A New Technique for Treating Posttraumatic Aniridia with Aphakia

Home , Aniridia, Aphakia, Ptosis (eyelid)

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

e 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 trans- scleralW 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 prom- ising approach for visual rehabilitation and cosmetic improvement. Arch Ophthalmol. 2012;130(6):771-775

Posttraumatic aniridia with aphakia often results. Other options, such as an iris re- causes significant debilitating glare, photo- construction lens consisting of a custom- phobia, and loss of vision in affected pa- made polymethylmethacrylate IOL with a tients.1,2 Management options include the polymethylmethacrylateirisdiaphragm(Ar- fitting of contact lenses with an artificial iris tisan;Ophtec,Inc),areonlysuitableforcases peripheral pigmentation painted on it,3 cor- of partial aniridia with enough iris tissue neal tattooing,4,5 and the implantation of a left for enclavation of the haptic portions.12 prosthetic iris intraocular lens (IOL). Iris Devices such as the endocapsular ring print contact lenses, however, need peri- (Morcher GmbH) require capsular support, odic replacement, may be difficult to fit in which often is absent after severe anterior eyes with posttraumatic anterior segment segment trauma.13 changes, and often are difficult for elderly Customized silicone iris prostheses that people to handle. Corneal tattooing can ad- can be implanted through a small incision dress only the symptoms of aniridia, such have recently become available (Dr Schmidt as glare and photophobia, but not the de- Intraocularlinsen GmbH, distributed by Hu- creased visual acuity caused by aphakia. manOptics AG). This iris prosthesis not only Thus, a permanent surgical solution decreasesglaresymptomsinaniridiabutalso would be desirable in many patients. Ide- provides excellent cosmetic outcomes.14-17 ally, a prosthetic iris IOL would completely However, the iris prosthesis cannot correct replace the missing iris for its full periph- foraphakia.Inthispatientseries,wedescribe eral extension along 360°. Unfortunately, a new surgical technique for treating trau- current large-diameter iris IOLs suitable for matic aniridia with aphakia and the lack of sulcus fixation have a diameter of 9.0 to 10.0 capsular support by using haptic fixation of mm and thus require a large incision.6-11 an IOL on a foldable, custom-tailored iris Moreover, the iris color of these prosthe- prosthesis and its outcome. ses is black or—with the exception of the rigidirisprosthesis(MorcherGmbH)—only METHODS available in a limited number of colors and cannot be customized for optimal cosmetic PATIENTS

Author Affiliations: Department of Ophthalmology, University Eye Hospital We included 4 patients with traumatic aphakia Tübingen, Centre of Ophthalmology, Eberhard-Karls University Tübingen, and aniridia. All patients underwent primary Tübingen, Germany. wound closure as soon as possible, with addi-

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Time From Trauma Patient to Iris No./Sex/ Prosthesis–IOL Age, y Type of Trauma VA at Presentation Ocular Procedures for Trauma Repair Implantation, mo 1/M/72 Globe rupture with loss of iris, lens, and Light perception Wound closure and PPV with silicone oil 4 vitreous; vitreous hemorrhage for retinal detachment; silicone oil removal 2 mo later 2/M/39 Severe corneal laceration with lens and Hand movements Wound closure; lens removal and 6.5 iris loss anterior vitrectomy 4 d later 3/M/78 Globe rupture with loss of iris and IOL Light perception Wound closure (patient developed 11 postoperative chronic CME) 4/M/56 Penetrating injury with corneal Hand movements Wound closure; PPV 3 d later 10 laceration, partial iris loss, and loss of (pronounced corneal scar) lens, vitreous, and choroid

