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Transscleral fixation of a black diaphragm intraocular lens in severely traumatized eyes requiring vitreoretinal surgery
CASE REPORT
Transscleral fixation of a black diaphragm intraocular lens in severely traumatized eyes requiring vitreoretinal surgery Zeynep Ozbek, MD, Suleyman Kaynak, MD, Ozgur Zengin, MD
The records of 4 eyes of 4 patients who had transscleral fixation of black diaphragm intraocular lenses (IOLs) after vitreoretinal surgery due to complications of severe perforating trauma were retrospectively reviewed. The transscleral fixation was performed 4 to 13 months after the vitreoretinal surgery. All patients reported a subjective decrease in glare and photophobia, with improved visual acuity in 2 eyes during a mean follow-up of 3 years. Cystoid macular edema was noted in 1 eye and transitory intraocular pressure elevation due to intraocular silicone oil in 1 eye. Severe perforating eye injury is frequently associated with extensive iris defects and lenticular and vitreoretinal complications. Although visual acuity may not be the primary concern in these eyes, favorable visual rehabilitation can be achieved following proper management of the retinal complications and transscleral fixation of black diaphragm IOLs to overcome glare and photophobia. J Cataract Refract Surg 2007; 33:1494–1498 Q 2007 ASCRS and ESCRS
Blunt or perforating eye injuries are frequently associated with iris defects, lens injury, hyphema, vitreous prolapse, and vitreous hemorrhage.1,2 Visual recovery is closely related to the extent of perforation, involvement of the lens, and possible complications such as vitreous hemorrhage, retinal detachment (RD), and endophthalmitis. However, timely management and meticulous follow-up can restore useful vision. We present 4 eyes that had vitreoretinal surgery following ruptured eye repair and later had transscleral fixation of a black diaphragm intraocular lens (IOL). CASE REPORTS Case 1 A 32-year-old man presented in November 2000 with pain and decreased vision following blunt trauma to the
Accepted for publication April 13, 2007. From the Department of Ophthalmology, Dokuz Eylul University, School of Medicine, Izmir, Turkey. No author has a financial or proprietary interest in any material or method mentioned. Presented in part at the VII Mediterreannean Ophthalmological Society Congress, May 19, 2002, Alicante, Spain, 2002. Corresponding author: Zeynep Ozbek, MD, Mithatpasa Cad, EROK Sit., D Blok. 95/19, Balcova 35330, Izmir, Turkey. E-mail: zeynep_ozbek @hotmail.com.
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right eye. The visual acuity was hand motions in the right eye and 20/20 in the left eye. The right eye had an extensive subconjunctival hematoma and about 6.0 mm of hyphema; it appeared slightly deformed. There was a wide iris defect of 6 clock hours involving the superior and temporal quadrants. The lens was subluxated inferiorly, with vitreous in the anterior chamber. The right fundus was not visible. B-mode orbital ultrasound detected a vitreous hemorrhage. Ophthalmologic examination of the left eye was unremarkable. Exploration of the right eye under general anesthesia revealed a linear perforation (about 5.0 to 6.0 mm) in the temporal sclera, which was repaired using 5-0 polyester sutures. The patient was admitted and monitored for hyphema and intraocular pressure (IOP). Systemic broad-spectrum antibiotics were prescribed, along with topical ciprofloxacin every hour, topical cycloplegics 3 times a day, and topical corticosteroids 6 times a day. The patient was discharged a week later as the IOP was stable and the hyphema was subsiding. Two days later, the patient returned with pain and visual deterioration. Examination of the anterior chamber revealed 3C cells and 2.0 mm of hypopyon. The visual acuity was light perception. The patient was readmitted; vitreous tap was performed, and samples were sent for microscopy and culture. An intravitreal injection of vancomycin 1 mg/mL and cefazolin 2 mg/mL was given, and hourly fortified topical antibiotics were started. Although marked improvement was noted in the anterior chamber, the vitreous remained dense and scleral buckling, pars plana vitrectomy (PPV), endophacoemulsification of the dropped lens, endolaser treatment under perfluorocarbon liquid (PFC), and fluid– air exchange and silicone oil injection were performed 2 weeks later. The visual acuity was 20/50 with aphakic correction during the first month and remained stable at 20/60 during 0886-3350/07/$dsee front matter doi:10.1016/j.jcrs.2007.04.030
CASE REPORT: BLACK DIAPHRAGM IOLS AFTER VITREORETINAL SURGERY
the follow-up. There was severe glare and photophobia. Transscleral fixation of a black diaphragm IOL (aniridia IOL type 67F, Morcher GmbH) and silicone removal were performed in October 2001. The IOL was perfectly centered (Figure 1), and the best corrected visual acuity (BCVA) was 20/60 with 2.00 1.75 120 during 13 months of follow-up. In March 2003, the patient noted a decrease in vision. The visual acuity was counting fingers. Fundus examination and fluorescein angiography revealed cystoid macular edema (CME). Oral fluocortolone 40 mg/day and topical prednisolone acetate 1% 6 times a day were started. After a tapered course of oral steroids, the visual acuity improved to 20/40. In January 2006, the patient returned with a decrease in vision. Fundus examination, fluorescein angiography, and optical coherence tomography detected CME, with a macular thickness of 524 mm. An intravitreal injection of triamcinolone acetonide was given 1 week later. In February 2006, the BCVA improved to 20/60 with 2.50 2.00 120; the macular thickness was 212 mm.
