ABSTRACT. Sorath Noorani Siddiqui, FCPS; Ayesha Khan, FCPS, FRCS

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1 Visual Outcome and Changes in Corneal Endothelial Cell Density Following Aphakic Iris-Fixated Intraocular Lens Implantation in Pediatric Eyes With Subluxated Lenses Sorath Noorani Siddiqui, FCPS; Ayesha Khan, FCPS, FRCS ABSTRACT Purpose: To evaluate the visual outcome and corneal endothelial cell density after Artisan aphakic intraocular lens (IOL) implantation (Ophtec, Groningen, the Netherlands) in pediatric eyes with subluxated lenses. Methods: Artisan aphakic IOLs were implanted in 18 eyes of 11 children with subluxated lenses. Idiopathic subluxations and ectopia lentis due to Marfan syndrome were included, whereas subluxations due to trauma or buphthalmos were excluded. Best-corrected visual acuity (BCVA) and endothelial cell density were monitored. Mean postoperative BCVA and endothelial cell density at last follow-up visit were calculated. Results: The age of children at the time of Artisan aphakic IOL implantation ranged from 8 to 16 years (mean: ± 2.9 years). Mean follow-up was 9.12 ± 4.30 months. Mean postoperative logarithm of the minimum angle of resolution BCVA was 0.26 ± 0.13 (P =.001) and mean postoperative endothelial cell density was 2,860 ± 435 cells/mm 2 (P =.000). Mean endothelial cell loss was 17.1%. Conclusion: Artisan aphakic IOL implantation is a safe surgical choice in the management of ectopia lentis in the pediatric age group. It has minimal complications and is less traumatic to pediatric eyes. However, longterm follow-up of these children is required. [J Pediatr Ophthalmol Strabismus 2013;50(3): ] INTRODUCTION Berryat described the first reported case of lens dislocation in 1749 and Stellwag subsequently coined the term ectopia lentis in Subluxated lenses may occur as an isolated ocular finding or associated with a systemic disorder such as Marfan syndrome, homocystinuria, Weill Marchessani syndrome, and Ehlers Danlos syndrome. Familial or idiopathic ectopia lentis is not associated with systemic abnormalities. Surgical intervention is required when best-corrected visual acuity (BCVA) is not achieved with optical correction. In the past, we have used other surgical options to manage subluxated lenses in pediatric eyes such as simple lensectomy followed by aphakic glasses for optical correction, use of capsular tension ring, and scleral fixation of posterior chamber intraocular lens (IOL) in selected cases. In our country, this is the first time the Artisan aphakic IOL has been used in pediatric eyes. We From the Department of Pediatric Ophthalmology and Strabismus (SNS), Al-Shifa Trust Eye Hospital, Rawalpindi, Pakistan; and the Department of Pediatric Ophthalmology and Strabismus (AK), Montreal Children Hospital, Montreal, Canada. Submitted: April 5, 2012; Accepted: October 15, 2012; Posted online: January 15, 2013 The authors have no financial or proprietary interest in the materials presented herein. Correspondence: Sorath Noorani Siddiqui, FCPS, 46/II Main Khayaban-e-Shamsheer, Phase 5 Defence Housing Authority, Karachi 75500, Pakistan. sorathnoorani@yahoo.com doi: / Copyright SLACK Incorporated

2 TABLE 1 Preoperative and Postoperative BCVA and ECD (N = 17) Eye Patient Age (y) Follow-up (mo) Preop BCVA Snellen Postop BCVA Snellen Preop ECD (cells/mm 2 ) Postop ECD at Last Follow-up Complications a / /40 3,381 3,262 None /114 20/40 3,446 2,760 Rise in IOP /80 20/60 3,750 2,904 IOL disengaged due to trauma /114 20/25 2,974 2,877 None / /20 3,117 1,773 None /114 20/30 3,443 3,210 Endophthalmitis /80 20/30 3,201 2,990 None /80 20/30 3,270 2,658 Horizontally oval /80 20/40 3,718 3,005 None /114 20/40 3,283 3,016 None /80 20/60 3,616 3,095 None /200 20/60 3,534 2,859 None / /30 2,899 1,812 Horizontally oval /40 20/30 3,379 3,010 None /40 20/40 3,837 2,957 Horizontally oval /80 20/40 3,897 3,285 Horizontally oval /114 20/40 3,905 3,150 None BCVA = best-corrected visual acuity; ECD = endothelial cell