Custom Iris Prosthesis for Subtotal Traumatic Aniridia From Combat Ocular Trauma.

Traumatic aniridia from combat ocular trauma can cause visual disability. A 41-year-old male Army Veteran was referred for evaluation of light sensitivity and glare secondary to subtotal traumatic aniridia of his left eye from an improvised explosive device blast. A custom-made artificial iris prosthesis was implanted in the ciliary sulcus and secured using Gore-Tex sutures. After surgery, the patient reported improvement of his light sensitivity and quality of life. The custom iris prosthesis is a surgical option for visual disability resulting from traumatic aniridia from combat ocular trauma.

Pediatric glaucoma in aniridia in a population of Egyptian children.

PURPOSE: To report on the incidence, characteristics, and treatment of glaucoma in association with aniridia in a population of Egyptian children. METHODS: A retrospective chart review was conducted of children (<18 years of age) presenting with aniridia between 2007 and 2022. Diagnosis of glaucoma was based on cup:disk ratio (>0.3) and IOP (>16 mm Hg). Success of glaucoma surgery was defined as IOP ≤16 mm Hg and stable or regressing cup:disk ratio. RESULTS: A total of 93 eyes of 47 children were included. The mean patient age at presentation was 41.2 ± 44.7 months. The mean intraocular pressure (IOP), corneal diameter, cup:disk ratio, and axial length on presentation were 16.4 ± 10.3 mm Hg, 11.4 ± 1.3 mm, 0.4 ± 0.3, and 22.43 ± 2.6 mm Hg. Of eyes with IOP and cup:disk ratio data at presentation, 23 (30%) were diagnosed with glaucoma. Fourteen eyes underwent surgery after presentation; follow-up data were available for half the remaining eyes. At the final follow-up, glaucoma was present in 20 eyes (49%). Glaucoma surgery was performed in 17 eyes, with a complete or qualified success rate of 82.4% at final follow-up, with 15 eyes having at least 12 months' follow-up. CONCLUSIONS: In this study cohort, children with aniridia presented at around 3 years of age, with glaucoma at presentation in almost one-third of the cases.

Globe rupture with aphakia, aniridia, secondary glaucoma and late corneal decompensation: A multi-staged approach to management.

Severe blunt ocular trauma may result in immediate and delayed complications requiring appropriate management algorithms. We hereby report a case of globe rupture, aphakia, traumatic aniridia, and secondary glaucoma in a 33-year-old male following road traffic accident. He was treated initially by primary repair followed by novel combined approach of aniridia IOL with Ahmed glaucoma valve implantation. Delayed corneal decompensation required deferred penetrating keratoplasty. After a follow-up of 3.5 years after last surgery, patient maintains good functional vision with stable IOL, clear corneal graft and controlled intraocular pressure. A meticulously planned and staged management approach appears better suited in complex ocular trauma in such scenarios giving a good structural and functional outcome.

Artificial iris: state of the art.

Surgical correction of traumatic aniridia aims to improve the quality of vision, compartmentalize the anterior and posterior chamber, and re-establish a satisfying cosmetic appearance. Various types of prosthetic iris devices (PIDs) are available, which differ in technical difficulty of implant and design: artificial iris (AI)-intraocular lens prosthesis, endocapsular capsular tension ring-based PID, and customized AI. The choice depends on the preexisting clinical condition after severe ocular trauma and on patient functional and cosmetic expectations. This systematic review of the literature compared anatomical and functional outcomes of various types of PIDs. Of 185 articles found in the literature, 70 fulfilled the eligibility criteria. 5 subgroups of PIDs were Ophtec, artificial iris from Ophtec BV, Morcher GmbH, HumanOptics AG, and other prosthesis. Both glare and aesthetic outcome improved postoperatively; in comparison with other PIDs, intraocular pressure rise was higher in the Morcher group (40%), whereas prosthesis dislocation was higher in the Ophtec group (39%).

Artificial iris implantation in congenital aniridia: A systematic review.

