In part one (Click here) of this two-part article, I provided tips to facilitate the pediatric eye exam. Here, in part two, I discuss the optometrist’s role in treating the most prevalent pediatric conditions effectively. These are: Refractive error, strabismus, amblyopia, congenital cataract, retinoblastoma, retinopathy of prematurity, nystagmus, Stargardts, and color blindness.
Refractive error
This is the easiest to treat, but sometimes the hardest to diagnose, as pediatric patients have no point of reference for “clear” vision, and they can have difficulty verbalizing that they are not seeing well.
To facilitate diagnosis, I suggest asking the young patient and/or their caregiver whether there is sensitivity to the sun, complaints of headaches, the holding of items abnormally close, or difficulty with eye-hand coordination.
It is important to note that in the pediatric population, your prescription can have a large effect on the patient's visual development and future refractive error. Therefore, I recommended you prescribe the least amount of power to have the most impact on the visual system, while considering other factors, like binocularity, accommodation, the natural course of emmetropization, and the visual goals for your patient.
Prescribing for pediatric patients is truly an art and can feel overwhelming at times. If you are new to this population, I highly recommend Dr. Suzanne Leat, from the School of Optometry Waterloo’s, guideline for spectacle prescribing in infants and children. See the guideline here!
Strabismus
This condition, whether it be exotropia, esotropia, or hyper/hypotropia, is often obvious to the pediatric patient’s caregivers. Accommodative esotropia is a common type, estimated at 1% to 2% of the population with no sex or race predilection.1 It can present at any time between ages of four months and six years old, with most presenting between the ages of two and three. What happens is that the pediatric patient’s caregiver starts to notice the eye turning inward while the child is playing or reading. The condition may come on suddenly, slowly, or after the child is sick.
Treatment considerations are dependent on the frequency, size, and consistency of the strabismus. That said, treatment options are compensatory prism, vision therapy, surgery, or a combination thereof, based on an assessment of the induced refractive error and how it affects the eye turn.
Of note, a child can have a non-strabismic binocular dysfunction, such as a convergence insufficiency or accommodative insufficiency, that can be managed through lenses, prisms or vision therapy.
Amblyopia
This is a monocular manifestation of a binocular disorder with much more areas of deficit than just reduced VA.2 Identifying the etiology of amblyopia (refractive vs. strabismus vs. deprivation) determines the course of treatment. Treatment includes immediate proper refractive correction (if indicated), patching, and implementing a binocular approach to developing visual acuity, and other parts of the visual system (accommodation, oculomotor skills, depth perception, etc.) through activities, such as monocular fixation in a binocular field.3 The Pediatric Eye Disease Investigator Group (PEDIG) has done extensive research on the benefits of these treatments for children up to age 17,4 but clinically we know that treatment can extend far beyond that age.
Congenital cataract
This can be unilateral or bilateral, occur in three to four infants out of 10,000 births, and is caused by genetics, metabolic disorders, trauma, and maternal infection.5 After surgical treatment, which must occur within the pediatric patient’s first year of life to help preserve visual development, the optometrist should provide contact lenses to correct vision post-surgically in the aphakic patient. Spectacles are often not ideal due to the need for high-powered lenses that may result in distortions or aniseokonia in the case of unilateral cataracts. IOL placement for congenital cataracts is controversial at this point, though secondary IOL placement can occur when the child is older. This is where biometry would come in for the assessment of IOL power calculation.
Retinoblastoma
This cancer of the retina most commonly affects children younger than age five, with approximately 300 children in the United States affected.6 The two classic signs of retinoblastoma are leukocoria or a “white pupil” (often seen in photos) or strabismus. This cancer can be genetic or idiopathic and may be unilateral or bilateral. Prognosis is dependent on a multitude of factors, but early detection and quick assessment are key in the management of this disease.7
The OD’s role here is to manage the aftereffects of chemotherapy/radiation/surgery. As an example, the child may be left with reduced vision or complete enucleation, so the role would be to provide all functional vision recommendations and low vision support when necessary.
Retinopathy of prematurity
Retinopathy of prematurity (ROP) occurs from the abnormal development of blood vessels in the retina and is a direct result of premature birth (< 36 weeks gestation) or low birth weight (<1.5kg/3.3lbs). The overall prevalence of ROP is 31.9% with severe ROP affecting 7.5% of premature infants.7 ROP can be classified into five categories and dictates the appropriate management of these patients.8 Depending on modality of practice, optometrists will often comanage with ophthalmological colleagues on these cases.
Follow-up visits can range from weekly to monthly appointments during the early phases of ROP, monitoring the retina closely for changes in status. As the retina becomes more perfused, the spacing of the exams will extend out. As for the refractive and strabismic interventions, these babies follow the same protocols as their full-term equivalents.