density; preop = preoperative; postop = postoperative; IOP = intraocular pressure; IOL = intraocular lens a Iris depigmentation was seen at fixation sites in all eyes. share our experience of lensectomy followed by Artisan iris-fixated aphakic IOL (Ophtec, Groningen, the Netherlands) implantation in eyes of children with ectopia lentis. PATIENTS AND METHODS This prospective study was performed in the Pediatric Ophthalmology unit of our tertiary care center from December 2010 to February Approval was obtained from the ethical review committee of our institution. Informed written consent was obtained from the parents or guardians of the children. Eighteen eyes of 11 children aged 8 years and older were included in our study. In this study, the indication for surgery was BCVA 20/60 or worse except in eyes 14 and 15 with preoperative BCVA of 20/40 as shown in Table 1. In eye 14, the patient and his parents opted for surgery because he had remarkable postoperative improvement in visual acuity after Artisan aphakic IOL implantation in his fellow eye (eye 7 in Table 1). Eye 15 had familial ectopia lentis and his sibling had ocular complications due to untreated ectopia lentis, so the parents chose surgery. The follow-up period ranged between 5 and 14 months. Secondary subluxations due to buphthalmos or trauma were excluded. Evaluation of patients included detailed history, BCVA (phakic or aphakic), slit-lamp examination, and intraocular pressure recorded by Goldmann applanation tonometer. Dilated funduscopy was performed on all patients. Preoperative and postoperative endothelial cell count was recorded using noncontact specular microscopy (SP2000P; Topcon Medical Systems, Oakland, CA). Routine investigations and consultation for general anesthesia and cardiac assessment of patients with Marfan syndrome were advised. All eyes were Journal of Pediatric Ophthalmology & Strabismus Vol. 50, No. 3,

3 implanted with the Artisan aphakic IOL, a polymethylmethacrylate lens with a diameter of 8.5 mm including the haptics. Haptics grasp the peripheral iris for fixation and centration. Surgical Technique Under general anesthesia, full aseptic measures were taken. A sclerocorneal tunnel was made at the 12-o clock position after making two ports at the 10- and 2-o clock positions. Lensectomy and anterior vitrectomy was performed using the Bausch and Lomb Millennium system (Rochester, NY). After lensectomy, carbachol 0.01% was used to constrict the and peripheral iridectomy was performed. The Artisan aphakic IOL was introduced into the anterior chamber with lens-holding forceps (Ophtec) and fixed to the iris with a specially designed enclavation needle (Ophtec). The anterior chamber was washed and the wound and ports were closed with 10-0 polyglactin interrupted sutures. Intracameral 0.1 ml moxifloxacin hydrochloride 0.5% (Vigamox; Alcon Laboratories, Inc., Fort Worth, TX) and dexamethasone 0.4 mg were given. Postoperative Care and Follow-up Antibiotic steroid drops were used and tapered after 4 weeks. Improvement in BCVA, intraocular pressure, IOL positioning, anterior and posterior segment examination, and specular microscopy were recorded on follow-up visits. Statistical Analysis Data analysis was performed using SPSS for Windows software (SPSS, Inc., Chicago, IL). Mean ± standard deviations were calculated. The paired t test was used and differences were considered statistically significant when the P value was less than.05. RESULTS Artisan aphakic IOLs were implanted in 18 eyes of 11 children with subluxated lenses. Seven (63.6%) were female and 4 (36.4%) were male. Five (45.5%) children had Marfan syndrome and 6 (54.5%) had idiopathic ectopia lentis. One patient who had unilateral Artisan aphakic IOL implantation was lost to follow-up in the early postoperative period and hence excluded from the study. The age of the children at the time of IOL implantation ranged from 8 to 16 years (mean: ± 2.9 years). Mean follow-up was 9.12 ± 4.30 months (range: 5 to 14 months). Mean