Incidence of Retinal Detachment Following Lens Surgery in Children and Young Adults with Non-Traumatic Ectopia Lentis

Abstract

Purpose:

To determine the incidence of retinal detachment following lens surgery in children and young adults with non-traumatic ectopia lentis.

Setting:

Population-based claims data.

Design:

Population-based retrospective cohort study

Methods:

A total of 210 patients (298 eyes) with non-traumatic ectopia lentis aged ≤ 30 years who had undergone lens surgery with or without intraocular lens (IOL) implantation and had ≥ 1 year of continuous enrollment after lens surgery were identified from the Optum deidentified Clinformatics Data Mart Database (2003–2019) and IBM® MarketScan® Databases (2007–2016). We assessed sex, age, etiology of ectopia lentis, IOL implantation and postoperative retinal detachment in both databases, separately. Univariate and multivariate analyses were conducted to identify the risk factors for postoperative retinal detachment.

Results:

IOL implantation was coupled with lens surgery in 151 (49.8%) eyes that underwent lens surgery for non-traumatic ectopia lentis. Median follow-up was 32 months in aphakic eyes and 29 months in pseudophakic eyes. Patients undergoing IOL implantation were older at the time of lens surgery (median age: no IOL, 6 years; IOL, 16 years; P < .001). Retinal detachment developed in 14 eyes (4.7%) (no IOL vs IOL; P = .30). Older age was the baseline characteristic that correlated most closely with the risk of retinal detachment (P = .05).

Conclusions:

The rate of retinal detachment was similar with or without IOL implantation after lens surgery for non-traumatic ectopia lentis in children and young adults.

Ectopia lentis may occur in association with a variety of systemic diseases, but most often occurs in patients with Marfan syndrome.¹ It may also develop after ocular trauma.² Good visual acuity can often be achieved in eyes with mild subluxation of the lens with spectacle correction alone. However, moderate to severe subluxation of the lens often must be treated with a lensectomy to achieve good visual acuity.³

In the past, eyes with ectopia lentis were usually left aphakic after a lensectomy and their aphakia was corrected with contact lenses or spectacles.⁴ In recent years, new surgical techniques have been developed to facilitate the implantation of intraocular lenses (IOLs) in eyes with ectopic lentis. These techniques include the implantation of an: iris-claw IOL, iris-sutured posterior chamber IOL (PCIOL), scleral-sutured PCIOL, intrascleral haptic fixated PCIOL, in-the-bag fixation using a capsular tension ring coupled with amputation of one haptic of a 3 piece PCIOL and in-the-bag fixation with a Cionni capsular tension ring sutured to the sclera.^5–11 A variety of complications have been reported after these surgical procedures including: suture erosions, haptic extrusion, glaucoma, chronic uveitis, pupillary block, optic capture, IOL decentration, IOL dislocation, cystoid macular edema, vitreous hemorrhage, suprachoroidal hemorrhage and retinal detachment.^11–14 In a recent review, the incidence of retinal detachment after IOL implantation in patients without zonular support was reported to be 0% – 8.2%.¹¹ While the incidence of retinal detachment after lens surgery in patients with ectopia lentis has been reported to be 0% – 17.2%, most of these reports are single-center studies from surgeons with a special expertise in the type of surgery.^{4–10, 14–20}

Using commercial insurance claims datasets, we investigated the incidence of retinal detachment after lens surgery in eyes with non-traumatic ectopia lentis to determine whether IOL implantation increased the risk of retinal detachment.

Methods

Study design and data source

This population-based retrospective cohort study was performed using claims data that were analyzed separately from the Optum SES Medical Claims dataset from Optum’s De-identified Clinformatics^® Data Mart (CDM) (2003–2019) and IBM^® MarketScan^® Research Databases (2007–2016).

