We describe a new surgical technique for treating traumatic aniridia with aphakia

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1 SURGICAL TECHNIQUE A New Technique for Treating Posttraumatic Aniridia With Aphakia First Results of Haptic Fixation of a Foldable Intraocular Lens on a Foldable and Custom-Tailored Iris Prosthesis Martin S. Spitzer, MD; Efdal Yoeruek, MD; Martin A. Leitritz, MD; Peter Szurman, MD; Karl U. Bartz-Schmidt, MD We describe a new surgical technique for treating traumatic aniridia with aphakia and its results in a small consecutive case series. We attached a 3-piece acrylic intraocular lens through the haptics to a customized silicone iris prosthesis. The combined implant was inserted through a 5-mm incision and fixated with a transscleral suture in the ciliary sulcus using a knotless technique (Z suture). In all patients, the combined implant stayed firmly fixed within the sulcus and showed a stable and centered position without any tilt or torque during follow-up. Thus, managing posttraumatic aniridia with aphakia by means of haptic fixation of a foldable intraocular lens on a custom-tailored iris prosthesis is a promising approach for visual rehabilitation and cosmetic improvement. Arch Ophthalmol. 2012;130(6): Posttraumatic aniridia with aphakia often causes significant debilitating glare, photophobia, and loss of vision in affected patients. 1,2 Management options include the fitting of contact lenses with an artificial iris peripheral pigmentation painted on it, 3 corneal tattooing, 4,5 and the implantation of a prosthetic iris intraocular lens (IOL). Iris print contact lenses, however, need periodic replacement, may be difficult to fit in eyes with posttraumatic anterior segment changes, and often are difficult for elderly people to handle. Corneal tattooing can address only the symptoms of aniridia, such as glare and photophobia, but not the decreased visual acuity caused by aphakia. Thus, a permanent surgical solution would be desirable in many patients. Ideally, a prosthetic iris IOL would completely replace the missing iris for its full peripheral extension along 360. Unfortunately, current large-diameter iris IOLs suitable for sulcusfixationhaveadiameterof9.0to10.0 mm and thus require a large incision Moreover, the iris color of these prostheses is black or with the exception of the rigidirisprosthesis(morchergmbh) only available in a limited number of colors and cannot be customized for optimal cosmetic Author Affiliations: Department of Ophthalmology, University Eye Hospital Tübingen, Centre of Ophthalmology, Eberhard-Karls University Tübingen, Tübingen, Germany. results. Other options, such as an iris reconstruction lens consisting of a custommade polymethylmethacrylate IOL with a polymethylmethacrylateirisdiaphragm(artisan; Ophtec, Inc), areonlysuitableforcases of partial aniridia with enough iris tissue left for enclavation of the haptic portions. 12 Devices such as the endocapsular ring (Morcher GmbH) require capsular support, which often is absent after severe anterior segment trauma. 13 Customized silicone iris prostheses that can be implanted through a small incision have recently become available (Dr Schmidt Intraocularlinsen GmbH, distributed by HumanOptics AG). This iris prosthesis not only decreasesglaresymptomsinaniridiabutalso provides excellent cosmetic outcomes However, the iris prosthesis cannot correct foraphakia. Inthispatientseries, wedescribe a new surgical technique for treating traumatic aniridia with aphakia and the lack of capsular support by using haptic fixation of an IOL on a foldable, custom-tailored iris prosthesis and its outcome. METHODS PATIENTS We included 4 patients with traumatic aphakia and aniridia. All patients underwent primary wound closure as soon as possible, with addi- 771