preoperative logarithm of the minimum angle of resolution (logmar) BCVA was 0.88 ± 0.55 and mean postoperative logmar BCVA was 0.26 ± 0.13 (P =.001). Mean preoperative endothelial cell count was 3,450 ± 308 cells/mm 2 and mean postoperative endothelial cell count was 2,860 ± 435 cells/mm 2 (P =.000). Mean endothelial cell loss was 17.1%. Table 1 shows preoperative and postoperative BCVA and endothelial cell density in 17 eyes. Surgeons were experienced in performing complicated anterior segment procedures and had performed prior wet lab for Artisan aphakic IOL implantation. The learning curve was short and there were no intraoperative complications encountered during the procedure. In the current study, transient rise in intraocular pressure was noticed in one eye (5.9%) and resolved with beta-blocker treatment, remaining within normal limits after discontinuing antiglaucoma drops. A transient horizontally oval shape was seen in 4 (23.5%) eyes on the first postoperative day and resolved in a week. Iris depigmentation was found at the sites of enclavation in all eyes. One eye (5.9%) had endophthalmitis 2 weeks after surgery. Traumatic disengagement of one haptic of the Artisan aphakic IOL was seen in 1 eye (5.9%). The patient had blunt trauma to her right eye due to an accidental fist blow from her younger sibling. Reenclavation of the IOL was performed successfully. Her endothelial cell count was 2,904 cell/mm 2 7 months after re-enclavation. DISCUSSION We examine a large number of children with subluxated lenses at our tertiary eye care center. In , we performed 54 lensectomies for subluxated lenses and another 28 eyes were prescribed glasses for optical correction. 2 Dealing with such a large number of pediatric eyes with subluxated lenses compels us to apply a safe surgical option for these growing eyes. Our concern is to make them pseudophakic and to provide them with better quality vision. In 2007, we published a study on the management of ectopia lentis in children and performed lensectomy followed by optical correction with aphakic glasses. 2 We believe that lensectomy followed by aphakic glasses is a safe procedure in pediatric eyes but not an ideal option because aphakic glasses have their own disadvantages. Aphakic glasses are heavy and cosmetically unacceptable, and 180 Copyright SLACK Incorporated

4 produce prismatic effects and image magnification. In our institution, scleral fixation of IOL has been performed commonly in adults but its application in pediatric eyes is limited. Buckley 3 reported on scleral fixation of IOL in children and found an increased rate of complications. BenEzra 4 experienced suture erosion and dislocation of IOL 3 years after scleral fixation. Moreover, scleral fixation of IOL is a time-consuming procedure and involves more surgical manipulation to pediatric eyes. Long-term stability of scleral fixation of IOL depends entirely on the strength of the 9-0 or 10-0 polypropylene suture. The biodegradability of polypropylene sutures is a major concern associated with this procedure. Anterior chamber IOL implantation is an alternative method; a study 4 showed high intraocular pressure, corneal decompensation, or both developed in 80% of children after follow-up of 10 years following anterior chamber IOL implantation. Due to the above-mentioned vision-threatening complications, we do not recommend anterior chamber IOL implantation in the pediatric age group. Vasavada et al. 5 reported on use of the Cionni intracapsular tension ring for posterior chamber IOL implantation. They found posterior capsular opacification in 60%, IOL decentration in 8.5%, cystoid macular edema in 6%, and chronic uveitis in 6%. We believe that use of the Cionni intracapsular tension ring for posterior chamber IOL implantation is a meticulous and technically difficult procedure and requires a highly experienced surgeon. The Cionni intracapsular tension ring is associated with high cost and prolonged surgical time. It is performed at only a few