Case 2 A 46-year-old man presented in August 2000. He had had corneal perforation repair at another institution in June 2000 and was referred to us because of endophthalmitis and traumatic cataract. The visual acuity was hand motions. Anterior segment examination revealed an iris defect of 6 clock hours associated with zonular loss in that area. The lens was partially dislocated and dense. There were 3C cells in the anterior chamber. B-mode orbital ultrasound detected a vitreous hemorrhage and possible RD. The patient was admitted. Vancomycin and cefazolin were injected intravitreally, and hourly fortified antibiotics were started. The clinical picture improved in 10 days. Scleral buckling together with lensectomy, PPV, endolaser photocoagulation under PFC, fluid–air exchange, and silicone oil injection were performed 2 weeks later. The retina reattached satisfactorily, and the visual acuity improved to 20/200. At 3 months, the silicone oil was removed because of uncontrolled IOP. The IOP decreased to 18 mm Hg within several weeks. The postoperative visual acuity was 20/70 with aphakic correction, but the patient complained of glare
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and photophobia. Transscleral fixation of a black diaphragm IOL (aniridia IOL type 67F) was performed in March 2001. The BCVA remained stable at 20/60 with 4.50 1.00 10 during the 3-year follow-up.
Case 3 A 44-year-old man presented in December 2000 with pain and decreased vision in the left eye. He had had a traffic accident 3 days earlier and had received prompt medical attention and corneal perforation repair at a local institution. He was referred for further evaluation. At the initial visit, the visual acuity was hand motions in the left eye and 20/20 in the right eye. Slitlamp examination revealed a wide subconjunctival hemorrhage, a repaired linear corneal perforation, an iris–zonular defect of 8 clock hours superiorly, and a dislocated lens in the left eye. B-mode orbital ultrasound detected a vitreous hemorrhage and RD. The patient was admitted. Systemic antibiotics, topical ciprofloxacin every hour, topical prednisolone acetate 6 times a day, and topical cycloplegics 3 times a day were administered. Scleral buckling, PPV, pars plana lensectomy, endolaser photocoagulation under PFC, silicone oil injection, and silicone–gas exchange were performed. At the end of the first month, the visual acuity was 20/70 with aphakic correction and remained stable at 20/60 during 1 year of follow-up. Transscleral fixation of a black diaphragm IOL (aniridia IOL type 67F) was performed in November 2001. The BCVA was 20/60 with a manifest refraction of 4.25 0.75 170 during 18 months of followup; no complications were noted.
Case 4 A 25-year-old man presented in December 2003 with traumatic corneal perforation in the left eye. The visual acuity was light perception and 20/20 in the right eye and left eye, respectively. Examination revealed an iris defect of 6 clock hours, anterior chamber hemorrhage, partial lens dislocation, traumatic cataract, and vitreous hemorrhage. The patient was admitted, and the corneal perforation was repaired. During the first 3 months of follow-up, the visual acuity in the left eye was hand motions. The IOP was elevated in the first month so topical timolol maleate and brimonidine were started. Systemic acetazolamide was added because the IOP was 35 mm Hg despite the topical therapy. In February 2004, scleral buckling, PPV, pars plana lensectomy, endolaser photocoagulation under PFC, internal limiting membrane peeling for macular hole, and intravitreal gas injection were performed. The IOP was regulated with topical therapy after the vitreoretinal surgery. At 1 month, the BCVA was 20/100 but the patient complained of severe glare and photophobia. A transsclerally fixated black diaphragm IOL (model 311 aniridia IOL, Ophtec BV) was implanted in July 2004. The black diaphragm IOL was centered (Figure 2) and the BCVA remained stable at 20/70 in the left eye with a manifest refraction of C2.00 20. No complications were noted during the 18-month follow-up.
Surgical Technique
Figure 1. Case 1, left eye, 2 months after implantation of a Morcher aniridia IOL type 67F.
All surgery was performed by the same surgeon (S.K.). Fornix-based conjunctival flaps were created. Blunt dissection of the episcleral tissue and slight cauterization of the surgical field were performed. Triangular scleral flaps were
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Figure 2. Case 4, left eye, 1 month after implantation of an Ophtec 311 aniridia IOL.
constructed 180 degrees apart at the 3 and 9 o’clock positions. A 6.0 mm infusion port was introduced in the lower quadrant. Two 10-0 polypropylene sutures (Ethicon, ZW 1713) were tied to the haptics of the black diaphragm IOL. A 10.0 mm limbal incision was created, and the needles of the polypropylene sutures were passed under the scleral flaps. Once the IOL was centered, each arm of the suture was secured to the sclera. The flaps and the conjunctiva were closed with 8-0 polyglactin sutures, and the cornea was closed with 10-0 nylon.