CDM Database (OptumInsight, Eden Prairie, MN) is a de-identified commercial and Medicare Advantage claims database. The dataset comprises patient claims information on approximately 15 to 18 million annually covered lives, for a total of over 63 million lives from a geographically diverse population spanning all 50 states of the US. The Optum dataset from CDM provides demographic and medical claims data for inpatient and outpatient services including surgery. The MarketScan Databases comprises more than 240 million patients insured by 350 unique health carriers. These data include health insurance claims across the continuum of care (e.g. inpatient, outpatient, outpatient pharmacy, carve-out behavioral healthcare) as well as enrollment data from large employers and health plans across the United States. Both medical claims datasets include the International Classification of Disease, 9th and 10th revisions (ICD-9-CM and ICD-10-CM, respectively), diagnosis codes, and Current Procedural Terminology (CPT) Version 4 procedure codes.

Data access for this project was provided by the Stanford Center for Population Health Science Data Core, which is supported by the National Institutes of Health National Center for the Advancing Translational Science Clinical and Translational Science Award (UL1 TR001085) and internal Stanford funding. The content of this article is solely the responsibility of the authors and does not necessarily represent the official views of the NIH. The Stanford University School of Medicine Institutional Review Board determined that this study did not require IRB approval because it only used deidentified data.

Study population

Patients were included in the study if they had been diagnosed with subluxation of the lens and underwent lens surgery without IOL implantation (CPT codes: 66840, 66850, 66852, 66920, 66930, 66940 and 67005) or with IOL implantation (CPT codes:66982, 66983, 66984, and 66985). We only included patients who had undergone lens surgery when ≤ 30 years of age and who had continuous enrollment for ≥ 1 year from the time of lens surgery. We excluded from the analysis patients with an ICD code for prior retinal detachment or ocular trauma (Table 1 and Figure 1). We also investigated the retinal detachment rate after lens surgery in patients with Marfan syndrome with or without IOL implantation. We assessed gender, age, etiology of ectopia lentis, IOL implantation and postoperative retinal detachment in both databases, separately. In addition, we investigated modifier codes (RT for right eye, LT for left eye and 50 for bilateral eyes) from ocular examinations and procedure codes from surgeries to identify eye laterality of lens surgeries and retinal detachment.

Table 1.

Internal classification of disease (ICD) codes used to identify patients.

ICD-9 codes	ICD-10 codes	Brief descriptor
Included diseases
379.32	H27.11, H27.11x	Subluxation of lens
361.00–361.05	H33.0xx	Retinal detachment
759.82	Q87.4, Q87.4x, Q87.4xx	Marfan syndrome
Excluded diseases
366.2, 366.20–366.23	H26.1, H26.1x, H26.1xx	Traumatic cataract
871, 871.x, 921, 921.x	S05, S05.x, S05.xx, S05.xxx, S05.xxxx	Eye or orbit trauma

Figure 1.

Flow diagram illustrating the path taken to identify the incidence of postoperative retinal detachment in patients underwent lens operation for non-traumatic ectopia lentis aged ≤ 30 years in Optum and MarketScan datasets.

Statistical analysis

The chi-square test was used to compare gender, laterality, Marfan syndrome and retinal detachment rate between aphakic and pseudophakic eyes, and between eyes with retinal detachment and no retinal detachment. The Mann-Whitney U test was used to compare age at the time of lens surgery and continuous enrollment period from the time of the lens surgery between the groups with and without IOL implantation, and between eyes with retinal detachment and no retinal detachment. We conducted univariate and multivariate logistic regression analyses to identify the risk factors for postoperative retinal detachment. A P value of < .05 was considered statistically significant. The final multivariate logistic model was used to calculate odds ratios and their 95% confidence intervals. All statistical analyses were conducted using R software (version 9.4).

Results

OPTUM dataset

A total of 6,941,657,331 claims for 96,287,504 patients were analyzed in the Optum SES Medical Claims dataset (version 3.0). Among these patients, 92 (125 eyes) had undergone lens surgery for subluxation of the lens aged ≤ 30 years and were enrolled continuously ≥ 1 year after their lens removal or IOL implantation surgery. The median age at the time of lens surgery was 11 (range, 0–30) years. IOL implantation was performed on 74 eyes (59.2%). The most common etiology for ectopia lentis was Marfan syndrome (n=61 (48.8%)). The median continuous enrollment period after lens surgery was 30 months (range, 12–166).

