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Edward J. Holland – One of the best experts on this subject based on the ideXlab platform.

  • surgical and visual outcomes of the type i boston keratoprosthesis for the management of aniridic fibrosis syndrome in congenital Aniridia
    American Journal of Ophthalmology, 2012
    Co-Authors: Pejman Bakhtiari, Edward J. Holland, Clara C Chan, Jeffrey D Welder, Jose De La Cruz, Ali R Djalilian


    Purpose To report the clinical features and surgical management of aniridic fibrosis syndrome using the type I Boston Keratoprosthesis (KPro). Design Interventional case series. Methods Retrospective chart review of 9 eyes in 9 patients with congenital Aniridia that developed aniridic fibrosis syndrome. Results All patients had clinical diagnosis of congenital Aniridia. Previously, all patients had undergone cataract surgery with posterior chamber intraocular lens (IOL) implantation and 7 patients had existing tube shunts. In all cases, fibrosis presented as progressive retrocorneal and retrolenticular membrane formation causing displacement of the IOL and secondary corneal decompensation. Two eyes had tractional folds in the retina with posterior extension of the membrane. The management included IOL explantation in 7 of 9 cases, removal of fibrosis with pars plana vitrectomy in all 9 patients, and implantation of a type I Boston KPro in all eyes. At a mean final follow-up of 26.1 months (range 6 to 48 months), vision remained improved in all patients. No patient had recurrence of the fibrotic membrane after KPro implantation. Conclusion This study represents another case series describing aniridic fibrosis syndrome and the largest study to report utilization of the type I Boston KPro in such patients. As the fibrosis can cause IOL dislocation, corneal decompensation, hypotony, and retinal detachment, monitoring for aniridic fibrosis syndrome in congenital Aniridia with early surgical intervention is recommended. Type I Boston KPro may be considered in the surgical treatment of this condition.

  • congenital Aniridia variant minimally abnormal irides with severe limbal stem cell deficiency
    Ophthalmology, 2011
    Co-Authors: Heather M Skeens, Brian P Brooks, Edward J. Holland


    Purpose To clinically and molecularly characterize a group of patients with progressive limbal stem cell deficiency (LSCD) due to aniridic keratopathy (AK), but with minimally affected irides. Design Retrospective case series. Participants A total of 12 eyes of 6 patients who underwent keratolimbal allograft (KLAL) for AK in the absence of the classic stigmata of Aniridia at the Cincinnati Eye Institute/University of Cincinnati between 2000 and 2007. Methods Retrospective chart review. Main Outcome Measures Ocular surface stability after KLAL and change in visual acuity. Results Subjects’ mean age was 32.57 years, 66% were female, and mean follow-up was 64.4 months (range, 20–115 months). All patients presented with a decline in their vision secondary to LSCD. Average preoperative best-corrected visual acuity (BCVA) logarithm of the minimum angle of resolution (logMAR) was 1.4 (range, 0.10–2.8). All patients had minimally affected irides with subtle abnormal findings, including ectropion uveae and stromal atrophy. All patients developed severe LSCD and required KLAL. Average postoperative logMAR BCVA was 0.35 (range, 0.00–1.00). All ocular surfaces remained stable throughout the follow-up period. Family history consistent with autosomal dominant inheritance was positive in 4 of 6 patients. PAX6 genetic testing identified 2 pathologic mutations and 1 possible disease-causing variant. Conclusions Aniridic keratopathy may present in the absence of other classic stigmata of Aniridia and be associated with minimally affected irides. A subset of these patients has definitive mutations in PAX6 and once identified can be counseled appropriately. These patients respond well to KLAL and may therefore benefit from early detection. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

  • a progressive anterior fibrosis syndrome in patients with postsurgical congenital Aniridia
    American Journal of Ophthalmology, 2005
    Co-Authors: Julie H Tsai, Gary S. Schwartz, John M Freeman, Chichao Chan, Elizabeth Derby, Michael R Petersen, Edward J. Holland


