- Email: [email protected]
Primary versus secondary IOL implantation following removal of infantile unilateral congenital cataract: outcomes after at least 5 years
Primary versus secondary IOL implantation following removal of infantile unilateral congenital cataract: outcomes after at least 5 years Dina Tadros, MD,a,b Rupal H. Trivedi, MD,a and M. Edward Wilson, MDa PURPOSE
METHODS
To report outcomes after at least 5 years’ follow-up of consecutive patients at a single center undergoing unilateral congenital cataract surgery during infancy with primary intraocular lens (IOL) implantation at the time of surgery or undergoing secondary IOL implantation later in childhood. The medical records of pseudophakic children who received initial cataract surgery before 7 months of age were retrospectively reviewed. Children with acquired cataract, persistent fetal vasculature, congenital glaucoma, or follow-up of \5 years were excluded.
RESULTS
Mean age at time of cataract surgery was 2.7 months in the primary group (n 5 13) and 1.9 months in the secondary group (n 5 13). Mean age at IOL implantation in the latter group was 4.9 2.2 years. Mean age at final follow-up was 10.7 4.2 years in the primary group and 8.7 3.0 years in the secondary group. Glaucoma surgery was performed in 2 primary group eyes and 1 secondary group eye; it was medically controlled in 2 additional eyes of the latter group. One secondary group eye was diagnosed as glaucoma suspect. Visual axis opacification required surgery in 5 primary group eyes and 2 secondary group eyes before IOL implantation. Unplanned IOL exchange or removal for high myopia was required in 3 primary group eyes and 1 secondary group eye. Strabismus surgery was performed in 3 primary group eyes and 7 secondary group eyes. Median visual acuity at final follow-up was 20/150 in both groups. Refraction at last follow-up was 2.9 3.3 D in the primary and 1.8 2.5 D in the secondary group.
CONCLUSIONS
Reoperations were common in both groups over long-term follow-up. Close monitoring for glaucoma is also needed in both groups. ( J AAPOS 2016;20:25-29)
U
nilateral infantile cataracts can be managed with primary intraocular lens (IOL) implantation or secondary IOL implantation later in childhood, when IOL power prediction may be more reliable. The multicenter Infant Aphakia Treatment Study (IATS) was designed to compare the use of immediate IOL implantation for correction of aphakia, with the use of a contact lens after cataract surgery performed in infants 1-7 months with unilateral congenital cataract.1 The 5-year follow-up outcomes did not demonstrate visual benefit from implanting an IOL at the time of surgery and reported more adverse effects associated with primary IOL implantation.1
Author affiliations: aStorm Eye Institute, Medical University of South Carolina, Charleston, South Carolina; bOphthalmology Department, Faculty of Medicine, Tanta University, Egypt This research was supported in part by the Egyptian Cultural and Educational Bureau and an unrestricted grant to the Department of Ophthalmology, MUSC, from Research to Prevent Blindness, New York, NY. Submitted May 22, 2015. Revision accepted October 1, 2015. Correspondence: M. Edward Wilson, MD, Storm Eye Institute, Rm 218, Medical University of South Carolina, 127 Ashley Avenue, Charleston, SC 29401 (email: [email protected]). Copyright Ó 2016 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2015.10.010
Journal of AAPOS
The IATS did not include secondary IOL implantation outcomes. The present study compares single-center longterm outcomes (.5 years) following unilateral cataract surgery of infants receiving a primary IOL implantation and those undergoing secondary IOL implantation later in childhood, after an initial period of aphakia.
Subjects and Methods This study was approved by the Institutional Human Investigation Review Board of the Medical University of South Carolina and adheres to regulations of the Health Insurance Portability and Accountability Act of 1996. The medical records of consecutively operated children who underwent unilateral cataract surgery between 1995 and 2009 at the Storm Eye Institute were retrospectively reviewed. Pseudophakic eyes of children with a history of cataract surgery in one eye at \7 months of age were included. Eyes operated elsewhere, those with a noninfantile or acquired cataract (eg, post-traumatic, iatrogenic postnatal), retinopathy of prematurity, congenital glaucoma, persistent fetal vasculature with stretched ciliary processes, aphakic eyes with no IOL at the final visit and children with follow-up of \5 years after IOL implantation were excluded. All patients underwent keratometry (handheld KM 500 MARCO keratometer; Macro Ophthalmic Inc, Jacksonville,
25
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Tadros, Trivedi, and Wilson
FL) and axial length measurement by immersion A-scan (Ellex, Eden Prairie, MN) at the time of surgery. In addition, preoperative evaluation included intraocular pressure (IOP) measurement using a Tonopen (Tonopen XL, Reichert Inc., Depew, NY) and horizontal corneal diameter (using calipers). The type of cataract was noted by examining the eye under the operating microscope. A retinal fundus examination was performed if possible. When there was no view of the retina, a B-scan ultrasound was performed. The Holladay 1 formula was used to calculate IOL power.2 The target postoperative refraction was based on the patient’s age and biometry.3 One surgeon (MEW) performed all of the surgeries using posterior capsulectomy and vitrectomy techniques published previously.4,5 The infants were divided into two study groups: infants receiving IOL implantation at the time of cataract surgery (primary group) and those who did not undergo IOL implantation at the time of cataract surgery, requiring fitting of a contact lens or glasses when there was contact lens incompatibility, with secondary IOL implantation later in childhood (secondary group). Patients were selected for primary IOL implantation if compliance with contact lens wear was doubtful (ie, poor compliance with contact lens wear by another family member), if other social factors were likely to preclude adequate follow-up of contact lens management (eg, long travel time/distance for office visits, poor vision of parents or caregiver), or if the parents strongly preferred primary IOL implantation when given a choice of IOL now versus IOL later. The infants were then followed at 1 day, 1 week, 4 weeks, 3 months, and then at intervals of 3-6 months during the first year after surgery and once per year afterward. Glaucoma was defined as IOP .21 mm Hg plus one or more of the following anatomical changes: corneal enlargement, asymmetrical progressive myopic shift coupled with enlargement of the corneal diameter and/or axial length, increased optic nerve cupping (ie, an increase of 0.2 in the cup:disk ratio), or the use of a surgical procedure for IOP control.6 A patient was designated as a glaucoma suspect if there was either 2 consecutive IOP measurements .21 mm Hg on different dates after topical corticosteroids had been discontinued, but without any of the anatomical changes listed above, or glaucoma medication use was needed to control IOP, but without any of the anatomical changes noted above.6
Results A total of 99 infants underwent surgery at our center for unilateral cataract prior to 7 months of age. Of these, 26 met the inclusion criteria: 13 in the primary group and 13 in the secondary group. Excluded children included follow-up \5 years, persistent fetal vasculature, Peters anomaly, retinopathy of prematurity, corneal diameter \9 mm, and aphakia. Table 1 shows preoperative parameters and postoperative outcome of both groups. Secondary IOL implantation was performed at an average age of 4.9 years (median, 5 years; range, 0.8-9.4 years). Both groups were comparable at baseline in terms of age at cataract surgery, preoperative corneal diameter, preoperative axial length, age at last follow-up, and duration of followup after the original cataract surgery.