MarketScan dataset

A total of 8,713,134,185 claims for 123,637,720 patients were analyzed in the MarketScan Databases (version 2.0). Among these patients, 118 (173 eyes) had undergone lens surgery for subluxation of the lens aged ≤ 30 years and were enrolled continuously ≥ 1 year after their lens removal or IOL implantation surgery. The median age of lens surgery with or without IOL implantation was 9 (range: 0–30) years. IOL implantation was performed in 77 eyes (44.5%). The most common etiology for ectopia lentis was Marfan syndrome (n=113 (65.3%)). The median continuous enrollment period after lens surgery was 29 months (range, 12–109).

Clinical Findings

During the study period, 210 patients (298 eyes) underwent lens surgery for subluxation of the lens before the age of 30 years and were enrolled continuously ≥ 1 year after their lens removal or IOL implantation surgery. The median age of lens surgery with or without IOL implantation was 9 (range: 0–30) years. IOL implantation was performed in 151 eyes (49.8%). The most common etiology for ectopia lentis was Marfan syndrome (n=177 (59.4%)). Retinal detachment developed in 14 eyes (4.7%). The median continuous enrollment period after lens surgery was 29 months (range, 12–166).

The patients who were left aphakic after lens surgery were younger than those who underwent IOL implantation (P < .001). No differences were observed in terms of: gender, laterality, the diagnosis of Marfan syndrome or the continuous enrollment period after lens operation (P > 0.05). There was no difference in the retinal detachment occurrence rate between aphakic and pseudophakic eyes (P = .30) (Table 2). The median time interval from lens surgery to retinal detachment was 41 days (range, 14–1590) in aphakic eyes and 119 days (range, 37–721) in pseudophakic eyes (P = .61).

Table 2.

Patient demographics and characteristics.

	Aphakia	Pseudophakia	P Value
Number of eyes	147	151
Age (median, range, years)	6 (0–29)	16 (1–30)	<.001
Sex (n (%))
Female	66 (44.9%)	59 (39.1%)	.31
Male	81 (55.1%)	92 (60.9%)
Laterality (n (%))
Right	72 (49.0%)	69 (45.7%)	.20
Left	64 (43.5%)	61 (40.4%)
Unknown	11 (7.5%)	21 (13.9%)
Marfan syndrome (n (%))	93 (63.3%)	84 (55.6%)	.18
Retinal detachment (n)*	< 11	< 11	.30
Continuous enrollment period after the lens surgery (median, range, months)	32 (12–166)	29 (12–157)	.10

^*The report of cells less than 11 is prohibited by the center for population health sciences at Stanford University School of Medicine.

With univariate analysis, an older age at the time of lens surgery was associated with higher odds of retinal detachment (P = .02). However, the odds of retinal detachment decreased with multivariate logistic regression (P = .05) (Table 3).

Table 3.

Univariate and multivariate logistic regression analysis of retinal detachment after lens surgery in patients with ectopia lentis.

	Univariate analysis		Multivariate analysis
	OR (95% CI)	P Value	OR (95% CI)	P Value
Age	1.075 (1.009–1.144)	.02	1.070 (0.999–1.146)	.05
Sex	1.407 (0.481–4.117)	.53
Marfan syndrome	1.752 (0.536–5.719)	.35	1.678 (0.504–5.590)	.40
Intraocular lens implantation	1.800 (0.589–5.504)	.30	1.162 (0.339–3.988)	.81
Continuous enrollment period	1.005 (0.990–1.021)	.52

CI = confidence interval; OR = odds ratio

Discussion

We found a 4.7% incidence of retinal detachment after lens surgery for non-traumatic ectopia lentis after a median follow-up of 2 ½ years in a large cohort of children and young adults using commercial insurance claims data. More than one-half of the patients had been diagnosed with Marfan syndrome. Generally, patients who underwent IOL implantation were older. The incidence of retinal detachment was similar with or without IOL implantation.

