Sutureless Artificial Iris after Phacoemulsification in

1040-5488/15/924S-0S36/0 VOL. 92, NO. 4S, PP. S36YS39 OPTOMETRY AND VISION SCIENCE Copyright * 2015 American Academy of Optometry

CLINICAL CASE

Sutureless Artificial Iris after Phacoemulsification in Congenital Aniridia Ester Fernańdez-Lo´pez*, Francisco Pastor Pascual†, Marta Pe´rez-Lo´pez†, Alejandro Madrigal Quevedo*, and Cristina Peris Martıńez†

ABSTRACT Purpose. This article reports the first case of a sutureless artificial iris prosthesis used in combination with cataract surgery for congenital aniridia with successful visual and cosmetic results. Case Report. A 15-year-old woman with congenital bilateral partial aniridia, cataracts, and intense photophobia presented to the Cornea and Refractive Surgery Unit of the Ophthalmology Department. She was managed with an artificial iris implant (ArtificialIris, Dr. Schmidt Intraocularlinsen GmbH, Human Optics) fixed in the ciliary sulcus without any sutures after small-incision cataract surgery. At the 1-year follow-up, subjective complaints of glare and photophobia as well as binocular near visual acuity improved significantly. The cosmetic result was excellent. No postoperative complications have been recorded within this period. Conclusions. The ArtificialIris is a promising device for treating photophobia in congenital aniridia. ArtificialIris does not require suture fixation with adequate capsular support and iris remnants. (Optom Vis Sci 2015;92:S36YS39) Key Words: artificial iris, iris prosthesis, aniridia, photophobia, iris implant

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ongenital aniridia is a rare disorder that is associated with cataracts in 50 to 85% of patients.1 Sensory nystagmus owing to impaired fixation in the context of foveal hypoplasia is present in up to 75% of patients. Cataract surgery in these cases represents a therapeutic challenge because of photophobia, glare, loss of contrast sensitivity, diplopia, and low visual acuity after phacoemulsification. This may limit the postoperatory visual outcome. Several therapeutic options including pigmented contact lenses,2 corneal tattooing,3 segmental iris rings,4 or iris diaphragm lenses5Y7 have been described to correct posttraumatic aniridia and less frequently congenital aniridia. Recently, a new foldable custom-made artificial iris prosthesis (ArtificialIris, Dr. Schmidt Intraocularlinsen GmbH, Human Optics) has been developed. It has been reported to be useful in treating mainly traumatic aniridias.3Y7 We report the case of a patient with congenital aniridia, bilateral cataracts, and sensory nystagmus who underwent implantation of this new foldable custom-made Gore-Tex artificial iris that was placed under the peripheral remnant iris after cataract surgery in both eyes. To the authors’ knowledge, after a comprehensive

*MD † MD, PhD FISABIO Oftalmologıá Me´dica, Valencia, Spain (all authors); and Newcastle upon Tyne NHS Hospital, Newcastle, United Kingdom (MPL).

literature search, this is the first reported case of congenital aniridia treated bilaterally with an artificial iris device without any sutures in combination with cataract surgery.

CASE REPORT A 15-year-old woman with congenital aniridia referred to the Cornea and Refractive Surgery Unit of the Ophthalmology Department presented with progressive vision loss in both eyes over the past year. The patient complained of intense photophobia and glare. Distance best-corrected visual acuity was 20/125 (Snellen) in her right eye (OD) and 20/200 in her left eye (OS), with a near binocular visual acuity of 20/40. She refracted to OD +0.50 j1.50 007 and OS +1.00 j1.00 175. Sensory horizontal and rotatory nystagmus without blocking position but decreased frequency and amplitude in convergence was present. Neither movement nor visual acuity improved using prisms. Hirschberg testing showed about 5 degrees of deviation, and suppression of the OS was seen in a vectograph test. Slit-lamp examination showed clear corneas, partial bilateral congenital aniridia, and cortical cataract C4 in the OD and C5 in the OS according to the Lens Opacities Classification System scale8 (Fig. 1). Nasal conjunctival scars were also noted in both eyes owing to bilateral medial rectus recession performed in childhood for esotropia correction. Intraocular pressure was 16 mm Hg OD and OS by

Optometry and Vision Science, Vol. 92, No. 4S, April 2015

Copyright © American Academy of Optometry. Unauthorized reproduction of this article is prohibited.