Volume 20 Number 1 / February 2016 Table 1. Preoperative parameters and follow-up of primary and secondary IOL implantation Category Number of eyes Mean age at cataract surgery, months No. #48 days at surgery Mean age at IOL implantation, years Sex Female Male Type of cataract Nuclear Cortical Posterior lentiglobus Posterior polar Anterior polar Mean preoperative axial length, mm Mean preoperative corneal diameter, mm Number of PB IOLsa Number of capsular bag/sulcus IOLs (excluding piggyback IOL) Duration between cataract surgery and last follow-up, years Mean age at last follow-up, years
Primary IOL implantation
Secondary IOL implantation
13 2.7 2.2
13 1.9 1.8
6 0.2 0.2
7 4.9 2.2
10 3
4 9
7 3 1 1 1 18.6 1.3 10.9 0.7
11 0 1 1 0 17.8 1.1 10.3 0.8
4 8/1
1 7/5
10.5 4.2
8.6 3
10.7 4.2
8.7 3
IOL, intraocular lens; PB, piggyback IOL. a Piggyback IOL 5 1 IOL in the capsular bag and 1 in the ciliary sulcus.
The targeted immediate postoperative refraction for primary single IOL implantation was 18 D when the patient was \2 months of age and 16 D for age at surgery of 27 months. For secondary IOL implantation, the targeted refraction was 12 D for 1- to 3-year-olds and plano for children operated on at age $4 years of age. For a piggyback IOL, the targeted immediate postoperative refraction was plano. Seven IOL models were used: AcrySof SA60AT, SN60WF, MA60BM (Alcon, Fort Worth, TX); Pharmacia 811A, 809A (Pharmacia & Upjohn, NY); Rayner 570CFlex (Rayner Intraocular Lenses Ltd, Hove, East Sussex, England); and MC60 (Alcon, Fort Worth, TX). IOLs were implanted either in the capsular bag or in the ciliary sulcus. When double IOL implantation was performed, an AcrySof SA60AT or MA60BM was implanted in the capsular bag, and an AcrySof MA60BM (Alcon, Fort Worth, TX) was implanted in the ciliary sulcus. Of the 13 patients in the primary group, 2 underwent glaucoma surgery: 1 received a Baerveldt glaucoma implant (Abbot Medical Optics, Abbott Park, IL); the other, an Ahmed glaucoma valve (New World Medical Inc, Rancho Cucamongo, CA). Of the 13 patients in the secondary group, 1 received an Ahmed glaucoma valve after IOL implantation. Two other secondary group patients developed glaucoma (controlled on antiglaucoma drugs) and 1 became a glaucoma suspect, 2 to 3 years after cataract extraction, but before IOL implantation. Surgical removal of a visual axis opacification via a pars plana vitrectomy (2 patients) or an anterior approach (3 patients) was performed 3 months to 1 year after
Journal of AAPOS
Volume 20 Number 1 / February 2016
Tadros, Trivedi, and Wilson
Table 2. Complications following cataract surgery in infants \7 months of age with IOL implantation (primary group) or receiving an IOL as a secondary procedure (secondary group) Primary group (n 5 13)
Type of complication Glaucoma Surgery Medical Glaucoma suspect Visual axis opacification surgery Uveitis IOL exchange/removal Planned piggyback IOL removal Strabismus surgery ET XT
Secondary group (n 5 13)
After CS After CS with but before After later IOL implant IOL implant IOL implant 2 0 0 5
2 1 2
1 0
3 3 exchange 3
0 0 0
0 1 removal 1
1 2
2 1
3 1
CS, cataract surgery. ET, esotropia; IOL, intraocular lens; XT, exotropia.
