaffected eye if one side is affected.

In Marphans syndrome, nontraumatic ectopia lentils are a very common ocular presentation. The subluxation is present at the superotemporal meridian. The patient presents with poorly dilating pupils and has transillumination of the iris. Usually, the effect is seen bilaterally, and prophylactic treatment is started in the fellow eye. A giant retinal tear is seen in retinal detachment. In Marfan syndrome, there is no abnormality seen in the vitreous gel.

What Is the Treatment of Retinal Detachments?

1. Congenital Abnormalities (Colobomas): Repair of colobomas associated with retinal detachments are treated surgically, including the case of optic nerve involvement and other ocular abnormalities that are associated with it, such as cataracts, lens coloboma, and microphthalmia. In the case of retinal detachment without the involvement of coloboma surgical treatment still remains the option, and the principles are also the same.

2. Coats Disease: It can be treated to preserve the globe and the vision. The goal is to eradicate all abnormal vessels and the nonperfusion areas so that there is a reduction in further exudation and the progression of disease retards. If the patient is in phase one, only observation is needed, but in phase two, there is laser ablation of aneurysms and further reduction in exudation. In early diagnosis, the results can be amazing. In a study in the United States, patients with Coats disease were treated with laser photocoagulation, which is successful in almost 92 percent of cases. In severe cases, surgical repair is the only option, and the results are not very favorable with laser or cryotherapy.

3. Anti-VEGF Injections: Intraviteral triamcinolone and anti-VEGF injections are a therapy to improve anatomic and visual results. Improvement in visual outcome and reduction in exudate. In a few cases, the development of vitreous hemorrhage is seen in children after the anti-VEGF injections. This was because of increased tractional forces.

4. Surgical Option: Surgery is the only choice in some cases. It usually includes vitrectomy, scleral buckle, or sometimes a combination of both procedures. In retinal detachment, scleral buckling is done and can also be treated with vitrectomy when indicated. Bimanual techniques using a cutter and forceps are usually preferred by dissecting the vitreous membrane detaching from the retinal surface. Children who show symptoms at three years of age usually do not have a good prognosis.

Conclusion

The treatment of retinal disease is usually complicated, and surgical treatment with experience gives a successful result. The postoperative period is more extended in children than adults, and close management is required. Anxious patients are challenging to treat, but follow-ups are essential for the progress of treatment and results.

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Dr. Veerabhadrudu Kuncham
Dr. Veerabhadrudu Kuncham

Pediatrics

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