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 haptics of the IOL extended over the edges Usingapush-pullhook,the2sutureswere plana vitrectomy for internal reconstruc- of the prosthesis. Thus, the haptics were pulled out through the superior tunnel tion) a few days later if needed. The pa- slightly bent with the use of a needle (Figure 2). The sutures were cut, and tient characteristics are given in Table 1. holder to decrease the maximum diam- we ensured throughout the next steps that Patient 1 required pars plana vitrectomy eter of the combined implant (Figure 1C the resulting 4 free ends were attached with silicone oil filling. The patient sub- and D) (intraoperative video frames are to the corresponding quadrant of the iris sequently developed secondary glau- found in the eFigure, http://www prosthesis. The free suture ends were coma that required silicone oil removal. .archophthalmol.com). pulled through the iris prosthesis along The conjunctiva was opened circum- a loop that was created with an additional SURGICAL TECHNIQUE ferentially at the limbus, and an infusion polypropylene suture. Thereafter, the su- cannula was positioned in the pars plana tures could be firmly tied to the iris Iris prostheses custom tailored to the color inthetemporalinferiorquadrant.Remain- prosthesis–IOLimplant.Theimplantthen of the patients’ fellow iris were obtained ing anterior vitreous was removed by vi- was partially folded using IOL implan- (Dr Schmidt Intraocularlinsen GmbH). trectomy when necessary. A superior cor- tation forceps and introduced through the On 2 pair of opposite sides, 2 small stab neoscleral tunnel approximately 5 mm tunnel incision (Figure 3). incisions using a 0.9-mm microsurgical in length was created. For correct suture The prosthesis was centered and fix- blade were created about 1 mm apart and placement (each 90° apart), we used a ated to the sclera using a Z-suture tech- passing through the back of the custom- radial marker for keratoplasty. Corre- nique with 5 passes for external fixa- ized iris prosthesis (2 incisions at the 0° sponding marks were made on the iris tion of a transscleral polypropylene 20 and 2 at the 180° positions). The haptics prosthesis. Two scleral needles with a suture as reported previously. Fi- of a 3-piece IOL (Tecnis ZA9003, Ab- double-armed 10-0 polypropylene suture nally, the sutures were simply cut at the bott Medical Optics) were docked inside (Prolene; Ethicon, Inc) were passed level of the sclera and left without any the tip of a 28-gauge hollow needle and obliquely through the sclera approxi- knot (Figure 4). pulled through the small previously cre- mately 1.2 mm posterior to the limbus ated tunnel in the iris prosthesis from the outside inward in an ab externo RESULTS (Figure 1A and B). Owing to the high technique and docked inside the tip of a elasticity and stability of the iris prosthe- 28-gauge hollow needle that had been sis, the haptics were tightly attached to the passed through the ciliary sulcus on the In all patients, the combined im- back of the artificial iris. However, the opposite side as described previously.18,19 plant stayed firmly fixed within the

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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 cen- ing keratoplasty for corneal scar- nonsteroidal anti-inflammatory eye tered position without any tilt or ring due to the initial trauma. Pa- drops and parabulbar corticoste- torque during follow-up. Patient 2 tient 3 had persistent chronic cystoid roids but did not resolve com- underwent uncomplicated perforat- macular edema that responded to pletely. The same patient had glau-