Congenital aniridia is a rare, panocular disorder with a main phenotypic characteristic of a partial or complete absence of the iris existing alongside other ocular morbidities such as cataract, keratopathy, optic nerve and foveal hypoplasia, and nystagmus. The iris abnormality, however, often leads to symptoms such as photophobia, glare, and decreased visual acuity, as well as cosmetic dissatisfaction. Current management options for the iris deficit include colored iris contact lenses, corneal tattooing, and tinted contact lenses. Symptoms arising from small iris defects can be resolved with surgical management using micro-tying suture techniques such as McCannel or Siepser. Currently, larger iris defects can be treated with artificial iris implants. New prosthetic options range from colored intraocular lenses to flexible custom-made silicone iris implants. With a range of therapeutic options available and given the challenges of multiple comorbidities in aniridia, we evaluate the literature relating to the use of artificial iris implants in congenital aniridia, with a focus on the different surgical implantation techniques, the clinical outcomes achieved, complications occurred, and risk of bias of the studies included.

Surgical correction of corneal opacity and aniridia with penetrating keratoplasty and a new iris prosthesis implant.

PURPOSE: This study is to describe the clinical outcome of penetrating keratoplasty combined with implantation of a novel intraocular lens with an artificial iris, aided by continuous vitreous chamber infusion, in patients with severe aniridia and corneal alterations. METHODS: This was a prospective single-center case series study involving five patients with corneal alterations and aniridia. All subjects underwent simultaneous penetrating keratoplasty and implantation of a new intraocular lens with an artificial iris with the assistance of infusion into the vitreous chamber to regulate intraocular pressure during the surgical procedure. Visual acuity, corneal endothelial cell density, and intraocular pressure assessments were performed in the postoperative period. The final cosmetic outcome of the iris prosthesis placement was also evaluated. RESULTS: In all cases, increased visual acuity and a good aesthetic result were observed in all affected eyes except one in which, despite the excellent aesthetic outcome, the eye was very hypotonic as it had high myopia and had undergone several previous surgeries. CONCLUSION: The single surgical procedure combining implantation of an intraocular lens-iris prosthesis with penetrating keratoplasty is an effective technique for the simultaneous treatment of aphakia and aniridia. However, larger series with longer-term follow-up are needed to definitively establish the benefits of this technique.

Corneal transplantation in aniridia-related keratopathy with a two-year follow-up period, an uncommon disease with precarious course.

PURPOSE: The purpose of this study is to study the frequency, surgical transplantation technique and outcome in patients with aniridia-related keratopathy (ARK) with two-year follow-up period. METHODS: A retrospective registry-study including all ARK cases performed in Sweden and Denmark between 2001 and 2016 and registered in the Swedish Cornea Transplant Registry. RESULTS: A total of 36 eyes of 26 patients were subjected to corneal transplantation due to ARK during 2001 to 2016. Penetrating keratoplasty (PK) was the procedure of choice in 58.3% (n = 21) of the eyes, followed by a combination of PK and limbal stem cell transplantation in 13.9% (n = 5) and keratolimbal allograft in 13.9% (n = 5). Boston keratoprosthesis was used in 8.3% (n = 3), and anterior lamellar keratoplasty in 5.6% (n = 2). Thirteen of the procedures (36.1%) were retransplantations. Two years after surgery 26 cases were available to follow-up of which 16 of the grafts were functioning (61.5%). The median visual acuity showed a trend of improvement from hand motion to counting fingers. CONCLUSIONS: A majority of the ARK cases (61.5%) had a graft providing useful vision for the patient 2 years after corneal transplantation, but the visual gain was modest at best. Longer follow-up time is required to evaluate functional graft outcomes. Despite the introduction of limbal stem cell transplantation as a suitable treatment, PK was the most common surgical method in the present study.

Isolated traumatic aniridia with full and partial iris expulsion in pseudophakic eyes.