DISCUSSION Aniridia can be classified as congenital or traumatic. Congenital aniridia is a developmental abnormality associated with low vision due to various types of ocular involvement such as optic nerve head and foveal hypoplasia, glaucoma, cataract, and aniridic keratopathy. Traumatic aniridia is usually accompanied by substantial damage in the anterior segment, resulting in corneal scarring, cataract, and secondary glaucoma.1–3 An intact iris diaphragm reduces the spherical and chromatic aberrations arising from the lens and increases the depth of focus.1,3,4,5 Therefore, aniridia causes incapacitating glare and photophobia. Eyelid surgery, colored contact lenses, and corneal tattooing may offer good cosmetic alternatives in severely traumatized eyes in which no useful vision is expected.6 However, if there is considerable vision after the acute effects of the trauma have subsided, visual rehabilitation with IOL implantation may be considered. A major complaint of IOL patients is photophobia due to wide iris defects. In that circumstance, artificial irides or colored IOLs, namely, black diaphragm IOLs, may offer a 1-step solution instead of standard IOL and artificial iris implantation. Use of an IOL with an artificial iris is not new. An anterior chamber IOL with an optic surrounded by
a colored diaphragm was designed by Choyce in 1959.7 In 1994, Sundmacher et al.8 implanted a newly designed posterior chamber black diaphragm IOL (Morcher GmbH) in 5 eyes with congenital aniridia and 8 eyes with traumatic aniridia. They reported improved visual acuity in 9 of 13 eyes with congenital aniridia and cataracts.9 Reinhard et al.10 reported satisfactory long-term results in 19 eyes of 14 patients with congenital aniridia treated with implantation of this IOL. The first reports described implantation of the IOL in the sulcus, but in 1999, Tanzer and Smith11 reported transscleral fixation of the aniridia IOL in a case with contact lens–intolerant traumatic aniridia and painful aphakic bullous keratopathy. Thompson et al.12 reported a series of 7 patients with traumatic aniridia treated with a black diaphragm IOL (Morcher type 67G); 6 required scleral fixation. Simultaneous penetrating keratoplasty was performed in 4 eyes, and anterior vitrectomy in 5 eyes. The BCVA improved in 5 patients and was unchanged in 1. Excellent visual acuity (6/6 to 6/9) was achieved in 3 patients. Lens decentration occurred in 2 eyes, postoperative hemorrhage in 1 eye, endophthalmitis in 1 eye, and RD in 1 eye. These complications were not observed in our series. Osher and Burk13 described 6 patients with aniridia who had implantation of various types of diaphragmatic devices, including endocapsular rings with multiple fins (Morcher type 50C), a sector iris device (Morcher type 96G) with a single fin, and a lens–iris device (Morcher type 67F). Two of the cases were traumatic, requiring cataract extraction and implantation of sector iris devices in the capsular bag. Mavrikaris et al.14 used Morcher aniridia IOL types 67F and 67G, the aniridia ring type 50C, and the coloboma diaphragm type 96G in 9 patients with traumatic iris defects, congenital aniridia or iris coloboma, and surgical or optical iridectomies. These cases differ from those in our series as none of our patients had capsule support and consequently needed transscleral fixation. Dong et al.15 implanted black diaphragm IOLs in 15 aphakic eyes with traumatic aniridia 6 to 72 months after PPV. The indications for PPV included unresolved vitreous hemorrhage, suppurative endophthalmitis, intraocular foreign-body removal, and retinal reattachment. One eye had extracapsular cataract extraction before PPV, 3 eyes lost their crystalline lens to trauma, and 11 eyes had simultaneous lensectomy during PPV. In all 15 eyes, the visual acuity improved and the glare decreased markedly. Omulecki and Synder2 reported a series of 6 patients treated for traumatic aniridia using PPV and transsclerally fixated black diaphragm IOLs. Total aniridia was observed in 3 eyes, and 4 eyes were aphakic. The BCVA
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improved in 5 patients and remained unchanged in 1. Good visual acuity (20/20 to 20/40) was achieved in all cases, and all IOLs were well-centered. No severe complications were noted. In our study, all eyes were aphakic with partial aniridia. Scleral buckling, PPV, endolaser photocoagulation under PFC with either intraocular gas or silicone oil injection were performed in all eyes. The patients were followed until the retina completely settled and then transscleral fixation of a black diaphragm IOL was performed. Scleral fixation was necessary because adequate capsule support was absent in all cases. The BCVA improved in 2 eyes and remained unchanged in the other 2 eyes compared with the preoperative visual acuity with aphakic correction. All patients reported a subjective decrease in glare and photophobia, although objective documentation was not performed. The most common complications reported after black diaphragm IOL implantation are persistent anterior chamber inflammation and glaucoma.2–4,10,12,15 Tanzer and Smith11 reported 3C anterior chamber cells and flare, which were treated aggressively with prednisolone acetate and subsided. Beltrame et al.4 reported postoperative persistent intraocular inflammation in 4 eyes, which disappeared within 3 months using topical steroids. No prolonged anterior chamber inflammation was documented in our series. Cystoid macular edema was detected in 1 patient 3 years and 5 years after the injury and managed with oral and topical steroids and intravitreal injection of triamcinolone acetonide, respectively. Elevated IOP was the most common postoperative complication reported after implantation of the fullsized black aniridia IOL.2–4,10–12,15 Elevated IOP occurred in 2 of 7 cases reported by Thompson et al.12 ; 1 required trabeculectomy. Three of 6 cases reported by Omulecki and Synder2 required topical glaucoma medications to achieve good IOP control. Dong et al.15 reported an IOP elevation in 5 of 15 cases. Four patients required timolol eyedrops and 1 patient, selective laser trabeculoplasty. Beltrame et al.4 reported 4 of 10 patients. Three cases were controlled medically and 1 case, surgically (trabeculectomy). Menezo et al.3 reported 2 eyes with postoperative ocular hypertension in 5 traumatic cases. One was controlled by topical bblockers, and the other needed 3 antiglaucoma drops and cyclodiode laser therapy. In our study, 2 eyes had elevated IOP and 1 patient was controlled after silicone removal. The other patient’s IOP was controlled with topical therapy after vitreoretinal surgery. Perforating or blunt eye injury often causes serious lenticular and vitreoretinal complications. We believe the first objective should be to provide the structural integrity of the eye by repairing the primary wound.