The incidence of retinal detachment in our cohort of children and young adults that underwent lens surgery for ectopia lentis was higher than in most other single-center series (Table 4).^{4–10,14–20} Our series uses claims data which is less likely to exclude patients and is nearly four times larger than any previous series. In addition, single-center series are often reported by surgeons who have a special expertise in the type of surgery being reported, whereas claims data includes surgeons with a broader range of expertise. Older studies reported much higher rates of retinal detachment following lens surgery for ectopia lentis (8–38%) than in our series, but these studies were performed before the advent of modern surgical techniques.^1,21,22 The nearly 5% incidence of retinal detachment in our series is much higher than the < 1% incidence of retinal detachment reported in most other pediatric cataract surgery cohorts. Repka et al²³ reported retinal detachment in only 8 of 1132 (0.7%) eyes in children who underwent routine cataract surgery.

Table 4.

Review of lens surgery studies for children and young adults with ectopia lentis.

Author (year)	Country	Number of eyes	Mean age at lens surgery (years)	Eyes with retinal detachment (%)	Type of surgery	Mean follow-up (years)
Wu-Chen et al (2005)4	United States	17	7.7	0 (0%)	Lensectomy/No IOL	8.5
Kim et al (2008)15	Korea	78	5.7	1 (1.3%)	Lensectomy/No IOL	7.1
Vasavada et al (2008)5	United States	35	8.2	0 (0%)	PCIOL with CTR	2.3
Arraes et al (2010)6	Brazil	17	7–22*	0 (0%)	PCIOL with CTR	2.8
Hirsashima et al (2010)16	Brazil	16 15	26.0 25.0	2 (12.5%) 1 (6.7%)	IF-PCIOL IC-ACIOL	1.0
Zheng et al (2012)17	China	32 39	22.5 18.6	0 (0%) 2 (5.1%)	IC-ACIOL SS-PCIOL	1.0
Kumar et al (2012)7	India	41	10.7	0 (0%)	ISHF	1.5
Hsu et al (2012)20	United States	13	6.1	0 (0%)	SS-PCIOL	2.0
Fan et al (2014)14	China	64	16.5	11 (17.2%)	No IOL or IC-ACIOL or SS-PCIOL	3.0
Miraldi et al (2014)18	United States	42	10.2	1 (2.4%)†	Lensectomy/No IOL	4.9
Thapa et al (2015)9	India	28	8.1	0 (0%)	PCIOL with CTR or ISHF	1.0
Manning et al (2016)8	Ireland	14 16	4.6 12.7	0 (0%) 0 (0%)	Lensectomy/No IOL IC-ACIOL	13.8 4.1
Català -Mora et al (2017)10	Spain	21	8.0	1 (5%)†	IC-ACIOL	3.2
Sen et al (2020)19	India	73	11.2	3 (4.1%)	SS-PCIOL	3.9
Our study‡	United States	298	9	14 (4.7%)	Lensectomy/No IOL or IOL	2.4

ACIOL = anterior chamber intraocular lens; CTR = capsular tension ring; IC-ACIOL = iris-claw anterior chamber intraocular lens; IF-PCIOL = iris-fixed posterior chamber intraocular lens; IOL = intraocular lens; ISHF = intrascleral haptic fixation with glue; PCIOL = posterior chamber intraocular lens; SS-PCIOL = scleral sutured posterior chamber intraocular lens

^*Range of age

^†Following blunt trauma

^‡Median values of age at lens surgery and follow up period

We found that older patients were more likely to have undergone IOL implantation in our series. There are several possible explanations for this. First, surgeons may be reluctant to implant IOLs in young children who are at risk of developing a myopic shift as their eyes grow. Second, pediatric ophthalmologists, who perform most lens surgeries in young children in the United States, are not generally trained to implant IOLs in eyes with ectopia lentis. The one exception is the implantation of an iris-claw anterior chamber IOL. However, this lens is only currently available in the United States as part of a clinical trial (NCT01547442).²⁴

Our results showed that postoperative retinal detachment occurred less frequently in younger patients than in older patients in a multivariate analysis (P = .05). Other studies have also reported a low rate of retinal detachment in the eyes of young children after lens surgery for ectopia lentis (Table 4). This may be partially due to the fact that younger patients with ectopia lentis have traditionally undergone lensectomies without IOL implantation. Kim et al¹⁵ reported retinal detachment in only 1 of 78 eyes after lensectomies in children with ectopia lentis at a mean age of 5.7 years. Manning et al⁸ reported no retinal detachments in 30 eyes of children who underwent lensectomies at a mean age of 4.6 years even after 16 of these eyes later underwent IOL implantation. Even when lens surgery is combined with IOL implantation, the rate of retinal detachment in young patients has been reported to be low. Hsu et al²⁰ reported no retinal detachments following lensectomies with scleral-fixated IOL implantation among patients with non-traumatic ectopia lentis who were approximately 10 years of age.