2 Table 1. Patients Characteristics Patient No./Sex/ Age, y Type of Trauma VA at Presentation Ocular Procedures for Trauma Repair 1/M/72 Globe rupture with loss of iris, lens, and vitreous; vitreous hemorrhage Light perception Wound closure and PPV with silicone oil for retinal detachment; silicone oil removal 2 mo later Wound closure; lens removal and anterior vitrectomy 4 d later 2/M/39 Severe corneal laceration with lens and iris loss Hand movements 3/M/78 Globe rupture with loss of iris and IOL Light perception Wound closure (patient developed postoperative chronic CME) 4/M/56 Penetrating injury with corneal Hand movements Wound closure; PPV 3 d later laceration, partial iris loss, and loss of (pronounced corneal scar) lens, vitreous, and choroid Time From Trauma to Iris Prosthesis IOL Implantation, mo Abbreviations: CME, cystoid macular edema; IOL, intraocular lens; PPV, pars plana vitrectomy; VA, visual acuity. A B C D Figure 1. First stage of implantation with iris prosthesis and intraocular lens (IOL). A, Two small stab incisions using a 0.9-mm microsurgical blade are created about 1 mm apart, passing through the back of the customized iris prosthesis (2 incisions at the 0 position and 2 at the 180 position). B, The haptics of a 3-piece IOL are docked inside the tip of a 28-gauge hollow needle and pulled through the small previously created tunnel in the iris prosthesis. C, Because of the high elasticity and stability of the iris prosthesis, the haptics are tightly attached to the back of the artificial iris. D, The haptics are slightly bent by using a needle holder to decrease the maximum diameter of the combined implant. Intraoperative video frames of this technique are found in the efigure. tional secondary procedures (eg, pars plana vitrectomy for internal reconstruction) a few days later if needed. The patient characteristics are given in Table 1. Patient 1 required pars plana vitrectomy with silicone oil filling. The patient subsequently developed secondary glaucoma that required silicone oil removal. SURGICAL TECHNIQUE Iris prostheses custom tailored to the color of the patients fellow iris were obtained (Dr Schmidt Intraocularlinsen GmbH). On 2 pair of opposite sides, 2 small stab incisions using a 0.9-mm microsurgical blade were created about 1 mm apart and passing through the back of the customized iris prosthesis (2 incisions at the 0 and 2 at the 180 positions). The haptics of a 3-piece IOL (Tecnis ZA9003, Abbott Medical Optics) were docked inside the tip of a 28-gauge hollow needle and pulled through the small previously created tunnel in the iris prosthesis (Figure 1A and B). Owing to the high elasticity and stability of the iris prosthesis, the haptics were tightly attached to the back of the artificial iris. However, the haptics of the IOL extended over the edges of the prosthesis. Thus, the haptics were slightly bent with the use of a needle holder to decrease the maximum diameter of the combined implant (Figure 1C and D) (intraoperative video frames are found in the efigure, The conjunctiva was opened circumferentially at the limbus, and an infusion cannula was positioned in the pars plana inthetemporalinferiorquadrant. Remaining anterior vitreous was removed by vitrectomy when necessary. A superior corneoscleral tunnel approximately 5 mm in length was created. For correct suture placement (each 90 apart), we used a radial marker for keratoplasty. Corresponding marks were made on the iris prosthesis. Two scleral needles with a double-armed 10-0 polypropylene suture (Prolene; Ethicon, Inc) were passed obliquely through the sclera approximately 1.2 mm posterior to the limbus from the outside inward in an ab externo technique and docked inside the tip of a 28-gauge hollow needle that had been passed through the ciliary sulcus on the opposite side as described previously. 18,19 Usingapush-pullhook, the2sutureswere pulled out through the superior tunnel (Figure 2). The sutures were cut, and weensuredthroughoutthenextstepsthat the resulting 4 free ends were attached to the corresponding quadrant of the iris prosthesis. The free suture ends were pulled through the iris prosthesis along a loop that was created with an additional polypropylene suture. Thereafter, the sutures could be firmly tied to the iris prosthesis IOLimplant. Theimplantthen was partially folded using IOL implantationforcepsandintroducedthroughthe tunnel incision (Figure 3). The prosthesis was centered and fixated to the sclera using a Z-suture technique with 5 passes for external fixation of a transscleral polypropylene suture as reported previously. 20 Finally, the sutures were simply cut at the level of the sclera and left without any knot (Figure 4). RESULTS In all patients, the combined implant stayed firmly fixed within the 772