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Management of the coexisting traumatic cataract, lens dislocation, vitreous hemorrhage, and RD can be planned in the following weeks (or when they occur) under better conditions. This gives the surgeon and the patient the chance of orderly concomitant surgery with less risk. We usually prefer to place a buckle before PPV even if an RD is not detected because these patients have a high risk for retinal tears and detachment. We also perform endolaser treatment and gas or silicone oil injection to ensure better attachment of the retina. This series differs from those previously reported in that aspect. All our patients had transscleral fixation of a black diaphragm IOL when the retina was fully settled and the visual acuity was stable, well after the vitreoretinal surgery. Patients with trauma and multiple surgeries need ongoing posterior segment care postoperatively. One might think that black diaphragm IOLs would hinder visualization of the retinal periphery. However, visualization of the retinal periphery close to the equatorial region is possible with extreme ductions of the eye during binocular indirect ophthalmoscopy. More peripheral lesions may be visualized using a Mainster wide-field lens or a Goldmann 3-mirror lens with indentation. Several reports show that implantation of a black diaphragm IOL is a safe and effective option for managing eyes with traumatic aniridia and aphakia as it reduces glare and photophobia. However, the overall diameter of these IOLs is large and they are brittle because of the coloring procedure. They therefore necessitate wide incisions and careful handling. We suggest that proper management of the accompanying vitreoretinal problems in aphakic and aniridic eyes should be done before black diaphragm IOL implantation, which is challenging and involves a wide incision and transscleral fixation. REFERENCES 1. Mavrikakis I, Casey JMH. Phacoemulsification and endocapsular implantation of an artificial iris intraocular lens in traumatic cataract and aniridia [letter]. J Cataract Refract Surg 2002; 28:1088–1091 2. Omulecki W, Synder A. Pars plana vitrectomy and transscleral fixation of black diaphragm intraocular lens for the management of traumatic aniridia. Ophthalmic Surg Lasers 2002; 33: 357–361 3. Menezo JL, Martı´nez-Costa R, Cisneros A, Desco MC. Implantation of iris devices in congenital and traumatic aniridias: surgery solutions and complications. Eur J Ophthalmol 2005; 15:451–457 4. Beltrame G, Salvetat ML, Chizzolini M, et al. Implantation of a black diaphragm intraocular lens in ten cases of post-traumatic aniridia. Eur J Ophthalmol 2003; 13:62–68 5. Chen Y-J, Wu P-C. Favorable outcome using a black diaphragm intraocular lens for traumatic aniridia with total iridectomy. J Cataract Refract Surg 2003; 29:2455–2457
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6. Remky A, Redbrake C, Wenzel M. Intrastromal corneal tattooing for iris defects [letter]. J Cataract Refract Surg 1998; 24:1285–1287 7. Choyce P. Intra-Ocular Lenses and Implants. London, England, HK Lewis, 1964;21 8. Sundmacher R, Reinhard T, Althaus C. Black-diaphragm intraocular lens for correction of aniridia. Ophthalmic Surg 1994; 25:180–185 9. Sundmacher T, Reinhard T, Althaus C. Black diaphragm intraocular lens in congenital aniridia. Ger J Ophthalmol 1994; 3: 197–201 10. Reinhard T, Engelhardt S, Sundmacher R. Black diaphragm aniridia intraocular lens for congenital aniridia: long-term follow-up. J Cataract Refract Surg 2000; 26:375–381 11. Tanzer DJ, Smith RE. Black iris-diaphragm intraocular lens for aniridia and aphakia. J Cataract Refract Surg 1999; 25: 1548–1551 12. Thompson CG, Fawzy K, Bryce IG, Noble BA. Implantation of a black diaphragm intraocular lens for traumatic aniridia. J Cataract Refract Surg 1999; 25:808–813
13. Osher RH, Burk SE. Cataract surgery combined with implantation of an artificial iris. J Cataract Refract Surg 1999; 25: 1540–1547 14. Mavrikakis I, Mavrikakis E, Syam PP, et al. Surgical management of iris defects with prosthetic iris devices. Eye 2005; 19:205–209 15. Dong X, Yu B, Xie L. Black diaphragm intraocular lens implantation in aphakic eyes with traumatic aniridia and previous pars plana vitrectomy. J Cataract Refract Surg 2003; 29:2168–2173
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First author: Zeynep Ozbek, MD Department of Ophthalmology, Dokuz Eylul University, School of Medicine, Izmir, Turkey
Transscleral fixation of a black diaphragm intraocular lens in severely traumatized eyes requiring vitreoretinal surgery Zeynep Ozbek, MD, Suleyman Kaynak, MD, Ozgur Zengin, MD