BACKGROUND: Total aniridia after ocular trauma without disruption of the intraocular lens (IOL) has been reported in patients with a history of small-incisional cataract surgery. We report one case each of total and partial aniridia after accidental falls experienced by two elderly Japanese women. CASE PRESENTATIONS: Case 1. A 76-year-old woman with a history of small-incisional cataract surgery more than 10 years previously fell onto concrete and had a contusion that affected the left side of her face. At the initial visit, the best-corrected visual acuity (BCVA) was hand motions and the intraocular pressure (IOP) was 38 mmHg in her left eye (OS). A blood clot was present in the well-formed anterior chamber and expulsed iris tissue was seen beneath the conjunctiva. Exploratory surgery showed no scleral laceration other than the previous sclerocorneal tunnel. After hyphema removal, total aniridia and an intact in-the-bag fixed IOL were seen. By 4 months, the BCVA was 1.2 and the IOP was 13 mmHg OS. CASE 2: An 88-year-old woman with a history of small-incisional cataract surgery more than 10 years previously had a fall that resulted in right-sided zygomatic and maxillary bone fractures. The BCVA was light perception and the IOP was 29 mmHg in her right eye (OD). Exploratory surgery showed no scleral laceration and the previous sclerocorneal tunnel was found; iris strand prolapsing from the sclerocorneal tunnel was seen. After hyphema removal, partial iris loss and an intact lens position were seen. By 1 week postoperatively, the BCVA was 0.05 OD and the IOP was 12 mmHg OD. CONCLUSIONS: It has been postulated that previously created small-incision tunnels can function as release valves during blunt trauma by preventing further global rupture and limiting IOL prolapse or retinal injury. Our cases suggested this can happen even long periods after cataract surgery. The case with partial aniridia demonstrated the process of the expulsive aniridia, and its findings do not contradict the postulated mechanisms.

Descemet Stripping Endothelial Keratoplasty for Congenital Aniridia: An Interesting and Challenging Story.

Congenital aniridia is a rare condition affecting a wide range of ocular structures, from the ocular surface to the retina. We present the case of a 59-year-old woman with PAX6- and WT1-negative congenital aniridia who developed aniridia-associated keratopathy and progressive endothelial dysfunction with corneal decompensation after cataract surgery. The patient underwent successful ultrathin Descemet stripping endothelial keratoplasty. Despite the challenges faced with an unstable iridolenticular diaphragm, we were pleasantly surprised to see improvement not only of corneal edema and endothelial function but also of the whole cornea, including anterior corneal anatomy and appearance. In conclusion, endothelial transplantation in a patient with aniridia resulted in improvement of all the corneal structures from the endothelium to the stroma, epithelium, and possibly even the ocular surface.

Clinical outcomes and visual prognostic factors in congenital aniridia.

BACKGROUND: Evaluate outcomes and identify prognostic factors in congenital aniridia. METHODS: Retrospective interventional case series of patients with congenital aniridia treated between 2012-2020. Ocular examination and surgical details were collected. Surgical failure was defined as disease progression or need for additional surgery for same/related indication. Kaplan-Meier survival curves, Wilcoxon test, and univariate and multivariate linear regression analyses were performed. RESULTS: Ninety-four patients with congenital aniridia presented at median 19.0 years. Two-thirds of patients underwent ≥ 1intraocular surgery, with average of 1.7 ± 2.3 surgeries/eye. At final follow-up (median 4.0 years), 45% of eyes had undergone lensectomy. Aphakic eyes showed worse visual acuity (VA) than phakic or pseudophakic eyes. Glaucoma affected 52% of eyes, of which half required IOP-lowering surgery. Glaucoma drainage devices showed the highest success rate (71%) at 14.2 ± 15.4 years of follow-up. Keratopathy affected 65% of eyes and one-third underwent corneal surgery. Keratoprosthesis had the longest survival rates at 10-years (64% with 95% CI [32,84]). LogMAR VA at presentation and final follow-up were not statistically different. Half of patients were legally blind at final follow-up. Final VA was associated with presenting VA, glaucoma diagnosis, and cataract or keratopathy at presentation. Penetrating keratoplasty and keratoprosthesis implantation correlated with worse BCVA. CONCLUSIONS: Most aniridic patients in this large US-based cohort underwent at least 1 intraocular surgery. Cataract, glaucoma, and keratopathy were associated with worse VA and are important prognostic factors to consider when managing congenital aniridia.

KeraKlear Artificial Cornea Implantation Assisted by Femtosecond Laser in Eyes With Aniridia.