3 A B C D Figure 2. Suturing technique for placement of the iris prosthesis. A, Two scleral needles with a double-armed 10-0 polypropylene suture are passed obliquely through the sclera approximately 1.2 mm posterior to the limbus from the outside inward in an ab externo technique. B and C, The sutures are docked inside the tip of a 28-gauge hollow needle, which has been passed through the ciliary sulcus on the opposite side. D, Using a push-pull hook, the 2 sutures are pulled out through the superior tunnel. A B C D E F Figure 3. Securing the ends of the sutures. A, The sutures are cut. B, The resulting 4 free ends are attached to the corresponding quadrant of the iris prosthesis. C and D, The free suture ends are pulled through the iris prosthesis along a loop that has been created with an additional polypropylene suture. E, Thereafter, the sutures can be firmly tied to the combined iris prosthesis intraocular lens implant. F, The implant is partially folded and introduced through the tunnel incision. sulcus and showed a stable and centered position without any tilt or torque during follow-up. Patient 2 underwent uncomplicated perforating keratoplasty for corneal scarring due to the initial trauma. Patient 3 had persistent chronic cystoid macular edema that responded to nonsteroidal anti-inflammatory eye drops and parabulbar corticosteroids but did not resolve completely. The same patient had glau- 773

4 coma before the injury; however, intraocular pressure was well controlled with topical glaucoma drugs (Table 2). Patient 1 was admitted with suspected postoperative endophthalmitis a few days after surgery. Diagnostic vitrectomy was performed and intravitreal antibiotics were given. Symptoms improved rapidly thereafter. No microorganisms could be grown from the vitreal samples taken during diagnostic vitrectomy. The final outcome was excellent in this patient, with an uncorrected visual acuity of 20/25. The corneal endothelium was not compromised in any of the patients. At the final examination, visual acuity had increased in all patients (range, 20/800 to 20/25). No patient complained of photophobia or glare, and the cosmetic appearance was much improved. The IOL haptics (fixated to the iris prosthesis) and the scleral sutures were barely visible (Figure 5). No evidence of suture erosion, suture loosening, scleral atrophy, or chronic inflammation was observed. COMMENT Haptic fixation of a foldable IOL on a foldable and custom-tailored iris prosthesis for treating posttraumatic aniridia with aphakia offers a number of advantages. First, the fixation of the IOL to the iris prosthesis is very firm owing to the high elastic stability of the silicone material of the prosthesis, and the tucking of the haptics can be performed quickly and easily. Second, the implantation requires only a small incision of about 4 mm because the combined implant remains foldable. Furthermore, the iris prosthesis is custom tailored to match the iris color of the patient s fellow iris exactly. The tucked haptics were barely visible on the anterior surface of the iris prosthesis and could only be seen during slitlamp examination or by obtaining a higher-magnification photograph (Figure 5). Moreover, the 4-point Z-suture fixation technique offers the advantages of a knotless approach. By avoiding suture knots, the risk for scleral atrophy and suture erosion may be lowered. Late suture erosion with knot exposure is a well-known problem in transscleral suturing. Hence, burying the suture knots under a scleral flap or in a scleral groove is generally recommended. 18 However, a 73% longterm rate of suture erosion even through scleral flaps has been reported in one study, suggesting that this approach delays but does not prevent this complication. 