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©2012 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 coma before the injury; however, thesis) and the scleral sutures were ing slitlamp examination or by ob- intraocular pressure was well con- barely visible (Figure 5). No evi- taining a higher-magnification trolled with topical glaucoma drugs dence of suture erosion, suture loos- photograph (Figure 5). Moreover, (Table 2). Patient 1 was admitted ening, scleral atrophy, or chronic in- the 4-point Z-suture fixation tech- with suspected postoperative en- flammation was observed. nique offers the advantages of a knot- dophthalmitis a few days after sur- less approach. By avoiding suture gery. Diagnostic vitrectomy was per- COMMENT knots, the risk for scleral atrophy and formed and intravitreal antibiotics suture erosion may be lowered. Late were given. Symptoms improved Haptic fixation of a foldable IOL on suture erosion with knot exposure rapidly thereafter. No microorgan- a foldable and custom-tailored iris is a well-known problem in trans- isms could be grown from the vit- prosthesis for treating posttrau- scleral suturing. Hence, burying the real samples taken during diagnos- matic aniridia with aphakia offers a suture knots under a scleral flap or tic vitrectomy. The final outcome number of advantages. First, the fixa- in a scleral groove is generally rec- was excellent in this patient, with an tion of the IOL to the iris prosthesis ommended.18 However, a 73% long- uncorrected visual acuity of 20/25. is very firm owing to the high elastic term rate of suture erosion even The corneal endothelium was not stability of the silicone material of the through scleral flaps has been re- compromised in any of the pa- prosthesis, and the tucking of the ported in one study, suggesting that tients. At the final examination, vi- haptics can be performed quickly and this approach delays but does not sual acuity had increased in all pa- easily. Second, the implantation re- prevent this complication.21 In ad- tients (range, 20/800 to 20/25). No quires only a small incision of about dition, after complex trauma with patient complained of photopho- 4 mm because the combined im- scleral injuries and thinning, the cre- bia or glare, and the cosmetic ap- plant remains foldable. Further- ation of scleral flaps may be diffi- pearance was much improved. The more, the iris prosthesis is custom tai- cult or even impossible. In con- IOL haptics (fixated to the iris pros- lored to match the iris color of the trast, the Z-suture technique reliably patient’s fellow iris exactly. The secures the external suture in the tucked haptics were barely visible on sclera without any knot and thereby the anterior surface of the iris pros- obviates the need for scleral flaps or thesis and could only be seen dur- grooves.

A B

Figure 4. Fixation of the combined implant. The implant is sutured to the sclera at 4 points with Figure 5. Clinical photographs of patient 1. A, Before iris prosthesis–fixated intraocular lens (IOL) a polypropylene suture using a Z-suture implantation, near-complete iris loss and aphakia is observed. Visual acuity at this time was 20/63 (with technique in a zigzag-shaped pattern with 5 ϩ11 diopters [D]), and the patient complained of debilitating glare and photophobia. B, Appearance at the suture passes. The sutures are then cut at the last follow-up visit. The patient’s visual acuity was 20/25 (with −0.5 D sphere), and glare and photophobia level of the sclera and left without any knot. were absent. The IOL haptics are barely visible on the anterior surface of the iris prosthesis.

Table 2. Patient Results

Postoperative VA and Length of Patient VA and Refraction Before Iris Refraction at Last Follow-up After Additional Procedures After Ocular Comorbidities No. Prosthesis–IOL Implantation Follow-up Implantation, mo Implantation and Persisting Problems 1 20/60 (with ϩ11 sphere) 20/25 (with −0.5 sphere) 48 Diagnostic PPV and intravitreal Mild/dry AMD antibiotics for endophthalmitis; ptosis repair 2 20/600 (with ϩ9 sphere) 20/50 (with ϩ0.5 sphere, 24 Penetrating keratoplasty for Irregular astigmatism −3.0 cylinder, 70°) corneal scarring (pending fitting of rigid CL) 3 20/50 (with ϩ10.25 sphere, 20/40 (with ϩ1.25 15 Parabulbar corticosteroids Persisting CME; glaucoma 3.0 cylinder, 65°) sphere, −2.0 cylinder, therapy 81°) 4 20/800 (with ϩ14 sphere) 20/800 (plano) 4 Scheduled for penetrating Pronounced central corneal keratoplasty scarring

Abbreviations: AMD, age-related macular degeneration; CL, contact lens; CME, cystoid macular edema; IOL, intraocular lens; PPV, pars plana vitrectomy; VA, visual acuity.