cataract surgery in the primary group (5 patients total). None of the secondary group patients developed opacification after secondary IOL implantation during followup; however, 2 patients in this group underwent surgery to remove a visual axis opacification via an anterior approach before IOL implantation 2-4 months after cataract extraction (Table 2). High myopia was diagnosed in 3 primary group eyes. An unplanned IOL exchange was performed in these 3 eyes 2-7 years after original IOL implantation. The myopia ranged from 7.5 D to 10 D prior to IOL exchange. Three primary group eyes required planned removal of the piggyback sulcus IOL 2-12 years after implantation. In the secondary group, 1 eye required planned removal of the secondary piggyback sulcus IOL 6 years after implantation, and 1 eye required an unplanned removal of the secondary IOL 3 years after implantation due to high myopia ( 11 D prior to IOL exchange). Patients in both groups underwent strabismus surgery; 3 patients in the primary group (1 for esotropia, 3 years after implantation; 2 for exotropia, 1-13 years after implantation, 1 of whom underwent 7 strabismus surgeries) and 7 patients in the secondary group (3 before IOL implantation [2 for esotropia and 1 for exotropia] and 4 after IOL implantation [3 for esotropia and 1 for exotropia]). At final follow-up, 8 patients in the primary group had no strabismus, 4 had esotropia, and 1 had exotropia; 5 patients in the secondary group had no strabismus, 2 had esotropia, and 6 patients had dissociated vertical deviation. The median visual acuity at final follow-up was 20/150 in both groups (range, counting fingers at 1 feet to 20/20). Refraction at final follow-up was 2.9 3.3 D and 1.8 2.5 D in the primary and secondary groups, respectively.
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The total number of additional intraocular surgeries after the initial cataract surgery was 13 for the primary group and 18 for the secondary group. These surgeries included 2 procedures after the cataract surgery but before the secondary IOL implantation, 13 secondary IOL implantation surgeries, and 3 surgeries performed after secondary IOL implantation.
Discussion This study found comparable long-term outcomes in unilateral cataracts operated on in infancy with both primary and secondary IOL implantation. Patients in both groups had early unilateral cataract removal with posterior capsulectomy and central anterior vitrectomy. Comparison of primary and secondary IOL implantation requires longterm follow-up with meticulous recording of any adverse effects. Including only patients that ultimately received an IOL allows direct comparison of the now-or-later decision that faces the surgeon when the cataract is removed in infancy. Studies that compare aphakia to pseudophakia will include patients who may never receive a secondary IOL. However, in the present long-term follow-up study we include only children who are pseudophakic and look at the advantages or disadvantages of placing the IOL after several years of aphakia. Note, to preserve the single surgeon, consecutive case series, all cases meeting inclusion criteria were analyzed. It is possible that 1 patient from the primary group and up to 5 from the secondary group may have also been included in the IATS. However, no data about secondary IOL implantation and outcomes after that procedure have been published on any of these patients. Our patients were followed closely for the development of signs of glaucoma. Some authors have theorized that primary IOL implantation reduces the risk of glaucoma.7 However, selection bias in these retrospective reports may confound the data that implies a protective effect of the IOL.8 In the recently published IATS 5-year outcome study,1 researchers showed that the incidence of glaucoma in a prospective randomized clinical trial was similar for infants implanted with an IOL (19%) compared to those left aphakic (16%). Our study used the same definitions of glaucoma and glaucomasuspect as established in the IATS. Glaucoma was diagnosed in 2 of 13 eyes (15%) in the primary group: both eyes required surgery. In the secondary group, 3 eyes (23%) developed glaucoma, with only 1 eye requiring glaucoma surgery; 1 additional eye became a glaucoma-suspect. From these data, it appears that a primary IOL implanted in infancy is not protective against the development of glaucoma. Choosing an IOL for implantation in very young infants requires special consideration because of the small size of the eye and the increased reactivity of the eye in infancy. Knight-Nanan and colleagues9 reported the occurrence of uveitis in only 7 of 24 eyes (29%) aged 1-22 months
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Tadros, Trivedi, and Wilson
undergoing surgery for congenital cataract with primary IOL implantation, all with a favorable outcome. Ram and colleagues10 reported the occurrence of significant fibrinous reaction in 4 of 45 eyes (9%) aged 3 weeks to 2 years with congenital cataract and primary IOL implantation. In our study, 3 patients (23%) in the primary group had moderate to marked uveitis that resolved spontaneously with frequent topical steroids. No patient in the secondary group had more than mild postoperative uveitis. Despite removal of the center of the posterior capsule and performance of an anterior vitrectomy, further surgery was required for postoperative visual axis opacification in 5 (38%) in the primary group and 2 (15.4%) in the secondary group (before IOL implantation). In the IATS,1 surgery was needed to clear the visual axis in 39 of 55 patients in the primary IOL group (68%) and in 8 of 57 patients in the contact lens group (14%). Based on previous studies,11,12 the higher reported incidence of opacification after IOL implantation is thought to arise because the IOL in the capsular bag prevents the anterior and posterior capsule leaflets from fusing together, thereby not sequestrating the proliferating lens epithelial cells as well as when the eye is aphakic and the capsule edges seal more effectively. Previous studies suggest that the presence of an IOL in early life may affect subsequent eye growth. Kora and colleagues13 reported a tendency for operated eyes with an IOL implanted to become myopic, because as axial length increases with growth the proportion occupied by the vitreous body (ie, posterior to the IOL) also increases. Other studies suggest that pseudophakic eyes undergo greater axial length elongation, and thus more myopic shift.14,15 The IATS published a longitudinal change in globe axial length after monocular cataract surgery at 1 year of age and found that operated eyes treated with contact lenses were 0.6 mm shorter than those treated with an IOL at 1 year of age.16 Median visual acuity at final follow-up was 20/150 in both groups in our study. This agrees with recently published IATS research.1 Compliance with postoperative treatment is expected to be a major factor in the outcome of visual rehabilitation in both groups, but perhaps even more in the secondary IOL group during the period when the child was left aphakic. Our study did not collect data on compliance with postoperative refractive treatment. We are planning to explore the myopic shift differences and variability between groups in future studies. Various reports have suggested that the frequency of strabismus depends on whether aphakia is treated with an IOL or contact lens. The IATS17 reported 66.7% strabismus among 38 pseudophakic patients after 12 months of follow-up, and 74.5% strabismus (mostly esotropia) among 42 infants treated with a contact lens; however, the difference was not statistically significant. In our study, strabismus surgery was required in 3 patients of the primary group (23%) and 7 patients in the secondary group (54%).
