Abstract
In this cohort study, we report demographics and post-lensectomy outcomes in 30 children with Down syndrome and cataract (39 eyes). Their mean age at first lensectomy was 3.0 years (range: 0.04 – 12.9 years); 16 of 30 (53%) had lensectomy before 12 months of age. The 5-year cumulative incidence of glaucoma-related adverse events was 38% (95% CI: 0–69%), and retinal detachment was 3% (95% CI: 0–9%). The median myopic shift in children with refractive data at 5 years was −10.00 D (interquartile range: −12.50 to −8.38 D) in 11 aphakic eyes and −1.50 D (interquartile range: −3.00 to −0.25 D) in 7 pseudophakic eyes. The period prevalence (combining cases at baseline with those developing the condition during follow up) of strabismus by 5 years was 81% (95% CI: 40–94%), and nystagmus was 68% (95% CI: 41%−83%). Among 11 children able to perform optotype visual acuity testing, 2 of 12 study eyes (17%, 95% CI: 3%−56%) were 20/60 or better at 5 years.
Introduction:
Among children with cataracts, 2–7% have Down syndrome.1–3 In Down syndrome, the prevalence of lens opacities increases with age from about 1% in infancy to more than 72% among adults.2,4,5 Studies describing outcomes following lensectomy in children with Down syndrome are limited by retrospective design, small sample size, and variable follow-up.1,2,6 Our purpose is to describe outcomes following lensectomy in children with Down syndrome.
Methods:
The PEDIG cataract surgery registry enrolled children from birth to <13 years of age who had undergone lensectomy during the preceding 45 days.7 The protocol and Health Insurance Portability and Accountability Act-compliant informed consent forms were approved by each site’s institutional review board, and the parent or guardian of each child provided written informed consent for the data collection. Data were collected from annual medical record reviews for 5 years post-lensectomy. Herein, we report visual acuity (VA), change in refractive error, and cumulative incidences (with 95% confidence intervals; CI) of glaucoma-related adverse events,8 other complications, and surgeries in children with Down syndrome. The period prevalences of strabismus and nystagmus were determined by combining those with the condition at baseline with those developing the condition over follow-up.
Results:
Of 994 children undergoing bilateral or unilateral lensectomy,7 31 (3%) had Down syndrome; one did not complete any follow-up visits. Among the remaining 30 children with Down syndrome, 39 eyes were enrolled in the study (11 from children with unilateral cataracts and 28 from 19 children with bilateral cataracts). Baseline characteristics of these 30 children (39 eyes) are in eTables 1 and 2.
Mean age at first lensectomy was 3.0 years (range: 0.04–12.9 years; median [interquartile range (IQR)] 0.7 [0.1–4.1] years). Sixteen children (53%) underwent lensectomy before 12 months of age. Primary intraocular lens (IOL) insertion was performed at investigator discretion in 12 of 30 (40%) children. Twenty-three of 30 children (77%) completed their 5-year follow-up visits. Among 7 children (23%) not completing the 5-year visit, 1 had 4 years of follow-up, 2 had 3 years, 2 had 2 years, and 2 had 1 year.
The 5-year cumulative incidences of intraocular complications and surgeries are in Table 1. Excluding eyes with a history of trauma or pre-existing glaucoma, glaucoma-related adverse events occurred in 5 of 36 eyes (5-year cumulative incidence: 38%; 95% CI: 0%−69%) (N=3 children, all bilateral aphakia). Median age at lensectomy for these 5 eyes was 0.6 (range: 0.1–1.2) years. No child underwent glaucoma surgery during follow-up. Retinal detachment occurred in 1 of 39 eyes (aphakic) (5-year cumulative incidence: 3%; 95% CI: 0%−9%). There were no cases of endophthalmitis.
Table 1.