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©2012 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 One possible disadvantage of the Thus, the technique can be used for reconstruction lens and rigid contact lens for trau- this technique is that the surgeon both types of the iris prosthesis. The matic aniridia. Eye Contact Lens. 2009;35(2): 108-110. must meticulously keep track of the mesh-containing device, in our ex- 4. Alio JL, Sirerol B, Walewska-Szafran A. Corneal origin and course of the various su- perience, is mandatory only when tattooing (keratopigmentation) with new mineral ture ends. However, if confusion con- partial iris defects are approached. micronized pigments to restore cosmetic appear- cerning the suture ends is feared, the Partial iris defects often require su- ance in severely impaired eyes. Br J Ophthalmol. fixation of the transscleral sutures to turing through the prosthesis and 2010;94:245-249. 5. Kim JH, Lee D, Hahn TW, Choi SK. New surgical the iris prosthesis could be easily fixation to the iris after the implant strategy for corneal tattooing using a femtosec- modified by first passing only 1 of the has been introduced into the ante- ond laser. Cornea. 2009;28(1):80-84. 2 scleral double-armed 10-0 polypro- rior chamber. Under these circum- 6. Sundmacher R, Reinhard T, Althaus C. Black- pylene sutures obliquely through the stances, the mesh facilitates sutur- diaphragm intraocular lens for correction of aniridia. Ophthalmic Surg. 1994;25(3):180-185. sclera. Then, after pulling the suture ing. However, in the technique 7. Sundmacher T, Reinhard T, Althaus C. Black dia- through the tunnel incision, the cut described herein, all stitches through phragm intraocular lens in congenital aniridia. Ger free ends of the sutures are attached the iris prosthesis are made while the J Ophthalmol. 1994;3(4-5):197-201. to the corresponding position at the prosthesis is still outside the globe, 8. Thompson CG, Fawzy K, Bryce IG, Noble BA. iris prosthesis. The same procedure which is possible without a problem Implantation of a black diaphragm intraocular lens 23 for traumatic aniridia. J Cataract Refract Surg. is then performed at corresponding using either version of the device. 1999;25(6):808-813. sites with the second double-armed Our technique with fixation of 9. Burk SE, Da Mata AP, Snyder ME, Cionni RJ, Co- 10-0 polypropylene suture. Thus, the the free suture ends to the iris pros- hen JS, Osher RH. Prosthetic iris implantation for surgeon has to deal with only 2 free thesis by using temporarily placed congenital, traumatic, or functional iris deficiencies. suture ends at a time. We used 4-point suture loops avoids extensive intra- J Cataract Refract Surg. 2001;27(11):1732- 1740. instead of 3-point transscleral fixa- ocular manipulation with the long 10. Aslam SA, Wong SC, Ficker LA, MacLaren RE. tion in view of reports in the litera- solid needles used for the 10-0 poly- Implantation of the black diaphragm intraocular ture about hydrolysis of 10-0 polypropylene sutures. This should be lens in congenital and traumatic aniridia. propylene sutures that caused especially advantageous in posttrau- Ophthalmology. 2008;115(10):1705-1712. dislocation in some cases of trans- matic eyes in which the sclera, the 11. Pozdeyeva NA, Pashtayev NP, Lukin VP, Batkov 22 YN. Artificial iris-lens diaphragm in reconstruc- sclerally sutured IOLs. However, to angle, and the cornea might be com- tive surgery for aniridia and aphakia. J Cataract date we have not observed a case of promised because of long-term se- Refract Surg. 2005;31(9):1750-1759. suture erosion with the 10-0 poly- quelae of the injury. 