Volume 20 Number 1 / February 2016 Limitations of this study are the retrospective design and the relative small size of the two groups and the possibility that the selection of the secondary IOL implantation may have preferentially included those patients not succeeding with contact lens. However, in our practice, the vast majority of infants left aphakic are implanted at 4-7 years of age. This expectation is discussed with the parents at the time of initial surgery. They understand the choice at surgery to be one of now or later rather than IOL versus no IOL. Potential confounders related to visual outcome (eg, compliance to patching, compliance to prescribed refractive correction, age at secondary IOL implantation) are not accounted for in the analysis. Major strengths are the length of follow-up, the similarity of the patients in each group, and the uniformity in surgical technique due to the single surgeon. Of course, the results of this study may not be applicable in other settings. If compliance with future refractive correction is doubtful, it may be more appropriate to perform primary IOL implantation. In conclusion, our results suggest that either a primary IOL or a secondary IOL may be used in patients with unilateral infantile cataract. Both approaches can provide a long-term functional outcome. During 5 or more years of follow-up, additional surgeries and complications were found in both groups, and our data does not create a mandate for either treatment choice. It is likely that surgeons who perform cataract surgery on infants will offer both treatment options. Patient characteristics, surgeon experience, and available resources will determine the optimum treatment for each specific case.
Acknowledgments The authors thank Luanna R. Bartholomew, PhD, for critical review of the manuscript. References 1. Lambert SR, Lynn MJ, Hartmann EE, et al. Infant Aphakia Treatment Study Group. Comparison of contact lens and intraocular lens correction of monocular aphakia during infancy: a randomized clinical trial of HOTV optotype acuity at age 4.5 years and clinical findings at age 5 years. JAMA Ophthalmol 2014;132:676-82. 2. Vanderveen DK, Trivedi RH, Nizam A, Lynn MJ, Lambert SR, Infant Aphakia Treatment Study Group. Predictability of intraocular lens power calculation formulae in infantile eyes with unilateral congenital cataract: results from the Infant Aphakia Treatment Study. Am J Ophthalmol 2013;156:1252-60. 3. Trivedi RH, Wilson ME Jr. Selecting intraocular lens power in children. American Academy of Ophthalmology [Ophthalmic Pearls online]. Available at: http://www.aao.org; 2006. Accessed July 1, 2014. 4. Wilson ME, Bluestein EC, Wang XH, Apple DJ. Comparison of mechanized anterior capsulectomy and manual continuous capsulorhexis in pediatric eyes. J Cataract Refract Surg 1994;20:602-6. 5. Wilson ME, Saunders RA, Roberts EL, Apple DJ. Mechanized anterior capsulectomy as an alternative to manual capsulorhexis in children undergoing intraocular lens implantation. J Pediatr Ophthalmol Strabismus 1996;33:237-40. 6. Beck AD, Freedman SF, Lynn MJ, Bothun E, Neely DE, Lambert SR, Infant Aphakia Treatment Study Group. Glaucoma-related adverse
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7.
8.
9.
10.
11.
12.
events in the Infant Aphakia Treatment Study: 1-year results. Arch Ophthalmol 2012;130:300-305. Asrani S, Freedman S, Hasselblad V, et al. Does primary intraocular lens implantation prevent “aphakic” glaucoma in children? J AAPOS 2000;4:33-9. Trivedi RH, Wilson ME Jr, Gloub RL. Incidence and risk factors for glaucoma after pediatric cataract surgery with and without intraocular lens implantation. J AAPOS 2006;10:117-23. Knight-Nanan D, O’Keefe M, Bowell R. Outcome and complications of intraocular lenses in children with cataract. J Cataract Refract Surg 1996;22:730-36. Ram J, Brar GS, Kaushik S, Sukhija J, Bandyopadhyay S, Gupta A. Primary intraocular lens implantation in the first two years of life: safety profile and visual results. Indian J Ophthalmol 2007;55:185-9. Plager DA, Yang S, Neely D, Sprunger D, Sondhi N. Complications in the first year following cataract surgery with and without IOL in infants and older children. J AAPOS 2002;6:9-14. Trivedi RH, Wilson ME Jr, Bartholomew LR, Lal G, Peterseim MM. Opacification of the visual axis after cataract surgery and single acrylic
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13.
14.
15.
16.
17.
intraocular lens implantation in the first year of life. J AAPOS 2004;8: 156-64. Kora Y, Shimizu K, Inatomi M, Fukado Y, Ozawa T. Eye growth after cataract extraction and intraocular lens implantation in children. Ophthalmic Surg 1993;24:467-75. Hutchinson AK, Wilson ME, Saunders RA. Outcomes and ocular growth rates after intraocular lens implantation in the first 2 years of life. J Cataract Refract Surg 1998;24:846-52. Kugelberg M, Kugelberg U, Bobrova N, Tronina S, Zetterstr€ om C. After-cataract in children having cataract surgery with or without anterior vitrectomy implanted with a single-piece AcrySof IOL. J Cataract Refract Surg 2005;31:757-62. Lambert SR, Lynn MJ, DuBois LG, Cotsonis GA, Hartmann EE, Wilson ME, Infant Aphakia Treatment Study Groups. Axial elongation following cataract surgery during the first year of life in the infant Aphakia Treatment Study. Invest Ophthalmol Vis Sci 2012;53:7539-45. Bothun ED, Cleveland J, Lynn MJ, et al. One-year strabismus outcomes in the Infant Aphakia Treatment Study. Ophthalmology 2013;120:1227-31.