A recent study reported that IOL implantation with capsular bag fixation reduced the incidence of postoperative retinal detachment in patients with atopic cataracts. ²⁵ They hypothesized that forward displacement of the vitreous with eye movements and contraction of the residual lens capsule may contribute to the development of retinal detachment in aphakic eyes. Prior to performing our study, we thought there would be an increased risk of retinal detachment in eyes with ectopia lentis undergoing IOL implantation because of the increased complexity of these surgeries that often include suturing or fixating haptics to the sclera. However, we found a similar risk of postoperative retinal detachment in patients who underwent IOL implantation compared to those who were left aphakic (p=0.297).

In a recent review of retinal detachment after IOL implantation in eyes lacking zonular support, retinal detachment was 2 to 3 times higher scleral- and iris-sutured PCIOLs (2%−3.3%) compared with the nonsutured IOLs: iris claw, anterior chamber IOL, and PCIOL with intrascleral haptic fixation of PCIOLs fixation (0%−0.9%) in the patients without zonular support.¹¹ However, we were not able to investigate the kinds of implanted IOL in these datasets.

Patients with Marfan syndrome and ectopia lentis have been reported to be at increased risk of retinal detachment after lens surgery due to the instability of their subluxated lenses that causes traction to be exerted on the vitreous base leading to small tears or holes in the retinal periphery.²⁶ In one series, 28 (15%) of 185 adults with Marfan syndrome were reported to have had a retinal detachment at a mean age of 33 years.²⁷ Six of these patients had undergone lens surgery prior to retinal detachment. We did not find a difference in the postoperative retinal detachment rate of patients with ectopia lentis undergoing surgery with or without Marfan syndrome.

There are a number of limitations to our study. First, it was conducted using claims data, which does not include data on the severity of lens subluxation, visual acuity, refractive error, or axial length. We could not identify the methods used for IOL implantation or whether a capsular tension ring was used. In addition, we could not determine eye laterality in some patients. However, we were able to identify eye laterality in all patients with retinal detachment after lens surgery. Second, it is possible that some patients were included in both the Marketscan and Optum datasets we used. However, since lens surgery for ectopia lentis was a requirement to be in the analysis and since the insurance companies included in the Optum and Marketscan datasets do not overlap, this would have only occurred if a patient changed their insurance coverage between lens surgery on their first and second eyes Third, as is the case with all claims data, miscoding may have occurred. Fourth, we could not investigate patient data outside the enrollment period. Hence, we only included patients with ≥ 1 year of continuous enrollment after the lens surgery. We may have missed patients with continuous enrollment less than 1 year who experienced an early retinal detachment. Another limitation is the relatively short follow-up period (median, 29 months (range, 12–166)). With longer follow-up, more of these eyes may have developed retinal detachment. Large prospective studies are needed to confirm the incidence of long-term retinal detachment after lens surgery for ectopia lentis.

In conclusion, our claim data-based study reflecting real-world practice showed that retinal detachment occurred in 4.7% of eyes after lens surgery for non-traumatic ectopia lentis in children and young adults after a median follow-up of 2 ½ years. The rate of retinal detachment was similar with or without IOL implantation.

Value Statement.

What Was Known

• The incidence of retinal detachment after lens surgery in children and young adults with ectopia lentis has been reported to be 0% – 17.2%. However, most of these reports are single-center studies.

What This Paper Adds

• Retinal detachment occurred in 4.7% of eyes after lens surgery for non-traumatic ectopia lentis in children and young adults after a 2 ½ year follow-up in a large cohort of patients identified using claims data.

• The rate of postoperative retinal detachment was similar with or without intraocular lens implantation.