12. Sminia ML, Odenthal MT, Gortzak-Moorstein N, propylene transscleral Z suture, re- Wenniger-Prick LJ, Vo¨lker-Dieben HJ. Implanta- gardless of what type of implant tion of the Artisan iris reconstruction intraocular Submitted for Publication: July 9, lens in 5 children with aphakia and partial an- (IOLs, iris prostheses, or both) has 2011; final revision received Sep- iridia caused by perforating ocular trauma. been fixated to the sclera with this tember 2, 2011; accepted Septem- J AAPOS. 2008;12(3):268-272. 13. Ozturk F, Osher RH, Osher JM. Secondary pros- technique. Alternatively, 9-0 poly- ber 20, 2011. propylene sutures may be used. thetic iris implantation following traumatic total Correspondence: Martin S. Spitzer, aniridia and pseudophakia. J Cataract Refract Surg. Unfortunately, the artificial iris de- MD, Department of Ophthalmol- 2006;32(11):1968-1970. vice we used—like all other iris pros- ogy, University Eye Hospital Tübin- 14. Snyder ME. August consultation 2. J Cataract Re- theses mentioned—has not been ap- fract Surg. 2008;34(8):1231-1233. doi:10.1016 gen, Centre of Ophthalmology, Eb- /j.jcrs.2008.06.003. proved by the US Food and Drug erhard-Karls University Tübingen, Administration. Moreover, al- 15. Szurman P. August consultation 4. J Cataract Re- Schleichstrasse 12, 72076 Tübin- fract Surg. 2008;34(8):1234. doi:10.1016/j.jcrs though the prosthesis is color gen, Germany (martin.spitzer@med .2008.06.005. matched to a photograph of the un- .unituebingen.de). 16. Miller KM. August consultation 5. J Cataract Re- injured eye and the surface texture is fract Surg. 2008;34(8):1234-1235. doi:10.1016 Financial Disclosure: None re- /j.jcrs.2008.06.006. very natural in its appearance, the tex- ported. ture of the anterior surface of the de- 17. Rosenthal KJ. August consultation 7. J Cataract Online-Only Material: The eFig- Refract Surg. 2008;34(8):1231-1233. doi:10.1016 vice is identical for every device. In ure is available at http://www /j.jcrs.2008.06.008. addition, the pupillary diameter of .archophthalmol.com. 18. Lewis JS. Ab externo sulcus fixation. Ophthal- 3.35 mm is the same for all im- mic Surg. 1991;22(11):692-695. Additional Contributions: Regina 19. Lewis JS. Sulcus fixation without flaps. plants. The manufacturer provides the Hofer, MA, prepared the artwork for iris prosthesis in 2 versions, with or Ophthalmology. 1993;100(9):1346-1350. the manuscript. 20. Szurman P, Petermeier K, Aisenbrey S, Spitzer without an embedded polymer fiber MS, Jaissle GB. Z-suture: a new knotless tech- meshwork. The manufacturer rec- nique for transscleral suture fixation of intraocu- ommends using the fiber-contain- REFERENCES lar implants. Br J Ophthalmol. 2010;94(2):167- ing device when the prosthesis needs 169. to be sutured fixated, whereas the fi- 1. Williams DR, Yoon GY, Guirao A, Hofer H, Porter 21. Solomon K, Gussler JR, Gussler C, Van Meter WS. J. How far can we extend the limits of human Incidence and management of complications of ber-free device is recommended for vision? In: MacRae SM, Krueger RR, Applegate transsclerally sutured posterior chamber lenses. the treatment of full aniridia for su- RA, eds. Customized Corneal Ablation: The Quest J Cataract Refract Surg. 1993;19(4):488-493. tureless implantation. However, the for Super Vision. Thorofare, NJ: Slack; 2001:11- 22. Price MO, Price FW Jr, Werner L, Berlie C, Ma- fiber-free version of the device was 32. malis N. Late dislocation of scleral-sutured pos- 2. Neuhann IM, Neuhann TF. Cataract surgery and terior chamber intraocular lenses. J Cataract Re- used in 2 of the cases described herein aniridia. Curr Opin Ophthalmol. 2010;21(1): fract Surg. 2005;31(7):1320-1326. because the device without the mesh 60-64. 23. Szurman P, Jaissle G. Artificial iris. Ophthalmologe. is more flexible and easier to fold. 3. Chung MY, Miller KM, Weissman BA. 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