The records of 4 eyes of 4 patients who had transscleral fixation of black diaphragm intraocular lenses (IOLs) after vitreoretinal surgery due to complications of severe perforating trauma were retrospectively reviewed. The transscleral fixation was performed 4 to 13 months after the vitreoretinal surgery. All patients reported a subjective decrease in glare and photophobia, with improved visual acuity in 2 eyes during a mean follow-up of 3 years. Cystoid macular edema was noted in 1 eye and transitory intraocular pressure elevation due to intraocular silicone oil in 1 eye. Severe perforating eye injury is frequently associated with extensive iris defects and lenticular and vitreoretinal complications. Although visual acuity may not be the primary concern in these eyes, favorable visual rehabilitation can be achieved following proper management of the retinal complications and transscleral fixation of black diaphragm IOLs to overcome glare and photophobia. J Cataract Refract Surg 2007; 33:1494–1498 Q 2007 ASCRS and ESCRS
Blunt or perforating eye injuries are frequently associated with iris defects, lens injury, hyphema, vitreous prolapse, and vitreous hemorrhage.1,2 Visual recovery is closely related to the extent of perforation, involvement of the lens, and possible complications such as vitreous hemorrhage, retinal detachment (RD), and endophthalmitis. However, timely management and meticulous follow-up can restore useful vision. We present 4 eyes that had vitreoretinal surgery following ruptured eye repair and later had transscleral fixation of a black diaphragm intraocular lens (IOL). CASE REPORTS Case 1 A 32-year-old man presented in November 2000 with pain and decreased vision following blunt trauma to the
Accepted for publication April 13, 2007. From the Department of Ophthalmology, Dokuz Eylul University, School of Medicine, Izmir, Turkey. No author has a financial or proprietary interest in any material or method mentioned. Presented in part at the VII Mediterreannean Ophthalmological Society Congress, May 19, 2002, Alicante, Spain, 2002. Corresponding author: Zeynep Ozbek, MD, Mithatpasa Cad, EROK Sit., D Blok. 95/19, Balcova 35330, Izmir, Turkey. E-mail: zeynep_ozbek @hotmail.com.
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right eye. The visual acuity was hand motions in the right eye and 20/20 in the left eye. The right eye had an extensive subconjunctival hematoma and about 6.0 mm of hyphema; it appeared slightly deformed. There was a wide iris defect of 6 clock hours involving the superior and temporal quadrants. The lens was subluxated inferiorly, with vitreous in the anterior chamber. The right fundus was not visible. B-mode orbital ultrasound detected a vitreous hemorrhage. Ophthalmologic examination of the left eye was unremarkable. Exploration of the right eye under general anesthesia revealed a linear perforation (about 5.0 to 6.0 mm) in the temporal sclera, which was repaired using 5-0 polyester sutures. The patient was admitted and monitored for hyphema and intraocular pressure (IOP). Systemic broad-spectrum antibiotics were prescribed, along with topical ciprofloxacin every hour, topical cycloplegics 3 times a day, and topical corticosteroids 6 times a day. The patient was discharged a week later as the IOP was stable and the hyphema was subsiding. Two days later, the patient returned with pain and visual deterioration. Examination of the anterior chamber revealed 3C cells and 2.0 mm of hypopyon. The visual acuity was light perception. The patient was readmitted; vitreous tap was performed, and samples were sent for microscopy and culture. An intravitreal injection of vancomycin 1 mg/mL and cefazolin 2 mg/mL was given, and hourly fortified topical antibiotics were started. Although marked improvement was noted in the anterior chamber, the vitreous remained dense and scleral buckling, pars plana vitrectomy (PPV), endophacoemulsification of the dropped lens, endolaser treatment under perfluorocarbon liquid (PFC), and fluid– air exchange and silicone oil injection were performed 2 weeks later. The visual acuity was 20/50 with aphakic correction during the first month and remained stable at 20/60 during 0886-3350/07/$dsee front matter doi:10.1016/j.jcrs.2007.04.030
CASE REPORT: BLACK DIAPHRAGM IOLS AFTER VITREORETINAL SURGERY
the follow-up. There was severe glare and photophobia. Transscleral fixation of a black diaphragm IOL (aniridia IOL type 67F, Morcher GmbH) and silicone removal were performed in October 2001. The IOL was perfectly centered (Figure 1), and the best corrected visual acuity (BCVA) was 20/60 with 2.00 1.75 120 during 13 months of follow-up. In March 2003, the patient noted a decrease in vision. The visual acuity was counting fingers. Fundus examination and fluorescein angiography revealed cystoid macular edema (CME). Oral fluocortolone 40 mg/day and topical prednisolone acetate 1% 6 times a day were started. After a tapered course of oral steroids, the visual acuity improved to 20/40. In January 2006, the patient returned with a decrease in vision. Fundus examination, fluorescein angiography, and optical coherence tomography detected CME, with a macular thickness of 524 mm. An intravitreal injection of triamcinolone acetonide was given 1 week later. In February 2006, the BCVA improved to 20/60 with 2.50 2.00 120; the macular thickness was 212 mm.