specialized tertiary care centers in our country. In 1978, Jan Worst and Fechner invented the Artisan iris claw aphakic IOL. 6 The lens was known as the Worst Fechner claw lens. Van der Pol and Worst 7 reported the use of the Artisan aphakic IOL in children with cataract. Gabor et al. 8 described the use of the Artisan aphakic IOL in adult eyes with ectopia lentis. In the current study, we observed significant improvement in BCVA postoperatively in 16 eyes and 1 eye (eye 15 in Table 1) showed the same visual acuity postoperatively. Our mean postoperative logmar BCVA was 0.26 ± (P =.001). Lifshitz et al. 9 found similar results in their small retrospective study; BCVA was 6/12 (logmar 0.30) or better in 3 of 4 children who had Artisan aphakic IOL implantation due to subluxated lenses. Cleary et al. 10 also observed significant improvement in visual acuity in their series of 8 eyes of 5 children with subluxated lenses, with mean logmar postoperative visual acuity of 0.04 ± Sminia et al. 11 used the Artisan aphakic IOL in children with bilateral cataract. In their retrospective study, 13 eyes improved to 6/9 (logmar 0.18) or better and 3 eyes were 6/12 (logmar 0.30). In our series, 14 eyes showed 20/40 (6/12) or better visual acuity (Table 1). The current study and the data available in the above-mentioned studies suggested good visual outcome with Artisan aphakic IOL implantation in pediatric eyes. A major concern with Artisan aphakic IOL implantation in children is its effect on corneal endothelial cells. In our study, the postoperative mean endothelial cell count at last follow-up was 2,860 ± 435 cells/mm 2 (P =.000) and the mean endothelial cell loss was 17.1%. Our mean postoperative endothelial loss is slightly higher due to a significant drop in endothelial cell density shown in both eyes of a patient (eyes 5 and 13 in Table 1). This patient showed dramatic improvement in postoperative visual acuity in both eyes. Surgery was unremarkable in both eyes. Postoperatively, both corneas were normal and no reaction was seen in the anterior chamber of both eyes during the follow-up period. We could not attribute any intraoperative or postoperative cause to the drop in endothelial cell density in this patient. Cleary et al. 10 showed postoperative mean endothelial cell count of 2,913 ± 268 cells and mean cell loss of 14.2% (P <.001). A study 9 reported that the children in their series were uncooperative for preoperative endothelial cell density count and they compared the endothelial cell density of the eye that was operated on with that of the eye that was not during the follow-up period. Their study did not show endothelial cell loss. Nucci et al. 12 studied normal endothelial cell density in children and reported that the mean ± standard deviation value ranged from 3,591 ± 399 cells/mm 2 at age 5 years to 2,697 ± 246 cells/mm 2 for older subjects. Their results showed decrease in cell density of 13% between ages 5 and 7 years and an additional decrease of 12% by age 10 years. Our mean endothelial cell density and endothelial cell loss is within the range of values reported in the literature in this age group. Urban et al. 13 observed corneal endothe- Journal of Pediatric Ophthalmology & Strabismus Vol. 50, No. 3,

5 lial changes after pediatric cataract surgery in patients 9 to 19 years old (mean: 12.9 years). They showed postoperative mean endothelial cell density of 2,639.2 and 2,479.9 cells/mm 2 after 6 and 12 months, respectively. Mean endothelial cell loss in their study was 22.68% at 12 months after surgery. In our series, postoperative mean endothelial cell density is slightly better than the mean postoperative endothelial cell density after routine pediatric cataract surgery shown in the above study. 