Five-year Cumulative Incidence of Intraocular Complications and Intraocular Surgeries Post Lensectomy (Eye Level)
| Bilateral Aphakia | Bilateral Pseudophakia | Unilateral Aphakia | Unilateral Pseudophakia | Overall | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Intraocular Complications | N | Events | 5-Year Incidence (95% CI)a |
N | Events | 5-Year Incidence (95% CI)a |
N | Events | 5-Year Incidence (95% CI)a |
N | Events | 5-Year Incidence (95% CI)a |
N | Events | 5-Year Incidence (95% CI)a |
| Glaucoma-Related Adverse Eventsb | 21 | 5 | 44% (0% - 74%) |
7 | 0 | 0% | 4 | 0 | 0% | 4 | 0 | 0% | 36 | 5 | 38% (0% - 69%) |
| Glaucomab | 21 | 4 | 41% (0% - 72%) |
7 | 0 | 0% | 4 | 0 | 0% | 4 | 0 | 0% | 36 | 4 | 36% (0% - 67%) |
| Glaucoma Suspectb | 21 | 1 | 5% (0% - 13%) |
7 | 0 | 0% | 4 | 0 | 0% | 4 | 0 | 0% | 36 | 1 | 3% (0% - 8%) |
| Retinal Detachment | 21 | 1 | 5% (0% - 15%) |
7 | 0 | 0% | 5 | 0 | 0% | 6 | 0 | 0% | 39 | 1 | 3% (0% - 9%) |
| Visual Axis Opacificationc | 20 | 3 | 16% (0% - 34%) |
5 | 1 | 18% (0% - 43%) |
5 | 0 | 0% | 4 | 0 | 0% | 34 | 4 | 13% (0% - 26%) |
| Intraocular Surgeries Not at the Time of Lensectomy | N | Events | 5-Year Incidence (95% CI)a |
N | Events | 5-Year Incidence (95% CI)a |
N | Events | 5-Year Incidence (95% CI)a |
N | Events | 5-Year Incidence (95% CI)a |
N | Events | 5-Year Incidence (95% CI)a |
| Secondary Intraocular Lens Implantd | 21 | 6 | 30% (0% - 52%) |
7 | 0 | 0% | 5 | 2 | 50% (0% - 83%) |
6 | 0 | 0% | 26d | 8 | 34% (5% - 55%) |
| Glaucoma Surgerye | 21 | 0 | 0% | 7 | 0 | 0% | 4 | 0 | 0% | 4 | 0 | 0% | 36 | 0 | 0% |
| Retinal Detachment Surgery | 21 | 1 | 5% (0% - 15%) |
7 | 0 | 0% | 5 | 0 | 0% | 6 | 0 | 0% | 39 | 1 | 3% (0% - 9%) |
| Surgery to Clear the Visual Axisf | 20 | 3 | 15% (0% - 33%) |
5 | 1 | 18% (0% - 43%) |
5 | 0 | 0% | 4 | 0 | 0% | 34 | 4 | 13% (0% - 26%) |
The 95% CI is not reported when the incidence is 0.
Glaucoma-related adverse events defined as either glaucoma (IOP>21mmHg + corneal enlargement >11mm or increase in >0.2 cup-to-disc ratio or use of IOP lowering med or glaucoma surgery) and glaucoma suspect (IOP >21 mmHg without optic nerve cupping); eyes with ocular trauma and eyes with preexisting glaucoma were excluded. The glaucoma-related adverse events occurred in 3 of 23 eyes for those under 1 year of age at lensectomy (5-year cumulative incidence: 35%; 95% CI: 0%−67%). The age of surgery was 0.12, 0.16, and 0.56 years respectively.
Only eyes with posterior capsulotomy/anterior vitrectomy were included. Of the 5 eyes with no posterior capsulotomy/anterior vitrectomy at initial lensectomy, visual axis opacification was reported in 3 eyes (5-year cumulative incidence: 54%; 95% CI: 2%−79%).
Only eyes without primary intraocular lens implants were included.
Eyes with ocular trauma and eyes with preexisting glaucoma were excluded.
Only eyes with posterior capsulotomy/anterior vitrectomy were included. Of the 5 eyes that had no posterior capsulotomy/anterior vitrectomy at initial lensectomy, a procedure to clear the visual axis was performed in 2 eyes (5-year cumulative incidence: 36%; 95% CI: 0%−63%).
Of 34 eyes that had a posterior capsulotomy/anterior vitrectomy at initial lensectomy, visual axis opacification was reported in 4 eyes (3 aphakic and 1 pseudophakic) (5-year cumulative incidence:13%; 95% CI: 0%−26%); each had a procedure to clear the visual axis (5-year cumulative incidence: 13%; 95% CI: 0%−26%).
Of 5 eyes that had no posterior capsulotomy/anterior vitrectomy at initial lensectomy, visual axis opacification was reported in 3 eyes (5-year cumulative incidence: 54%; 95% CI: 2%−79%), and a procedure to clear the visual axis was performed in 2 eyes (pseudophakic) (5-year cumulative incidence: 36%; 95% CI: 0%−63%).
Eight of 26 eyes without primary IOL insertion underwent secondary IOL implantation at investigator discretion (5-year cumulative incidence: 34%; 95% CI: 5%−55%). The median (range) age at secondary IOL implantation was 3.0 (1.5–4.6) years.
Optotype VA at 5 years was reported for 12 of 39 eyes (31%) from 11 of 30 children; 2 of 12 eyes (17%: 95% CI 3%−56%) were 20/60 or better (eTable 3). Amblyopia was reported in 12 of 30 children (5-year cumulative incidence: 50%; 95% CI: 23%−67%).
The period prevalence of strabismus was 81% (21 of 30 children) (95% CI: 40%−94%); 8 had strabismus before lensectomy (Table 2). The period prevalence of nystagmus was 68% (19 of 30 children) (95% CI: 41%−83%); 3 had nystagmus before lensectomy.
Table 2.