I put all the drops I am going to use on a fluorescein strip and have the child close her eyes and tilt her chin upward, gently moving the lashes away so the strip can contact the lid margin. The drops flow nicely by capillary action and most of the time children do well with the approach. Stiffer fluorescin strips are easier to work with than the flimsy ones. With the flimsy strips, the drops must be staged on the white part of the strip. The firmer strips allow staging of drops on the colored end of the strip. —Contributed by Kenneth P. Adams, DO, FAOCO, Albuquerque, New Mexico
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METHODS
To report outcomes after at least 5 years’ follow-up of consecutive patients at a single center undergoing unilateral congenital cataract surgery during infancy with primary intraocular lens (IOL) implantation at the time of surgery or undergoing secondary IOL implantation later in childhood. The medical records of pseudophakic children who received initial cataract surgery before 7 months of age were retrospectively reviewed. Children with acquired cataract, persistent fetal vasculature, congenital glaucoma, or follow-up of \5 years were excluded.
RESULTS
Mean age at time of cataract surgery was 2.7 months in the primary group (n 5 13) and 1.9 months in the secondary group (n 5 13). Mean age at IOL implantation in the latter group was 4.9 2.2 years. Mean age at final follow-up was 10.7 4.2 years in the primary group and 8.7 3.0 years in the secondary group. Glaucoma surgery was performed in 2 primary group eyes and 1 secondary group eye; it was medically controlled in 2 additional eyes of the latter group. One secondary group eye was diagnosed as glaucoma suspect. Visual axis opacification required surgery in 5 primary group eyes and 2 secondary group eyes before IOL implantation. Unplanned IOL exchange or removal for high myopia was required in 3 primary group eyes and 1 secondary group eye. Strabismus surgery was performed in 3 primary group eyes and 7 secondary group eyes. Median visual acuity at final follow-up was 20/150 in both groups. Refraction at last follow-up was 2.9 3.3 D in the primary and 1.8 2.5 D in the secondary group.
CONCLUSIONS
Reoperations were common in both groups over long-term follow-up. Close monitoring for glaucoma is also needed in both groups. ( J AAPOS 2016;20:25-29)
U
nilateral infantile cataracts can be managed with primary intraocular lens (IOL) implantation or secondary IOL implantation later in childhood, when IOL power prediction may be more reliable. The multicenter Infant Aphakia Treatment Study (IATS) was designed to compare the use of immediate IOL implantation for correction of aphakia, with the use of a contact lens after cataract surgery performed in infants 1-7 months with unilateral congenital cataract.1 The 5-year follow-up outcomes did not demonstrate visual benefit from implanting an IOL at the time of surgery and reported more adverse effects associated with primary IOL implantation.1
Author affiliations: aStorm Eye Institute, Medical University of South Carolina, Charleston, South Carolina; bOphthalmology Department, Faculty of Medicine, Tanta University, Egypt This research was supported in part by the Egyptian Cultural and Educational Bureau and an unrestricted grant to the Department of Ophthalmology, MUSC, from Research to Prevent Blindness, New York, NY. Submitted May 22, 2015. Revision accepted October 1, 2015. Correspondence: M. Edward Wilson, MD, Storm Eye Institute, Rm 218, Medical University of South Carolina, 127 Ashley Avenue, Charleston, SC 29401 (email: [email protected]). Copyright Ó 2016 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2015.10.010
Journal of AAPOS
The IATS did not include secondary IOL implantation outcomes. The present study compares single-center longterm outcomes (.5 years) following unilateral cataract surgery of infants receiving a primary IOL implantation and those undergoing secondary IOL implantation later in childhood, after an initial period of aphakia.
Subjects and Methods This study was approved by the Institutional Human Investigation Review Board of the Medical University of South Carolina and adheres to regulations of the Health Insurance Portability and Accountability Act of 1996. The medical records of consecutively operated children who underwent unilateral cataract surgery between 1995 and 2009 at the Storm Eye Institute were retrospectively reviewed. Pseudophakic eyes of children with a history of cataract surgery in one eye at \7 months of age were included. Eyes operated elsewhere, those with a noninfantile or acquired cataract (eg, post-traumatic, iatrogenic postnatal), retinopathy of prematurity, congenital glaucoma, persistent fetal vasculature with stretched ciliary processes, aphakic eyes with no IOL at the final visit and children with follow-up of \5 years after IOL implantation were excluded. All patients underwent keratometry (handheld KM 500 MARCO keratometer; Macro Ophthalmic Inc, Jacksonville,
25
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Tadros, Trivedi, and Wilson
FL) and axial length measurement by immersion A-scan (Ellex, Eden Prairie, MN) at the time of surgery. In addition, preoperative evaluation included intraocular pressure (IOP) measurement using a Tonopen (Tonopen XL, Reichert Inc., Depew, NY) and horizontal corneal diameter (using calipers). The type of cataract was noted by examining the eye under the operating microscope. A retinal fundus examination was performed if possible. When there was no view of the retina, a B-scan ultrasound was performed. The Holladay 1 formula was used to calculate IOL power.2 The target postoperative refraction was based on the patient’s age and biometry.3 One surgeon (MEW) performed all of the surgeries using posterior capsulectomy and vitrectomy techniques published previously.4,5 The infants were divided into two study groups: infants receiving IOL implantation at the time of cataract surgery (primary group) and those who did not undergo IOL implantation at the time of cataract surgery, requiring fitting of a contact lens or glasses when there was contact lens incompatibility, with secondary IOL implantation later in childhood (secondary group). Patients were selected for primary IOL implantation if compliance with contact lens wear was doubtful (ie, poor compliance with contact lens wear by another family member), if other social factors were likely to preclude adequate follow-up of contact lens management (eg, long travel time/distance for office visits, poor vision of parents or caregiver), or if the parents strongly preferred primary IOL implantation when given a choice of IOL now versus IOL later. The infants were then followed at 1 day, 1 week, 4 weeks, 3 months, and then at intervals of 3-6 months during the first year after surgery and once per year afterward. Glaucoma was defined as IOP .21 mm Hg plus one or more of the following anatomical changes: corneal enlargement, asymmetrical progressive myopic shift coupled with enlargement of the corneal diameter and/or axial length, increased optic nerve cupping (ie, an increase of 0.2 in the cup:disk ratio), or the use of a surgical procedure for IOP control.6 A patient was designated as a glaucoma suspect if there was either 2 consecutive IOP measurements .21 mm Hg on different dates after topical corticosteroids had been discontinued, but without any of the anatomical changes listed above, or glaucoma medication use was needed to control IOP, but without any of the anatomical changes noted above.6
Results A total of 99 infants underwent surgery at our center for unilateral cataract prior to 7 months of age. Of these, 26 met the inclusion criteria: 13 in the primary group and 13 in the secondary group. Excluded children included follow-up \5 years, persistent fetal vasculature, Peters anomaly, retinopathy of prematurity, corneal diameter \9 mm, and aphakia. Table 1 shows preoperative parameters and postoperative outcome of both groups. Secondary IOL implantation was performed at an average age of 4.9 years (median, 5 years; range, 0.8-9.4 years). Both groups were comparable at baseline in terms of age at cataract surgery, preoperative corneal diameter, preoperative axial length, age at last follow-up, and duration of followup after the original cataract surgery.