Case 2 A 46-year-old man presented in August 2000. He had had corneal perforation repair at another institution in June 2000 and was referred to us because of endophthalmitis and traumatic cataract. The visual acuity was hand motions. Anterior segment examination revealed an iris defect of 6 clock hours associated with zonular loss in that area. The lens was partially dislocated and dense. There were 3C cells in the anterior chamber. B-mode orbital ultrasound detected a vitreous hemorrhage and possible RD. The patient was admitted. Vancomycin and cefazolin were injected intravitreally, and hourly fortified antibiotics were started. The clinical picture improved in 10 days. Scleral buckling together with lensectomy, PPV, endolaser photocoagulation under PFC, fluid–air exchange, and silicone oil injection were performed 2 weeks later. The retina reattached satisfactorily, and the visual acuity improved to 20/200. At 3 months, the silicone oil was removed because of uncontrolled IOP. The IOP decreased to 18 mm Hg within several weeks. The postoperative visual acuity was 20/70 with aphakic correction, but the patient complained of glare
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and photophobia. Transscleral fixation of a black diaphragm IOL (aniridia IOL type 67F) was performed in March 2001. The BCVA remained stable at 20/60 with 4.50 1.00 10 during the 3-year follow-up.
Case 3 A 44-year-old man presented in December 2000 with pain and decreased vision in the left eye. He had had a traffic accident 3 days earlier and had received prompt medical attention and corneal perforation repair at a local institution. He was referred for further evaluation. At the initial visit, the visual acuity was hand motions in the left eye and 20/20 in the right eye. Slitlamp examination revealed a wide subconjunctival hemorrhage, a repaired linear corneal perforation, an iris–zonular defect of 8 clock hours superiorly, and a dislocated lens in the left eye. B-mode orbital ultrasound detected a vitreous hemorrhage and RD. The patient was admitted. Systemic antibiotics, topical ciprofloxacin every hour, topical prednisolone acetate 6 times a day, and topical cycloplegics 3 times a day were administered. Scleral buckling, PPV, pars plana lensectomy, endolaser photocoagulation under PFC, silicone oil injection, and silicone–gas exchange were performed. At the end of the first month, the visual acuity was 20/70 with aphakic correction and remained stable at 20/60 during 1 year of follow-up. Transscleral fixation of a black diaphragm IOL (aniridia IOL type 67F) was performed in November 2001. The BCVA was 20/60 with a manifest refraction of 4.25 0.75 170 during 18 months of followup; no complications were noted.
Case 4 A 25-year-old man presented in December 2003 with traumatic corneal perforation in the left eye. The visual acuity was light perception and 20/20 in the right eye and left eye, respectively. Examination revealed an iris defect of 6 clock hours, anterior chamber hemorrhage, partial lens dislocation, traumatic cataract, and vitreous hemorrhage. The patient was admitted, and the corneal perforation was repaired. During the first 3 months of follow-up, the visual acuity in the left eye was hand motions. The IOP was elevated in the first month so topical timolol maleate and brimonidine were started. Systemic acetazolamide was added because the IOP was 35 mm Hg despite the topical therapy. In February 2004, scleral buckling, PPV, pars plana lensectomy, endolaser photocoagulation under PFC, internal limiting membrane peeling for macular hole, and intravitreal gas injection were performed. The IOP was regulated with topical therapy after the vitreoretinal surgery. At 1 month, the BCVA was 20/100 but the patient complained of severe glare and photophobia. A transsclerally fixated black diaphragm IOL (model 311 aniridia IOL, Ophtec BV) was implanted in July 2004. The black diaphragm IOL was centered (Figure 2) and the BCVA remained stable at 20/70 in the left eye with a manifest refraction of C2.00 20. No complications were noted during the 18-month follow-up.
Surgical Technique
Figure 1. Case 1, left eye, 2 months after implantation of a Morcher aniridia IOL type 67F.
All surgery was performed by the same surgeon (S.K.). Fornix-based conjunctival flaps were created. Blunt dissection of the episcleral tissue and slight cauterization of the surgical field were performed. Triangular scleral flaps were
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Figure 2. Case 4, left eye, 1 month after implantation of an Ophtec 311 aniridia IOL.
constructed 180 degrees apart at the 3 and 9 o’clock positions. A 6.0 mm infusion port was introduced in the lower quadrant. Two 10-0 polypropylene sutures (Ethicon, ZW 1713) were tied to the haptics of the black diaphragm IOL. A 10.0 mm limbal incision was created, and the needles of the polypropylene sutures were passed under the scleral flaps. Once the IOL was centered, each arm of the suture was secured to the sclera. The flaps and the conjunctiva were closed with 8-0 polyglactin sutures, and the cornea was closed with 10-0 nylon.