Nystagmus
Nystagmus is a condition that causes involuntary eye movements in infants and children. Determining the etiology of this condition includes ruling out albinism, retinal dystrophies, congenital cataracts or neurological issues. In the absence of any of these causes, the baby will be diagnosed with infantile idiopathic nystagmus. This type of nystagmus, found in one in 821 births, often does not go away, but can lessen.9
A specific type of nystagmus that usually presents in the first two years of life with head bobbing, and torticollis, is referred to as Spasm Nutans. This type of nystagmus often lessens or can be eliminated over time but may have other comorbid findings, such as amblyopia/strabismus.
Optical treatment of nystagmus can include refractive correction, prism to help move the young patient into the null point, vision therapy, and low vision interventions when necessary.
Stargardts
This is a genetic eye disease that affects 1 in 10,000 and is characterized by a dysfunction in how the body uses vitamin A, resulting in a buildup of fatty material (lipofuscin) in the macula, resulting in permanent vision loss.10 Presenting signs and symptoms include blurred vision, sensitivity to light, poor night vision, and color blindness and often begin between the ages of six and 12, but some cases go undiagnosed until adulthood.
A recent study shows the positive effects of omega-3 fatty acids on the slowing of Stargardt’s.11 Although there is no cure, treatment options include education on lifestyle habits (healthy diet, exercise, no smoking), low vision aids, and any refractive needs. There are many related ongoing clinical trials.
Color blindness
Color vision development occurs within the first year of life, reaching almost adult-like levels by four-to-five months (sensitivity with color vision continues to develop as the child grows). The youngest clinical color vision testing can be done around age three with shapes as the target. Every child’s early eye exam should include color vision testing to act as baseline, as color vision deficiencies can be inherited or acquired. Having this information early on can be helpful in making this distinction.
Although there is no treatment for color blindness, early detection in inherited individuals is important because color is often used as an early learning tool. Parents and educators of these children need to know how to present material in a different way to ensure academic success. A discussion of the potential restricted careers (i.e. pilot, military), and lifestyle modification is also recommended. OM
References:
1. Lembo A, Serafino M, Strologo MD, et al. Accommodative esotropia: the state of the art. Int Ophthalmol. 2019;39(2):497-505. doi: 10.1007/s10792-018-0821-6.
2. Boniquet-Sanchez S, Sabater-Cruz N. Current Management of Amblyopia with New Technologies for Binocular Treatment. Vision (Basel). 2021;5(2):31. doi: 10.3390/vision5020031.
3. Scheiman MM, Hertle RW, Beck RW, et al. Randomized trial of treatment of amblyopia in children aged 7 to 17 years. Arch Ophthalmol. 2005;123(4):437-47. doi: 10.1001/archopht.123.4.437.
4. Gupta P, Gurnani B, Patel BC. Pediatric Cataract. [Updated 2024 Jun 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK572080/
5. Hatton DD, Schwietz E, Boyer B, Rychwalski P. Babies Count: the national registry for children with visual impairments, birth to 3 years. JAAPOS. 2007;11(4):351-5. doi: 10.1016/j.jaapos.2007.01.107.
6. Delhiwala KS, Vadakkal IP, Mulay K, et al. Retinoblastoma: An Update. Semin Diagn Pathol. 2016;33(3):133-40. doi: 10.1053/j.semdp.2015.10.007.
7. Bishnoi K, Prasad R, Upadhyay T, Mathurkar S. A Narrative Review on Managing Retinopathy of Prematurity: Insights Into Pathogenesis, Screening, and Treatment Strategies. Cureus.2024;16(3):e56168. doi: 10.7759/cureus.56168.
8. Shah PK, Prabhu V, Karandikar SS, Ranjan R, Narendran V, Kalpana N. Retinopathy of prematurity: past, present and future. World J Clin Pediatr. 2016;5(1):35-46.doi: 10.5409/wjcp.v5.i1.35.
9. Nash DL, Diehl NN, Mohney BG. Incidence and Types of Pediatric Nystagmus. Am J Ophthalmol. 2017:182:31-34. doi: 10.1016/j.ajo.2017.07.006.
10. Blacharski PA. Fundus flavimaculatus. In: Newsome DA, ed. Retinal Dystrophies and Degenerations. New York: Raven Press; 1988: 135–159.
11. Prokopiou E, Kolovos P, Kalogerou M, et al. Omega-3 Fatty Acids Supplementation: Therapeutic Potential in a Mouse Model of Stargardt Disease. Invest Ophthalmol Vis Sci.2018;59(7):2757-2767. doi: 10.1167/iovs.17-23523.