Volume 20 Number 1 / February 2016 Table 1. Preoperative parameters and follow-up of primary and secondary IOL implantation Category Number of eyes Mean age at cataract surgery, months No. #48 days at surgery Mean age at IOL implantation, years Sex Female Male Type of cataract Nuclear Cortical Posterior lentiglobus Posterior polar Anterior polar Mean preoperative axial length, mm Mean preoperative corneal diameter, mm Number of PB IOLsa Number of capsular bag/sulcus IOLs (excluding piggyback IOL) Duration between cataract surgery and last follow-up, years Mean age at last follow-up, years
Primary IOL implantation
Secondary IOL implantation
13 2.7 2.2
13 1.9 1.8
6 0.2 0.2
7 4.9 2.2
10 3
4 9
7 3 1 1 1 18.6 1.3 10.9 0.7
11 0 1 1 0 17.8 1.1 10.3 0.8
4 8/1
1 7/5
10.5 4.2
8.6 3
10.7 4.2
8.7 3
IOL, intraocular lens; PB, piggyback IOL. a Piggyback IOL 5 1 IOL in the capsular bag and 1 in the ciliary sulcus.
The targeted immediate postoperative refraction for primary single IOL implantation was 18 D when the patient was \2 months of age and 16 D for age at surgery of 27 months. For secondary IOL implantation, the targeted refraction was 12 D for 1- to 3-year-olds and plano for children operated on at age $4 years of age. For a piggyback IOL, the targeted immediate postoperative refraction was plano. Seven IOL models were used: AcrySof SA60AT, SN60WF, MA60BM (Alcon, Fort Worth, TX); Pharmacia 811A, 809A (Pharmacia & Upjohn, NY); Rayner 570CFlex (Rayner Intraocular Lenses Ltd, Hove, East Sussex, England); and MC60 (Alcon, Fort Worth, TX). IOLs were implanted either in the capsular bag or in the ciliary sulcus. When double IOL implantation was performed, an AcrySof SA60AT or MA60BM was implanted in the capsular bag, and an AcrySof MA60BM (Alcon, Fort Worth, TX) was implanted in the ciliary sulcus. Of the 13 patients in the primary group, 2 underwent glaucoma surgery: 1 received a Baerveldt glaucoma implant (Abbot Medical Optics, Abbott Park, IL); the other, an Ahmed glaucoma valve (New World Medical Inc, Rancho Cucamongo, CA). Of the 13 patients in the secondary group, 1 received an Ahmed glaucoma valve after IOL implantation. Two other secondary group patients developed glaucoma (controlled on antiglaucoma drugs) and 1 became a glaucoma suspect, 2 to 3 years after cataract extraction, but before IOL implantation. Surgical removal of a visual axis opacification via a pars plana vitrectomy (2 patients) or an anterior approach (3 patients) was performed 3 months to 1 year after
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Table 2. Complications following cataract surgery in infants \7 months of age with IOL implantation (primary group) or receiving an IOL as a secondary procedure (secondary group) Primary group (n 5 13)
Type of complication Glaucoma Surgery Medical Glaucoma suspect Visual axis opacification surgery Uveitis IOL exchange/removal Planned piggyback IOL removal Strabismus surgery ET XT
Secondary group (n 5 13)
After CS After CS with but before After later IOL implant IOL implant IOL implant 2 0 0 5
2 1 2
1 0
3 3 exchange 3
0 0 0
0 1 removal 1
1 2
2 1
3 1
CS, cataract surgery. ET, esotropia; IOL, intraocular lens; XT, exotropia.
cataract surgery in the primary group (5 patients total). None of the secondary group patients developed opacification after secondary IOL implantation during followup; however, 2 patients in this group underwent surgery to remove a visual axis opacification via an anterior approach before IOL implantation 2-4 months after cataract extraction (Table 2). High myopia was diagnosed in 3 primary group eyes. An unplanned IOL exchange was performed in these 3 eyes 2-7 years after original IOL implantation. The myopia ranged from 7.5 D to 10 D prior to IOL exchange. Three primary group eyes required planned removal of the piggyback sulcus IOL 2-12 years after implantation. In the secondary group, 1 eye required planned removal of the secondary piggyback sulcus IOL 6 years after implantation, and 1 eye required an unplanned removal of the secondary IOL 3 years after implantation due to high myopia ( 11 D prior to IOL exchange). Patients in both groups underwent strabismus surgery; 3 patients in the primary group (1 for esotropia, 3 years after implantation; 2 for exotropia, 1-13 years after implantation, 1 of whom underwent 7 strabismus surgeries) and 7 patients in the secondary group (3 before IOL implantation [2 for esotropia and 1 for exotropia] and 4 after IOL implantation [3 for esotropia and 1 for exotropia]). At final follow-up, 8 patients in the primary group had no strabismus, 4 had esotropia, and 1 had exotropia; 5 patients in the secondary group had no strabismus, 2 had esotropia, and 6 patients had dissociated vertical deviation. The median visual acuity at final follow-up was 20/150 in both groups (range, counting fingers at 1 feet to 20/20). Refraction at final follow-up was 2.9 3.3 D and 1.8 2.5 D in the primary and secondary groups, respectively.