21 In addition, after complex trauma with scleral injuries and thinning, the creation of scleral flaps may be difficult or even impossible. In contrast, the Z-suture technique reliably secures the external suture in the sclera without any knot and thereby obviates the need for scleral flaps or grooves. A B Figure 4. Fixation of the combined implant. The implant is sutured to the sclera at 4 points with a polypropylene suture using a Z-suture technique in a zigzag-shaped pattern with 5 suture passes. The sutures are then cut at the level of the sclera and left without any knot. Figure 5. Clinical photographs of patient 1. A, Before iris prosthesis fixated intraocular lens (IOL) implantation, near-complete iris loss and aphakia is observed. Visual acuity at this time was 20/63 (with 11 diopters [D]), and the patient complained of debilitating glare and photophobia. B, Appearance at the last follow-up visit. The patient s visual acuity was 20/25 (with 0.5 D sphere), and glare and photophobia were absent. The IOL haptics are barely visible on the anterior surface of the iris prosthesis. Table 2. Patient Results Patient No. VA and Refraction Before Iris Prosthesis IOL Implantation Postoperative VA and Refraction at Last Follow-up Length of Follow-up After Implantation, mo Additional Procedures After Implantation 1 20/60 (with 11 sphere) 20/25 (with 0.5 sphere) 48 Diagnostic PPV and intravitreal antibiotics for endophthalmitis; ptosis repair 2 20/600 (with 9 sphere) 20/50 (with 0.5 sphere, 3.0 cylinder, 70 ) 3 20/50 (with sphere, 3.0 cylinder, 65 ) 20/40 (with 1.25 sphere, 2.0 cylinder, 81 ) 24 Penetrating keratoplasty for corneal scarring 15 Parabulbar corticosteroids therapy 4 20/800 (with 14 sphere) 20/800 (plano) 4 Scheduled for penetrating keratoplasty Ocular Comorbidities and Persisting Problems Mild/dry AMD Irregular astigmatism (pending fitting of rigid CL) Persisting CME; glaucoma Pronounced central corneal scarring Abbreviations: AMD, age-related macular degeneration; CL, contact lens; CME, cystoid macular edema; IOL, intraocular lens; PPV, pars plana vitrectomy; VA, visual acuity. 774

5 One possible disadvantage of the this technique is that the surgeon must meticulously keep track of the origin and course of the various suture ends. However, if confusion concerning the suture ends is feared, the fixation of the transscleral sutures to the iris prosthesis could be easily modified by first passing only 1 of the 2 scleral double-armed 10-0 polypropylene sutures obliquely through the sclera. Then, after pulling the suture through the tunnel incision, the cut free ends of the sutures are attached to the corresponding position at the iris prosthesis. The same procedure is then performed at corresponding sites with the second double-armed 10-0 polypropylene suture. Thus, the surgeon has to deal with only 2 free suture ends at a time. We used 4-point instead of 3-point transscleral fixation in view of reports in the literature about hydrolysis of 10-0 polypropylene sutures that caused dislocation in some cases of transsclerally sutured IOLs. 22 However, to date we have not observed a case of suture erosion with the 10-0 polypropylene transscleral Z suture, regardless of what type of implant (IOLs, iris prostheses, or both) has been fixated to the sclera with this technique. Alternatively, 9-0 polypropylene sutures may be used. Unfortunately, the artificial iris device we used like all other iris prostheses mentioned has not been approved by the US Food and Drug Administration. Moreover, although the prosthesis is color matched to a photograph of the uninjured eye and the surface texture is very natural in its appearance, the texture of the anterior surface of the device is identical for every device. In addition, the pupillary diameter of 3.35 mm is the same for all implants. The manufacturer provides the iris prosthesis in 2 versions, with or without an embedded polymer fiber meshwork. The manufacturer recommends using the fiber-containing device when the prosthesis needs to be sutured fixated, whereas the fiber-free device is recommended for the treatment of full aniridia for sutureless implantation. However, the fiber-free version of the device was used in 2 of the cases described herein because the device without the mesh is more flexible and easier to fold. Thus, the technique can be used for both types of the iris prosthesis. The mesh-containing device, in our experience, is mandatory only when partial iris defects are approached. Partial iris defects often require suturing through the prosthesis and fixation to the iris after the implant has been introduced into the anterior chamber. Under these circumstances, the mesh facilitates suturing. However, in the technique described herein, all stitches through the iris prosthesis are made while the prosthesis is still outside the globe, which is possible without a problem using either version of the device. 