DISCUSSION Aniridia can be classified as congenital or traumatic. Congenital aniridia is a developmental abnormality associated with low vision due to various types of ocular involvement such as optic nerve head and foveal hypoplasia, glaucoma, cataract, and aniridic keratopathy. Traumatic aniridia is usually accompanied by substantial damage in the anterior segment, resulting in corneal scarring, cataract, and secondary glaucoma.1–3 An intact iris diaphragm reduces the spherical and chromatic aberrations arising from the lens and increases the depth of focus.1,3,4,5 Therefore, aniridia causes incapacitating glare and photophobia. Eyelid surgery, colored contact lenses, and corneal tattooing may offer good cosmetic alternatives in severely traumatized eyes in which no useful vision is expected.6 However, if there is considerable vision after the acute effects of the trauma have subsided, visual rehabilitation with IOL implantation may be considered. A major complaint of IOL patients is photophobia due to wide iris defects. In that circumstance, artificial irides or colored IOLs, namely, black diaphragm IOLs, may offer a 1-step solution instead of standard IOL and artificial iris implantation. Use of an IOL with an artificial iris is not new. An anterior chamber IOL with an optic surrounded by
a colored diaphragm was designed by Choyce in 1959.7 In 1994, Sundmacher et al.8 implanted a newly designed posterior chamber black diaphragm IOL (Morcher GmbH) in 5 eyes with congenital aniridia and 8 eyes with traumatic aniridia. They reported improved visual acuity in 9 of 13 eyes with congenital aniridia and cataracts.9 Reinhard et al.10 reported satisfactory long-term results in 19 eyes of 14 patients with congenital aniridia treated with implantation of this IOL. The first reports described implantation of the IOL in the sulcus, but in 1999, Tanzer and Smith11 reported transscleral fixation of the aniridia IOL in a case with contact lens–intolerant traumatic aniridia and painful aphakic bullous keratopathy. Thompson et al.12 reported a series of 7 patients with traumatic aniridia treated with a black diaphragm IOL (Morcher type 67G); 6 required scleral fixation. Simultaneous penetrating keratoplasty was performed in 4 eyes, and anterior vitrectomy in 5 eyes. The BCVA improved in 5 patients and was unchanged in 1. Excellent visual acuity (6/6 to 6/9) was achieved in 3 patients. Lens decentration occurred in 2 eyes, postoperative hemorrhage in 1 eye, endophthalmitis in 1 eye, and RD in 1 eye. These complications were not observed in our series. Osher and Burk13 described 6 patients with aniridia who had implantation of various types of diaphragmatic devices, including endocapsular rings with multiple fins (Morcher type 50C), a sector iris device (Morcher type 96G) with a single fin, and a lens–iris device (Morcher type 67F). Two of the cases were traumatic, requiring cataract extraction and implantation of sector iris devices in the capsular bag. Mavrikaris et al.14 used Morcher aniridia IOL types 67F and 67G, the aniridia ring type 50C, and the coloboma diaphragm type 96G in 9 patients with traumatic iris defects, congenital aniridia or iris coloboma, and surgical or optical iridectomies. These cases differ from those in our series as none of our patients had capsule support and consequently needed transscleral fixation. Dong et al.15 implanted black diaphragm IOLs in 15 aphakic eyes with traumatic aniridia 6 to 72 months after PPV. The indications for PPV included unresolved vitreous hemorrhage, suppurative endophthalmitis, intraocular foreign-body removal, and retinal reattachment. One eye had extracapsular cataract extraction before PPV, 3 eyes lost their crystalline lens to trauma, and 11 eyes had simultaneous lensectomy during PPV. In all 15 eyes, the visual acuity improved and the glare decreased markedly. Omulecki and Synder2 reported a series of 6 patients treated for traumatic aniridia using PPV and transsclerally fixated black diaphragm IOLs. Total aniridia was observed in 3 eyes, and 4 eyes were aphakic. The BCVA
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improved in 5 patients and remained unchanged in 1. Good visual acuity (20/20 to 20/40) was achieved in all cases, and all IOLs were well-centered. No severe complications were noted. In our study, all eyes were aphakic with partial aniridia. Scleral buckling, PPV, endolaser photocoagulation under PFC with either intraocular gas or silicone oil injection were performed in all eyes. The patients were followed until the retina completely settled and then transscleral fixation of a black diaphragm IOL was performed. Scleral fixation was necessary because adequate capsule support was absent in all cases. The BCVA improved in 2 eyes and remained unchanged in the other 2 eyes compared with the preoperative visual acuity with aphakic correction. All patients reported a subjective decrease in glare and photophobia, although objective documentation was not performed. The most common complications reported after black diaphragm IOL implantation are persistent anterior chamber inflammation and glaucoma.2–4,10,12,15 Tanzer and Smith11 reported 3C anterior chamber cells and flare, which were treated aggressively with prednisolone acetate and subsided. Beltrame et al.4 reported postoperative persistent intraocular inflammation in 4 eyes, which disappeared within 3 months using topical steroids. No prolonged anterior chamber inflammation was documented in our series. Cystoid macular edema was detected in 1 patient 3 years and 5 years after the injury and managed with oral and topical steroids and intravitreal injection of triamcinolone acetonide, respectively. Elevated IOP was the most common postoperative complication reported after implantation of the fullsized black aniridia IOL.2–4,10–12,15 Elevated IOP occurred in 2 of 7 cases reported by Thompson et al.12 ; 1 required trabeculectomy. Three of 6 cases reported by Omulecki and Synder2 required topical glaucoma medications to achieve good IOP control. Dong et al.15 reported an IOP elevation in 5 of 15 cases. Four patients required timolol eyedrops and 1 patient, selective laser trabeculoplasty. Beltrame et al.4 reported 4 of 10 patients. Three cases were controlled medically and 1 case, surgically (trabeculectomy). Menezo et al.3 reported 2 eyes with postoperative ocular hypertension in 5 traumatic cases. One was controlled by topical bblockers, and the other needed 3 antiglaucoma drops and cyclodiode laser therapy. In our study, 2 eyes had elevated IOP and 1 patient was controlled after silicone removal. The other patient’s IOP was controlled with topical therapy after vitreoretinal surgery. Perforating or blunt eye injury often causes serious lenticular and vitreoretinal complications. We believe the first objective should be to provide the structural integrity of the eye by repairing the primary wound.