13 Moreover, our mean endothelial cell loss of 17.1% is much less than that seen after cataract surgery in the study by Urban et al. 13 In the current study, a standard deviation of 435 cells/mm 2 was seen in postoperative endothelial cell density. Similar results are reported by Odenthal et al. 14 with a standard deviation of 410 cells/ mm 2 in their series of Artisan aphakic IOL implantation in 3 eyes with unilateral cataract. Another study 11 observed a standard deviation of 800 cells/ mm 2, which is higher than our study. Cleary et al. 10 found a standard deviation of 268 cells/mm 2. Basti et al. 15 studied endothelial cell loss after cataract surgery with posterior chamber IOL implantation and anterior vitrectomy in children and found postoperative endothelial cell density had a standard deviation of 400 cells/mm 2. The standard deviation of postoperative endothelial cell density in our study is comparable to the values shown after routine cataract surgery with posterior chamber IOL implantation in children as reported in the above-mentioned study. 15 In our study, we observed that the Artisan aphakic IOL has an impact on corneal endothelial cell count similar to routine cataract surgery with posterior chamber IOL shown in studies. 13,15 Several studies 9-11,16,17 suggest that the Artisan aphakic IOL is safe for children. Similarly, in our study, no cases of hyphema, glaucoma, or corneal decompensation were seen. One eye developed endophthalmitis. The patient had 1 mm of hypopyon and it was successfully treated with aggressive topical and periocular fortified antibiotics as per the protocol of our center. 18 In one eye, the IOL haptic was disengaged from the iris due to blunt trauma and was successfully re-enclaved. We did not encounter any difficulty during the reoperation. We conclude that the Artisan aphakic IOL is particularly beneficial in our institutions because we deal with a large number of patients with ectopia lentis. Keeping in view the long life span of children, we believe that Artisan aphakic IOL implantation is safe for pediatric eyes because it is easy to perform and less time-consuming and carries a lesser rate of complications compared to angle-supported anterior chamber IOLs, scleral fixation of posterior chamber IOL or intracapsular tension ring, and posterior chamber IOL implantation. However, safety and efficacy needs to be established in long-term studies and multicenter trials. REFERENCES 1. Eifring CW. Ectopia lentis. Available at: 2. Noorani S, Khan A, Rubab S, Choudhary KA. Management of ectopia lentis in children. Pak J Ophthalmol. 2007;23: Buckley EG. Scleral fixated (sutured) posterior chamber IOL implantation in children. J AAPOS. 1999;3: BenEzra D. IOLs for unilateral pediatric aphakia: early lenses and long-term follow-up. Eur J Implant Refractive Surg. 1990;2: Vasavada V, Vasavada VA, Hoffman RO, et al. 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J Cataract Refract Surg. 2011;37: Nucci P, Brancato R, Mets MB, Shevell SK. Normal endothelial cell density range in childhood. Arch Ophthalmol. 1990;108: Urban B, Bakunowicz-lazarczyk A, Kretowska M. Evaluation of corneal endothelium after pediatric cataract surgery in children and adolescent. Klin Ocnza. 2005;107: Odenthal MT, Sminia ML, Prick LJ, Gortazek-Moorstein MN, Volker-Dieben HJ. Long-term follow-up of the corneal endothelium after artisan lens implantation for unilateral traumatic and unilateral congenital cataract in children: two case series. Cornea. 2006;25: Basti S, Aasuri MK, Reddy S, Rao GN. Prospective evaluation of corneal endothelial cell loss after pediatric cataract surgery. J Cataract Refract Surg. 1998;24: Aspiotis M, Asproudis I, Stefaniotou M, et al. Artisan aphakic intraocular lens implantation in cases of subluxated crystalline lenses due to Marfan syndrome J Refract Surg. 2006;22: Sminia ML, Odenthal MT, Prick LJ, Cobben JM, Mourits MP, Völker-Dieben HJ. Long-term follow-up after bilateral Artisan aphakia intraocular lens implantation in two children with Marfan syndrome. J AAPOS. 2012;16: Ishaq N. Al-Shifa Endophthalmitis Study: protocol of treatment and prognosis. Al-Shifa J Ophthalmol. 2005;1: Copyright SLACK Incorporated

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