PURPOSE: The purpose of this study was to evaluate the feasibility of treating aniridia-associated keratopathy with a nonpenetrating artificial cornea in 2 patients with corneal blindness secondary to aniridia. METHODS: This was a prospective, nonrandomized, interventional study of 2 consecutive patients with corneal blindness caused by aniridia. Ophthalmological examination was performed before the nonpenetrating keratoprosthesis surgery and then repeated 1, 7, 15, 30, 90, and 180 days and subsequently every 90 days thereafter. Optical coherence tomography was performed 90 days postsurgery to assess the position of the implant. RESULTS: Visual acuity improved significantly after the KeraKlear surgery. Postoperative findings included periprosthetic corneal thinning, neovascularization, and retroprosthetic opacity. CONCLUSIONS: KeraKlear nonpenetrating artificial corneas represent a promising alternative to keratolimbal allografts and Boston keratoprosthesis for the treatment of aniridia-associated keratopathy.

Endocapsular artificial iris implantation for iris defects: Reducing symptoms, restoring visual function and improving cosmesis.

BACKGROUND: To investigate repair of iris defects by endocapsular implantation of an artificial iris, in relation to visual outcomes, safety profile and patient satisfaction. METHODS: Retrospective, consecutive case series from Greenlane tertiary teaching hospital and Eye Institute, Auckland, New Zealand. Medical records of patients implanted with an endocapsular artificial iris were reviewed and followed for minimum 3 months. Patient characteristics, surgical management, clinical outcomes and subjective responses were recorded. RESULTS: Nineteen artificial irises were implanted in 18 patients. Etiologies were iris melanotic lesion excision (73.7%), trauma (10.5%), congenital aniridia (10.5%) and Urrets-Zavalia syndrome (5.3%). During postoperative follow-up [14.1 ± 12.4 months (range: 3 to 59 months)], best corrected visual acuity (BCVA) and intraocular pressure (IOP) did not change significantly [BCVA, 0.23 logarithm of the minimum angle of resolution (logMAR) (20/32 Snellen) preoperatively vs. 0.18 logMAR postoperatively (20/25 Snellen) (Z = -0.222, p = 0.824); IOP, 15 mmHg preoperatively vs. 17 mmHg postoperatively (Z = 1.377, p = 0.1447)]. Mild or self-limiting complications included: elevated IOP (42.1%), cystoid macular oedema (15.8%); persisting postoperative uveitis (15.8%) and minor vaulting of the prosthesis (15.7%). Moderate or severe complications included significant vaulting of prosthesis requiring surgical revision (5.3%) and a single eye (5.3%) with trabeculectomy and corneal graft failure. 94.4% of patients were very satisfied with the cosmesis and would be highly likely to have the procedure again. CONCLUSIONS: This study confirms that endocapsular insertion of an artificial iris is typically associated with good functional and cosmetic results and a relatively low risk of significant complications.

Traumatic aniridia with extensive episcleral pigmentation: a case report.

BACKGROUND: Traumatic aniridia has been documented in eyes with a history of cataract extraction through a clear corneal wound. The proposed hypothesis is that the iris tissue was squeezed out from the corneal wound as it is a relative weak point. However, traumatic aniridia with extensive pigmentation of the episclera has never been reported. CASE PRESENTATION: A patient, who has surgical histories of trabeculectomy and cataract surgery many years ago, presented with refractory high intraocular pressure (IOP), almost complete loss of the iris, and diffuse pigmentation of the episclera after he had suffered from a contusion injury. In addition to numerous pigment particles and cells in the anterior chamber and a well-centered intraocular lens, protruding uvea tissue with overlying conjunctiva adjacent to the site of trabeculectomy was noted. Gonioscopy showed absence of the iris with clear view of the ciliary body. CONCLUSIONS: The distinct presentation of this case indicates that the torn iris was displaced to the trapdoor instead of the clear cornea incision and was confined to the subconjunctival space. The scleral fistula serves as a less resistant point for releasing pressure compared to a healed corneal wound when the eye encounters a contusion injury. Further treatment options to lower IOP include repeated trabeculectomy, implantation of glaucoma drainage device, and endoscopic cyclophotocoagulation. Transscleral cyclophotocoagulation may be considered only after episcleral pigmentation has become less so as to avoid the risk of surface burn.