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Management of the coexisting traumatic cataract, lens dislocation, vitreous hemorrhage, and RD can be planned in the following weeks (or when they occur) under better conditions. This gives the surgeon and the patient the chance of orderly concomitant surgery with less risk. We usually prefer to place a buckle before PPV even if an RD is not detected because these patients have a high risk for retinal tears and detachment. We also perform endolaser treatment and gas or silicone oil injection to ensure better attachment of the retina. This series differs from those previously reported in that aspect. All our patients had transscleral fixation of a black diaphragm IOL when the retina was fully settled and the visual acuity was stable, well after the vitreoretinal surgery. Patients with trauma and multiple surgeries need ongoing posterior segment care postoperatively. One might think that black diaphragm IOLs would hinder visualization of the retinal periphery. However, visualization of the retinal periphery close to the equatorial region is possible with extreme ductions of the eye during binocular indirect ophthalmoscopy. More peripheral lesions may be visualized using a Mainster wide-field lens or a Goldmann 3-mirror lens with indentation. Several reports show that implantation of a black diaphragm IOL is a safe and effective option for managing eyes with traumatic aniridia and aphakia as it reduces glare and photophobia. However, the overall diameter of these IOLs is large and they are brittle because of the coloring procedure. They therefore necessitate wide incisions and careful handling. We suggest that proper management of the accompanying vitreoretinal problems in aphakic and aniridic eyes should be done before black diaphragm IOL implantation, which is challenging and involves a wide incision and transscleral fixation. REFERENCES 1. Mavrikakis I, Casey JMH. Phacoemulsification and endocapsular implantation of an artificial iris intraocular lens in traumatic cataract and aniridia [letter]. J Cataract Refract Surg 2002; 28:1088–1091 2. Omulecki W, Synder A. Pars plana vitrectomy and transscleral fixation of black diaphragm intraocular lens for the management of traumatic aniridia. Ophthalmic Surg Lasers 2002; 33: 357–361 3. Menezo JL, Martı´nez-Costa R, Cisneros A, Desco MC. Implantation of iris devices in congenital and traumatic aniridias: surgery solutions and complications. Eur J Ophthalmol 2005; 15:451–457 4. Beltrame G, Salvetat ML, Chizzolini M, et al. Implantation of a black diaphragm intraocular lens in ten cases of post-traumatic aniridia. Eur J Ophthalmol 2003; 13:62–68 5. Chen Y-J, Wu P-C. Favorable outcome using a black diaphragm intraocular lens for traumatic aniridia with total iridectomy. J Cataract Refract Surg 2003; 29:2455–2457
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6. Remky A, Redbrake C, Wenzel M. Intrastromal corneal tattooing for iris defects [letter]. J Cataract Refract Surg 1998; 24:1285–1287 7. Choyce P. Intra-Ocular Lenses and Implants. London, England, HK Lewis, 1964;21 8. Sundmacher R, Reinhard T, Althaus C. Black-diaphragm intraocular lens for correction of aniridia. Ophthalmic Surg 1994; 25:180–185 9. Sundmacher T, Reinhard T, Althaus C. Black diaphragm intraocular lens in congenital aniridia. Ger J Ophthalmol 1994; 3: 197–201 10. Reinhard T, Engelhardt S, Sundmacher R. Black diaphragm aniridia intraocular lens for congenital aniridia: long-term follow-up. J Cataract Refract Surg 2000; 26:375–381 11. Tanzer DJ, Smith RE. Black iris-diaphragm intraocular lens for aniridia and aphakia. J Cataract Refract Surg 1999; 25: 1548–1551 12. Thompson CG, Fawzy K, Bryce IG, Noble BA. Implantation of a black diaphragm intraocular lens for traumatic aniridia. J Cataract Refract Surg 1999; 25:808–813
13. Osher RH, Burk SE. Cataract surgery combined with implantation of an artificial iris. J Cataract Refract Surg 1999; 25: 1540–1547 14. Mavrikakis I, Mavrikakis E, Syam PP, et al. Surgical management of iris defects with prosthetic iris devices. Eye 2005; 19:205–209 15. Dong X, Yu B, Xie L. Black diaphragm intraocular lens implantation in aphakic eyes with traumatic aniridia and previous pars plana vitrectomy. J Cataract Refract Surg 2003; 29:2168–2173
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First author: Zeynep Ozbek, MD Department of Ophthalmology, Dokuz Eylul University, School of Medicine, Izmir, Turkey