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The total number of additional intraocular surgeries after the initial cataract surgery was 13 for the primary group and 18 for the secondary group. These surgeries included 2 procedures after the cataract surgery but before the secondary IOL implantation, 13 secondary IOL implantation surgeries, and 3 surgeries performed after secondary IOL implantation.
Discussion This study found comparable long-term outcomes in unilateral cataracts operated on in infancy with both primary and secondary IOL implantation. Patients in both groups had early unilateral cataract removal with posterior capsulectomy and central anterior vitrectomy. Comparison of primary and secondary IOL implantation requires longterm follow-up with meticulous recording of any adverse effects. Including only patients that ultimately received an IOL allows direct comparison of the now-or-later decision that faces the surgeon when the cataract is removed in infancy. Studies that compare aphakia to pseudophakia will include patients who may never receive a secondary IOL. However, in the present long-term follow-up study we include only children who are pseudophakic and look at the advantages or disadvantages of placing the IOL after several years of aphakia. Note, to preserve the single surgeon, consecutive case series, all cases meeting inclusion criteria were analyzed. It is possible that 1 patient from the primary group and up to 5 from the secondary group may have also been included in the IATS. However, no data about secondary IOL implantation and outcomes after that procedure have been published on any of these patients. Our patients were followed closely for the development of signs of glaucoma. Some authors have theorized that primary IOL implantation reduces the risk of glaucoma.7 However, selection bias in these retrospective reports may confound the data that implies a protective effect of the IOL.8 In the recently published IATS 5-year outcome study,1 researchers showed that the incidence of glaucoma in a prospective randomized clinical trial was similar for infants implanted with an IOL (19%) compared to those left aphakic (16%). Our study used the same definitions of glaucoma and glaucomasuspect as established in the IATS. Glaucoma was diagnosed in 2 of 13 eyes (15%) in the primary group: both eyes required surgery. In the secondary group, 3 eyes (23%) developed glaucoma, with only 1 eye requiring glaucoma surgery; 1 additional eye became a glaucoma-suspect. From these data, it appears that a primary IOL implanted in infancy is not protective against the development of glaucoma. Choosing an IOL for implantation in very young infants requires special consideration because of the small size of the eye and the increased reactivity of the eye in infancy. Knight-Nanan and colleagues9 reported the occurrence of uveitis in only 7 of 24 eyes (29%) aged 1-22 months
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undergoing surgery for congenital cataract with primary IOL implantation, all with a favorable outcome. Ram and colleagues10 reported the occurrence of significant fibrinous reaction in 4 of 45 eyes (9%) aged 3 weeks to 2 years with congenital cataract and primary IOL implantation. In our study, 3 patients (23%) in the primary group had moderate to marked uveitis that resolved spontaneously with frequent topical steroids. No patient in the secondary group had more than mild postoperative uveitis. Despite removal of the center of the posterior capsule and performance of an anterior vitrectomy, further surgery was required for postoperative visual axis opacification in 5 (38%) in the primary group and 2 (15.4%) in the secondary group (before IOL implantation). In the IATS,1 surgery was needed to clear the visual axis in 39 of 55 patients in the primary IOL group (68%) and in 8 of 57 patients in the contact lens group (14%). Based on previous studies,11,12 the higher reported incidence of opacification after IOL implantation is thought to arise because the IOL in the capsular bag prevents the anterior and posterior capsule leaflets from fusing together, thereby not sequestrating the proliferating lens epithelial cells as well as when the eye is aphakic and the capsule edges seal more effectively. Previous studies suggest that the presence of an IOL in early life may affect subsequent eye growth. Kora and colleagues13 reported a tendency for operated eyes with an IOL implanted to become myopic, because as axial length increases with growth the proportion occupied by the vitreous body (ie, posterior to the IOL) also increases. Other studies suggest that pseudophakic eyes undergo greater axial length elongation, and thus more myopic shift.14,15 The IATS published a longitudinal change in globe axial length after monocular cataract surgery at 1 year of age and found that operated eyes treated with contact lenses were 0.6 mm shorter than those treated with an IOL at 1 year of age.16 Median visual acuity at final follow-up was 20/150 in both groups in our study. This agrees with recently published IATS research.1 Compliance with postoperative treatment is expected to be a major factor in the outcome of visual rehabilitation in both groups, but perhaps even more in the secondary IOL group during the period when the child was left aphakic. Our study did not collect data on compliance with postoperative refractive treatment. We are planning to explore the myopic shift differences and variability between groups in future studies. Various reports have suggested that the frequency of strabismus depends on whether aphakia is treated with an IOL or contact lens. The IATS17 reported 66.7% strabismus among 38 pseudophakic patients after 12 months of follow-up, and 74.5% strabismus (mostly esotropia) among 42 infants treated with a contact lens; however, the difference was not statistically significant. In our study, strabismus surgery was required in 3 patients of the primary group (23%) and 7 patients in the secondary group (54%).