Long-term outcomes in Primary congenital glaucoma, aniridia and anterior segment dysgenesis.

AIM: To determine the long-term outcomes of a cohort of complex patients with primary congenital glaucoma, aniridia and anterior segment dysgenesis. METHODS: Retrospective consecutive series between 1990-2021 in two UK tertiary centres: Guy's and St Thomas' NHS Foundation Trust and King's College Hospital NHS Foundation Trust. We recorded the number and types of surgical and laser treatments along with preoperative and postoperative data, including intraocular pressures (IOP) and anti-glaucoma medications. RESULTS: A total of 41 eyes of 21 patients were included. Primary diagnoses were primary congenital glaucoma in 16 eyes (39.0%), aniridia in 14 eyes (34.2%), and anterior segment dysgenesis in 8 eyes (19.5%). Sixteen eyes (39.0%) had one or more glaucoma surgery or laser procedures for advanced glaucoma, and the long-term follow-up was 12.8 ± 3.6 years. There was a significant decrease in postoperative IOP (mmHg) at 3 months (16.5 ± 1.6; p = 0.0067), 6 months (18.7 ± 2.1; p = 0.0386), 12 months (18.6 ± 1.7; p = 0.0229), 3 years (14.7 ± 1.2; p = 0.0126), 5 years (15.5 ± 1.8; p = 0.0330) and 10 years (15.4 ± 2.3; p = 0.7780), compared to preoperatively (24.1 ± 2.6). Surgical success (complete and qualified) was 62.5%, 50.0%, 43.8%, 46.2%, 45.5% and 28.6% at 3 months, 6 months, 12 months, 3 years, 5 years and 10 years, respectively. There was no significant change in the number of anti-glaucoma drugs postoperatively (p > 0.05). Four eyes (25.0%) had postoperative complications (hyphaema, hypotony) that resolved after conservative management. CONCLUSIONS: Surgical management of these complex eyes with advanced glaucoma is challenging. Overall, the cohort had good surgical outcomes with a significant decrease in IOP by 36.1% after long-term follow-up.

A CASE OF TRAUMATIC SOFT CONTACT LENS DISLOCATION INTO THE VITREOUS SPACE AFTER CORNEAL PERFORATION IN A PHAKIC EYE: A NOVEL INDICATION FOR MEMBRANEBLUE-DUAL.

PURPOSE: To describe a case of traumatic soft contact lens dislocation into the vitreous space after a large-sized corneal perforation with a clout nail, causing aniridia but leaving the crystalline lens intact. METHODS: A case report of a 44-year-old male patient with a corneal perforation. RESULTS: The patient presented with a traumatic corneal perforation after a large clout nail impacted on his left eye. Aniridia and inferior zonulolysis were seen, but the crystalline lens was perfectly clear. Because computed tomography imaging and B-scan ultrasonography showed no signs of intraocular foreign body, a vitrectomy was performed seven days after primary repair to allow for proper wound closure. During vitrectomy, a completely intact soft contact lens was found in the vitreous cavity. The soft contact lens was stained with MembraneBlue-Dual (D.O.R.C., the Netherlands) to enhance visualization and ensure complete removal by the vitreous cutter. CONCLUSION: MembraneBlue-Dual can be used to facilitate complete removal of a clear and otherwise difficult to see corneal contact lens from the vitreous cavity. This case also demonstrates the importance of exploratory vitrectomy in all trauma cases with posterior segment involvement, even when imaging modalities show no signs of intraocular foreign bodies.

[Exchange of aniridia IOLs for customized iris IOL implants]. / Austausch von Aniridie-IOL gegen individuelle Iris-IOL-Implantate.