Sutureless Artificial Iris after Phacoemulsification in AniridiaVFernańdez-Lo´pez et al.

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FIGURE 1. Slit-lamp photographs and OCT-Visante images of both eyes before the cataract surgery and artificial iris implant. Dense cortical cataract is noticed bilaterally and remnant iris can be identified.

Goldmann applanation tonometry. Funduscopy showed lack of foveolar reflex in both eyes owing to foveal hypoplasia. After informed consent was obtained from the patient, bilateral sequential cataract surgery plus artificial iris implantation was planned. To determine the diameter of the artificial iris, we relied on measurement of white-to-white corneal horizontal diameter with a compass and OCT-Visante adding 0.5 mm; we then implanted an 11-mm-diameter iris prosthesis in each eye according to the manufacturer’s recommendations. Because the standard diameter of the artificial iris is 12.8 mm, the prosthesis was cut using an 11-mm trephine (Hessburg-Barron). A superior triangular peripheral iridectomy was also performed in the prosthesis before implanting it. The OS was operated on first under peribulbar anesthesia in August 2012, followed by the OD in September 2012. A 2.8-mm clear corneal incision was performed and three-piece acrylic intraocular AcrySof lenses (model MN60AC, Alcon) (+27 D OS, +29 D OD) were implanted within the capsular bag after uncomplicated cataract phacoemulsification. The artificial iris was folded with Kelman-McPherson forceps and was introduced carefully in a 2.8-mm Monarch II acrylic IOL injector (Alcon Laboratories, Inc) after adding cohesive viscoelastic (Healon). It was injected through the same 2.8-mm incision and implanted behind the remnant iris, in the ciliary sulcus without any sutures. Finally, intracameral cefuroxime was injected. A postoperative 5-week tapered course of topical corticoids was given as well as topical antibiotics for 1 week and topical nonsteroidal antiinflammatory drops for 1 month. At the 2-month postoperative follow-up, distance best-corrected visual acuity remained unchanged, at 20/100 in her OD and 20/

200 in her OS. However, a significant improvement in near binocular vision was found postoperatively from 20/40 to 20/25, which could be explained by a further decrease in nystagmus frequency and amplitude in convergence enhanced by clear media. Moreover, the patient experienced a subjective significant improvement of her photophobia and glare symptoms. She reported high satisfaction with the aesthetic result. The implant remained in the ciliary sulcus with a centered pupil in both eyes (Fig. 2) throughout the postsurgical 12 months of follow-up without any complications.

DISCUSSION Many factors contribute to poor visual acuity in patients with congenital aniridia, including macular or optic nerve hypoplasia, nystagmus, or amblyopia. However, glare and photophobia are reported in all patients owing to the lack of a complete iris diaphragm6 and may therefore limit postoperative visual outcomes. The 3.35-mm-diameter pupil of the artificial iris implant (ArtificialIris, Dr. Schmidt Intraocularlinsen GmbH, Human Optics) reduces the amount of light entering the eye, resulting in improved photophobia and glare symptoms. Because of the reduced aperture diameter, the implant promotes depth of focus, decreases optical aberration, and enhances near vision. These are clinically significant visual improvements as distance visual acuity may be severely reduced because of foveal hypoplasia. In the current literature, the most commonly used prosthetic iris devices are the iris diaphragm intraocular lenses.9Y13 The main disadvantages of these implants are the need of a large corneal incision, risk of secondary glaucoma, and chronic iritis. The



S38 Sutureless Artificial Iris after Phacoemulsification in AniridiaVFernańdez-Lo´pez et al.

FIGURE 2. Postoperative slit-lamp photographs and OCT-Visante images of the artificial iris placed under the remaining iris.

elasticity of this new artificial iris allows easy and less traumatic implantation through self-sealing tunnel incisions of 2.8 to 3.0 mm,9 thereby decreasing surgically induced astigmatism that might compromise achieving a good final visual acuity. Moreover, this prosthesis provides excellent cosmetic outcomes because it is fully color-customizable and structurally matches the appearance of the fellow iris or its remnants (Fig. 3). Noninvasive treatments like cosmetic colored contact lenses have also been used for this purpose.2 However, they need periodic replacement, and potential complications related to contact lens use are not to be underestimated, especially in young patients. When considering cataract surgery in patients with aniridia, an artificial iris could be inserted within the same procedure. This type of artificial iris can be suture fixated to the sulcus or iris remnants.3,6 In eyes with remaining capsules, the device can be placed in the sulcus or capsular bag without requiring sutures.9 Although this sutureless technique has been reported mainly for traumatic aniridias, this is believed to be the first reported use of the technique with congenital aniridias. The iris prosthesis was placed in the ciliary sulcus without sutures because there was adequate capsular support and enough iris remnants to avoid anterior displacement. Mainly patients with partial aniridia (more than two to three clock hours of iris remnants)14 would be the best candidates for this sutureless technique, but future studies should better define the minimal amount of iris remnants required. No major complications have been described in other cases treated with ArtificialIris.3Y7 However, the long-term side effect profile of this implant requires further study to discard complications reported with other types of iris prosthesis, such as glaucoma, endothelial damage, or uveitis.15 Another concern related to the use