Volume 20 Number 1 / February 2016 Limitations of this study are the retrospective design and the relative small size of the two groups and the possibility that the selection of the secondary IOL implantation may have preferentially included those patients not succeeding with contact lens. However, in our practice, the vast majority of infants left aphakic are implanted at 4-7 years of age. This expectation is discussed with the parents at the time of initial surgery. They understand the choice at surgery to be one of now or later rather than IOL versus no IOL. Potential confounders related to visual outcome (eg, compliance to patching, compliance to prescribed refractive correction, age at secondary IOL implantation) are not accounted for in the analysis. Major strengths are the length of follow-up, the similarity of the patients in each group, and the uniformity in surgical technique due to the single surgeon. Of course, the results of this study may not be applicable in other settings. If compliance with future refractive correction is doubtful, it may be more appropriate to perform primary IOL implantation. In conclusion, our results suggest that either a primary IOL or a secondary IOL may be used in patients with unilateral infantile cataract. Both approaches can provide a long-term functional outcome. During 5 or more years of follow-up, additional surgeries and complications were found in both groups, and our data does not create a mandate for either treatment choice. It is likely that surgeons who perform cataract surgery on infants will offer both treatment options. Patient characteristics, surgeon experience, and available resources will determine the optimum treatment for each specific case.
Acknowledgments The authors thank Luanna R. Bartholomew, PhD, for critical review of the manuscript. References 1. Lambert SR, Lynn MJ, Hartmann EE, et al. Infant Aphakia Treatment Study Group. Comparison of contact lens and intraocular lens correction of monocular aphakia during infancy: a randomized clinical trial of HOTV optotype acuity at age 4.5 years and clinical findings at age 5 years. JAMA Ophthalmol 2014;132:676-82. 2. Vanderveen DK, Trivedi RH, Nizam A, Lynn MJ, Lambert SR, Infant Aphakia Treatment Study Group. Predictability of intraocular lens power calculation formulae in infantile eyes with unilateral congenital cataract: results from the Infant Aphakia Treatment Study. Am J Ophthalmol 2013;156:1252-60. 3. Trivedi RH, Wilson ME Jr. Selecting intraocular lens power in children. American Academy of Ophthalmology [Ophthalmic Pearls online]. Available at: http://www.aao.org; 2006. Accessed July 1, 2014. 4. Wilson ME, Bluestein EC, Wang XH, Apple DJ. Comparison of mechanized anterior capsulectomy and manual continuous capsulorhexis in pediatric eyes. J Cataract Refract Surg 1994;20:602-6. 5. Wilson ME, Saunders RA, Roberts EL, Apple DJ. Mechanized anterior capsulectomy as an alternative to manual capsulorhexis in children undergoing intraocular lens implantation. J Pediatr Ophthalmol Strabismus 1996;33:237-40. 6. Beck AD, Freedman SF, Lynn MJ, Bothun E, Neely DE, Lambert SR, Infant Aphakia Treatment Study Group. Glaucoma-related adverse
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events in the Infant Aphakia Treatment Study: 1-year results. Arch Ophthalmol 2012;130:300-305. Asrani S, Freedman S, Hasselblad V, et al. Does primary intraocular lens implantation prevent “aphakic” glaucoma in children? J AAPOS 2000;4:33-9. Trivedi RH, Wilson ME Jr, Gloub RL. Incidence and risk factors for glaucoma after pediatric cataract surgery with and without intraocular lens implantation. J AAPOS 2006;10:117-23. Knight-Nanan D, O’Keefe M, Bowell R. Outcome and complications of intraocular lenses in children with cataract. J Cataract Refract Surg 1996;22:730-36. Ram J, Brar GS, Kaushik S, Sukhija J, Bandyopadhyay S, Gupta A. Primary intraocular lens implantation in the first two years of life: safety profile and visual results. Indian J Ophthalmol 2007;55:185-9. Plager DA, Yang S, Neely D, Sprunger D, Sondhi N. Complications in the first year following cataract surgery with and without IOL in infants and older children. J AAPOS 2002;6:9-14. Trivedi RH, Wilson ME Jr, Bartholomew LR, Lal G, Peterseim MM. Opacification of the visual axis after cataract surgery and single acrylic
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intraocular lens implantation in the first year of life. J AAPOS 2004;8: 156-64. Kora Y, Shimizu K, Inatomi M, Fukado Y, Ozawa T. Eye growth after cataract extraction and intraocular lens implantation in children. Ophthalmic Surg 1993;24:467-75. Hutchinson AK, Wilson ME, Saunders RA. Outcomes and ocular growth rates after intraocular lens implantation in the first 2 years of life. J Cataract Refract Surg 1998;24:846-52. Kugelberg M, Kugelberg U, Bobrova N, Tronina S, Zetterstr€ om C. After-cataract in children having cataract surgery with or without anterior vitrectomy implanted with a single-piece AcrySof IOL. J Cataract Refract Surg 2005;31:757-62. Lambert SR, Lynn MJ, DuBois LG, Cotsonis GA, Hartmann EE, Wilson ME, Infant Aphakia Treatment Study Groups. Axial elongation following cataract surgery during the first year of life in the infant Aphakia Treatment Study. Invest Ophthalmol Vis Sci 2012;53:7539-45. Bothun ED, Cleveland J, Lynn MJ, et al. One-year strabismus outcomes in the Infant Aphakia Treatment Study. Ophthalmology 2013;120:1227-31.
I put all the drops I am going to use on a fluorescein strip and have the child close her eyes and tilt her chin upward, gently moving the lashes away so the strip can contact the lid margin. The drops flow nicely by capillary action and most of the time children do well with the approach. Stiffer fluorescin strips are easier to work with than the flimsy ones. With the flimsy strips, the drops must be staged on the white part of the strip. The firmer strips allow staging of drops on the colored end of the strip. —Contributed by Kenneth P. Adams, DO, FAOCO, Albuquerque, New Mexico
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