BACKGROUND: Three groups of iris prostheses can be distinguished for the surgical treatment of iris defects: (1) segmental iris implants, (2) combined iris diaphragm intraocular lenses (IOL) and (3) pure iris implants. Most iris reconstructions are accompanied by aphakia correction with secondary IOL implantation. Although the primary goal is to create a pupil and to improve glare perception, contrast sensitivity and visual acuity, the esthetic result is also a relevant component. OBJECTIVE: Functional and esthetic results after replacement of an aniridia IOL implant with a custom-made artificial iris with IOL. MATERIAL AND METHODS: In this retrospective study with seven eyes from seven patients, an iris diaphragm IOL (Morcher GmbH, Stuttgart, Germany) was exchanged for medical reasons (subluxation) against a custom-made artificial iris made of silicone (ArtificialIris, HumanOptics, Erlangen, Germany) in combination with a sutured IOL. The follow-up period was at least 3 months. Best corrected distance visual acuity (BCVA), endothelial cell count (ECC), complications, glare perception as well as esthetic outcome and patient satisfaction were evaluated. RESULTS: The BCVA and ECC showed no statistically significant change between the preoperative and postoperative values (p > 0.05). There was a decentration of the iris IOL implant of 0.27 ± 0.19 mm three months postoperatively. On a visual analogue scale (VAS) from 1 to 10 (1 = not satisfied at all, 10 = extremely satisfied), satisfaction with the overall result was rated 8.6 ± 2.5. Subjective glare perception improved to 5.6 ± 3.5 and subjective esthetic impairment improved to 2.4 ± 2.0 on the VAS (1 = none, 10 = extremely strong). Postoperative complications included transient intraocular hypotonia in two eyes, intraocular pressure increase in two eyes, retinal detachment and transient vitreous hemorrhage in one eye each. Of the seven patients six would repeat the procedure. CONCLUSION: Compared to a rigid aniridia IOL implant, replacement with a custom-made artificial iris in combination with an IOL provides not only a good functional result but simultaneously also an esthetically pleasing result.

[Pupil reconstruction with an artificial iris]. / Pupillenrekonstruktion mit einer künstlichen Iris.

BACKGROUND: Patients with iris defects suffer from severe visual impairment, especially increased glare sensitivity, and cosmetic disturbances. This can constitute a great psychological strain for patients. In the recent past the treatment consisted of iris print contact lenses, sunglasses and simple iris prostheses. The indications for surgical treatment are colobomas of the iris, aniridia, traumatic iris defects and persistent mydriasis. The aim of this review article is to investigate the surgical approaches, complications, functional and aesthetic outcome after implantation of an individual artificial iris prosthesis made from silicone. OBJECTIVE: Analysis of the literature on the topic of surgical iris reconstruction with an artificial iris in combination with the own experience in more than 120 patients treated by the author in the last 10 years. MATERIAL AND METHODS: The custom-made flexible silicone iris prosthesis ArtificialIris (HumanOptics, Erlangen, Germany) assessed in this review is an innovative and versatile option for surgical treatment of iris defects. Patients were examined before and after iris reconstruction with respect to feasibility, complications and outcome. RESULTS: Change of best corrected visual acuity, intraocular pressure, pupillary opening, glare, contrast sensitivity, endothelial cell count, anterior chamber depth, anterior chamber angle and patient satisfaction were assessed. Furthermore, complications and color match to the residual and fellow iris were assessed. CONCLUSION: The implantation of an artificial iris is an effective option for the treatment of extensive traumatic iris defects and leads to an individual aesthetically appealing and good functional outcome as well as high patient satisfaction; however, this is an intervention that should not be underestimated because of a flat learning curve and various complications that can occur.

Mechanical modifications of soft actuators for the use as a dynamic iris implant.

Aniridia is a condition characterized by defects or absence of the iris. Since the eyes are a central point of attention in the human face, these deformities are often covered with cosmetic implants. However, patients suffer from the static pupil diameter of these implants, resulting in high light sensitivity or inadequate night vision. Therefore, we present a functional iris implant based on dielectric elastomer actuators. These electric drives are characterized by a silent and continuous adaptation as well as a small construction volume and a low heat emission. Since they normally exhibit in-plane uniaxial motion, this displacement must be focused to operate similarly to the iris sphincter. Therefore, we investigated possible mechanical modifications of the setups to generate a directional motion. The results of the study are presented and discussed.Clinical Relevance- The proposed system design enables the functional treatment of aniridia and other accidental iris defects. In addition, the system serves as a basis for later developments of e.g. functional lenses that allow focus adjustment.