23 Our technique with fixation of the free suture ends to the iris prosthesis by using temporarily placed suture loops avoids extensive intraocular manipulation with the long solid needles used for the 10-0 polypropylene sutures. This should be especially advantageous in posttraumatic eyes in which the sclera, the angle, and the cornea might be compromised because of long-term sequelae of the injury. Submitted for Publication: July 9, 2011; final revision received September 2, 2011; accepted September 20, Correspondence: Martin S. Spitzer, MD, Department of Ophthalmology, University Eye Hospital Tübingen, Centre of Ophthalmology, Eberhard-Karls University Tübingen, Schleichstrasse 12, Tübingen, Germany (martin.spitzer@med.unituebingen.de). Financial Disclosure: None reported. Online-Only Material: The efigure is available at Additional Contributions: Regina Hofer, MA, prepared the artwork for the manuscript. REFERENCES 1. Williams DR, Yoon GY, Guirao A, Hofer H, Porter J. How far can we extend the limits of human vision? In: MacRae SM, Krueger RR, Applegate RA, eds. Customized Corneal Ablation: The Quest for Super Vision. Thorofare, NJ: Slack; 2001: Neuhann IM, Neuhann TF. Cataract surgery and aniridia. Curr Opin Ophthalmol. 2010;21(1): Chung MY, Miller KM, Weissman BA. Morcher iris reconstruction lens and rigid contact lens for traumatic aniridia. Eye Contact Lens. 2009;35(2): Alio JL, Sirerol B, Walewska-Szafran A. Corneal tattooing (keratopigmentation) with new mineral micronized pigments to restore cosmetic appearance in severely impaired eyes. Br J Ophthalmol. 2010;94: Kim JH, Lee D, Hahn TW, Choi SK. New surgical strategy for corneal tattooing using a femtosecond laser. Cornea. 2009;28(1): Sundmacher R, Reinhard T, Althaus C. Blackdiaphragm intraocular lens for correction of aniridia. Ophthalmic Surg. 1994;25(3): Sundmacher T, Reinhard T, Althaus C. Black diaphragm intraocular lens in congenital aniridia. Ger J Ophthalmol. 1994;3(4-5): Thompson CG, Fawzy K, Bryce IG, Noble BA. Implantation of a black diaphragm intraocular lens for traumatic aniridia. J Cataract Refract Surg. 1999;25(6): 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(11): Aslam SA, Wong SC, Ficker LA, MacLaren RE. Implantation of the black diaphragm intraocular lens in congenital and traumatic aniridia. Ophthalmology. 2008;115(10): Pozdeyeva NA, Pashtayev NP, Lukin VP, Batkov YN. Artificial iris-lens diaphragm in reconstructive surgery for aniridia and aphakia. J Cataract Refract Surg. 2005;31(9): Sminia ML, Odenthal MT, Gortzak-Moorstein N, Wenniger-Prick LJ, Völker-Dieben HJ. Implantation of the Artisan iris reconstruction intraocular lens in 5 children with aphakia and partial aniridia caused by perforating ocular trauma. J AAPOS. 2008;12(3): Ozturk F, Osher RH, Osher JM. Secondary prosthetic iris implantation following traumatic total aniridia and pseudophakia. J Cataract Refract Surg. 2006;32(11): Snyder ME. August consultation 2. J Cataract Refract Surg. 2008;34(8): doi: /j.jcrs Szurman P. August consultation 4. J Cataract Refract Surg. 2008;34(8):1234. doi: /j.jcrs Miller KM. August consultation 5. J Cataract Refract Surg. 2008;34(8): doi: /j.jcrs Rosenthal KJ. August consultation 7. J Cataract Refract Surg. 2008;34(8): doi: /j.jcrs Lewis JS. Ab externo sulcus fixation. Ophthalmic Surg. 1991;22(11): Lewis JS. Sulcus fixation without flaps. Ophthalmology. 1993;100(9): Szurman P, Petermeier K, Aisenbrey S, Spitzer MS, Jaissle GB. Z-suture: a new knotless technique for transscleral suture fixation of intraocular implants. Br J Ophthalmol. 2010;94(2): Solomon K, Gussler JR, Gussler C, Van Meter WS. Incidence and management of complications of transsclerally sutured posterior chamber lenses. J Cataract Refract Surg. 1993;19(4): Price MO, Price FW Jr, Werner L, Berlie C, Mamalis N. Late dislocation of scleral-sutured posterior chamber intraocular lenses. J Cataract Refract Surg. 2005;31(7): Szurman P, Jaissle G. Artificial iris. Ophthalmologe. 2011;108(8):

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