of this iris prosthesis is the difficulty of exploring the retinal periphery because of the nonmodifiable 3.35-mm pupil. However, nowadays, ultra-widefield scanning laser ophthalmoscopy devices (Optomap Panoramic 200, Optos PLC) allow nonmydriatic imaging covering 180 to 200 degrees of the retina without requiring pupil dilation.

FIGURE 3. Appearance of the custom-colored ArtificialIris before surgical manipulation.



Sutureless Artificial Iris after Phacoemulsification in AniridiaVFernańdez-Lo´pez et al.

In conclusion, implantation of ArtificialIris (Dr. Schmidt Intraocularlinsen GmbH, Human Optics) combined with cataract surgery in congenital aniridia proved to be a successful technique improving photophobia and glare symptoms as well as near visual acuity in our patient. It also provided an excellent aesthetic outcome with no complications reported within the follow-up period of 12 months. To the authors’ knowledge, this is the first case described in the literature using this technique for congenital aniridia. The foldable artificial iris is a promising device for congenital aniridia. It can be easily implanted through the small incision of the cataract surgery in the sulcus with the advantage of not requiring suture fixation if there are enough capsular support and iris remnants to avoid its displacement.

ACKNOWLEDGMENTS No financial support was received for this article. None of the authors has a financial or proprietary interest in any material or method mentioned in this article and has no conflict to disclose. Received August 5, 2014; accepted October 7, 2014.

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6. Rana M, Savant V, Prydal JI. A new customized artificial iris diaphragm for treatment of traumatic aniridia. Cont Lens Anterior Eye 2013;36:93Y4. 7. Magnus J, Trau R, Mathysen DG, Tassignon MJ. Safety of an artificial iris in a phakic eye. J Cataract Refract Surg 2012;38: 1097Y100. 8. Chylack LT, Jr., Wolfe JK, Singer DM, Leske MC, Bullimore MA, Bailey IL, Friend J, McCarthy D, Wu SY. The Lens Opacities Classification System III. The Longitudinal Study of Cataract Study Group. Arch Ophthalmol 1993;111:831Y6. 9. Pozdeyeva NA, Pashtayev NP, Lukin VP, Batkov YN. Artificial irislens diaphragm in reconstructive surgery for aniridia and aphakia. J Cataract Refract Surg 2005;31:1750Y9. 10. Burk SE, Da Mata AP, Snyder ME, Cionni RJ, Cohen JS, Osher RH. Prosthetic iris implantation for congenital, traumatic, or functional iris deficiencies. J Cataract Refract Surg 2001;27: 1732Y40. 11. Osher RH, Burk SE. Cataract surgery combined with implantation of an artificial iris. J Cataract Refract Surg 1999;25:1540Y7. 12. Reinhard T, Engelhardt S, Sundmacher R. Black diaphragm aniridia intraocular lens for congenital aniridia: long-term follow-up. J Cataract Refract Surg 2000;26:375Y81. 13. Aslam SA, Wong SC, Ficker LA, MacLaren RE. Implantation of the black diaphragm intraocular lens in congenital and traumatic aniridia. Ophthalmology 2008;115:1705Y12. 14. Neuhann IM, Neuhann TF. Cataract surgery and aniridia. Curr Opin Ophthalmol 2010;21:60Y4. 15. Jonsson NJ, Sahlmuller MC, Ruokonen PC, Torun N, Rieck P. [Complications after cosmetic iris implantation]. Ophthalmologe 2011;108:455Y8.

Ester Fernańdez-Lo´pez ´ ´ Bifurcacion Pıo Baroja-General Avile´s, S/N 46015 Valencia Spain e-mail: [email protected]