Five-year Cumulative Incidence of Amblyopia and Strabismus Surgery and Period Prevalencea of Nystagmus and Strabismus (Child Level)
| Bilateral Aphakia | Bilateral Pseudophakia | Unilateral Aphakia | Unilateral Pseudophakia | Overall | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| N | Events | 5-Year Incidence (95% CI)b |
N | Events | 5-Year Incidence (95% CI)b |
N | Events | 5-Year Incidence (95% CI)b |
N | Events | 5-Year Incidence (95% CI)b |
N | Events | 5-Year Incidence (95% CI)b |
|
| 5-Year Cumulative Incidence | |||||||||||||||
| Amblyopia | 13 | 7 | 54% (17% - 75%) |
6 | 0 | 0% | 5 | 1 | 18% (0% - 45%) |
6 | 4 | 83% (0% - 98%) |
30 | 12 | 50% (23% - 67%) |
| Strabismus Surgery | 13 | 3 | 25% (0% - 47%) |
6 | 2 | 31% (0% - 58%) |
5 | 0 | 0% | 6 | 0 | 0% | 30 | 5 | 19% (2% - 33%) |
| 5-Year Period Prevalence | |||||||||||||||
| Strabismus | 13 | 10 | 88% (3% - 99%) |
6 | 3 | 49% (0% - 77%) |
5 | 3 | 75% (0% - 97%) |
6 | 5 | 74% (3% - 93%) |
30 | 21 | 81% (40% - 94%) |
| Nystagmus | 13 | 11 | 84% (40% - 96%) |
6 | 2 | 28% (0% - 55%) |
5 | 2 | 50% (0% - 83%) |
6 | 4 | 61% (0% - 85%) |
30 | 19 | 68% (41% - 83%) |
Period prevalence includes the children with the condition at baseline and those who developed it after lensectomy.
The 95% CI is not reported when the incidence is 0.
Eighteen eyes (7 pseudophakic, 11 aphakic) from 12 children with Down syndrome had refraction data at 5 years. In 11 aphakic eyes, mean myopic shift was −10.61 D (median: −10.00 D; IQR: −12.50 to −8.38 D) with a median age at lensectomy of 0.2 years (range: 0.1–1.2 years). In 7 pseudophakic eyes, mean myopic shift was −1.75 D (median: −1.50 D; IQR: −3.00 to −0.25 D) with a median age at lensectomy of 4.1 years (range: 1.5–12.0 years). Children with lensectomy before 12 months of age (n=9 aphakic eyes) had a mean myopic shift of −9.56 D (median: −8.75 D; IQR: −12.00 to −8.38 D). Aphakic children in the PEDIG registry without Down Syndrome who had lensectomy before 12 months of age and no secondary IOL (n=76; 109 eyes) had a mean myopic shift of −9.43 D (median: −9.00 D; IQR: −16.25 to −2.75 D).
Discussion:
In the prospective PEDIG registry of cataract surgery in 994 children <13 years of age, 31 (3%) had Down syndrome, with about half having lensectomy in the first year of life. The most common complication was glaucoma or glaucoma suspect (38%); retinal detachment occurred in 1 child, and none developed endophthalmitis. Previous reports of lensectomy in children with Down syndrome have noted glaucoma frequency ranges from 10 to 15% and retinal detachment from 6 to 9%.1,2,6
In the present study 17% (of 12 eyes) had 20/60 or better VA, compared with less than the 40% (of 33 eyes) reported by Gardiner et al at a mean 11.2 years, postoperatively.1 Gardiner et al found an average myopic shift of −7.96 (SD 4.7) D in aphakic eyes (n=16) and −8.06 (SD 7.4) D in pseudophakic eyes (n=17), which is lower than our aphakic children but greater than our pseudophakic children. However, their study had a wide range of follow-up (2.5 months to 25 years) and smaller sample sizes.1 The mean myopic change over 5 years in our study (−9.56 D; n=9 eyes) was somewhat less than that reported by Gardiner et al for surgery in the first 2 years of life (mean −11.50 D; n=19 eyes),2 but is comparable with the mean myopic shift of −9.43 D seen in children without Down syndrome and without secondary IOL implant operated in the first year of life in the PEDIG registry cohort (n=109 aphakic eyes).
Limitations of this study include the small number of children with Down syndrome and missing refraction data at the 5-year follow-up visit. Additionally, optotype VA testing was not possible in many cases due to developmental delay.
Despite the complexity of cataract surgical and postoperative care for children with Down syndrome, we found outcomes and rates of postoperative complications comparable with those for the overall PEDIG registry cohort without Down Syndrome.8
Supplementary Material
Funding/Support:
Supported by National Eye Institute of National Institutes of Health, Department of Health and Human Services EY011751, EY023198, and EY018810. The funding organization had no role in the design or conduct of this research.
Footnotes
Conflict of Interest: No conflicting relationships exist for any author.
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