    Purpose To report the characteristics of a newly recognized clinical entity in congential Aniridia that we have termed aniridic fibrosis syndrome . Design Interventional case series. Methods Retrospective chart review of 155 eyes in 80 patients with congenital Aniridia was carried out to identify and characterize eyes that had anterior chamber fibrosis. Histopathologic evaluation was performed in three eyes. Results Seven eyes in six patients were identified to have aniridic fibrosis syndrome. All eyes had undergone previous intraocular anterior segment surgery, some eyes with multiple procedures. Seven eyes had undergone cataract surgery with posterior chamber intraocular lens; six eyes had undergone previous implantation of tube shunt devices, and four eyes had undergone previous penetrating keratoplasty. Clinically, the syndrome was characterized by a progressive retrolenticular and retrocorneal membrane that caused forward displacement of intraocular lenses. Surgical findings indicated that the fibrotic membrane also can involve the ciliary body and anterior retina. Histopathologic evidence from three eyes indicated that the extensive fibrotic tissue originated from the root of the rudimentary iris and entrapped the intraocular lens haptics. Endothelial decompensation that was subsequent to the formation of the aniridic fibrosis syndrome was seen in all eyes. Conclusion Aniridic fibrosis syndrome is characterized by the development of a progressive anterior chamber fibrosis. A possible mechanism that promotes the formation of this fibrotic material may be the proximity or touching of intraocular devices on immature vessels in the rudimentary iris found in Aniridia. Patients with Aniridia with a history of penetrating keratoplasty, intraocular lenses, and tube shunts should be monitored for aniridic fibrosis syndrome; early surgical intervention is recommended.

P. Alessandro Mutolo – One of the best experts on this subject based on the ideXlab platform.

  • Combined pars plana vitrectomy and artificial iris diaphragm implant after globe rupture
    Graefe's Archive for Clinical and Experimental Ophthalmology, 2008
    Co-Authors: Tommaso Rossi, Barbara Boccassini, Mario Iossa, Guido Lesnoni, Maria Giulia Mutolo, P. Alessandro Mutolo


    Background Retinal detachment (RD) associated with Aniridia due to globe rupture (GR) is an uncommon condition with a severe prognosis. Surgical technique must address anterior and posterior segment issues secondary to the altered compartmentalization and increased risk for corneal toxicity. The purpose of this paper is to report a series of GR patients undergoing combined pars plana vitrectomy (PPV) and artificial iris diaphragm (AID) implant for the repair of RD associated to Aniridia. Methods The authors retrospectively reviewed 12 consecutive patients operated on by a single surgeon. Surgery consisted of a standard three-port PPV with extensive bimanual dissection of vitreous base and ciliary body membrane and combined AID implant. Office visits included Snellen visual acuity (VA), intraocular pressure measurement, biomicroscopy and indirect ophthalmoscopy. AID prosthesis included aniridic IOLs, Heimann’s PMMA and silicone diaphragm. Results Mean age was 53 years and mean follow-up was 19 months. At the end of follow-up, seven patients gained more than two lines (58.3%), two lost their vision (16.6%) and three were unchanged (25%). Seven patients (58.3%) had a VA better than 20/400 and one (8%) 20/40 vision. Eight patients (66.6%) retained a clear cornea, two (16.6%) had minimal corneal oedema and two (16.6%) corneal decompensation. Implanted prosthesis included two silicone diaphragms, four PMMA diaphragms and six aniridic IOLs. After an average 1.6 operations, the retina was completely attached in six patients (50%), partially attached in four (33.3%) and detached in two (16.6%). Conclusion RD associated to GR carries a guarded prognosis both due to RD complexity and hypotony. The combined repair of RD and Aniridia after GR offers the advantage of addressing all issues at one time allowing correct eye compartmentalization and better tamponade effect. Successful anatomical and functional results can be achieved although multiple surgeries are often needed.

Lixin Xie – One of the best experts on this subject based on the ideXlab platform.

  • Long term complications of black diaphragm Aniridia intraocular lens implant in traumatic Aniridia
    [Zhonghua yan ke za zhi] Chinese journal of ophthalmology, 2009
    Co-Authors: Xiao-guang Dong, Jun Cheng, Lixin Xie


    Objective To analyze long-term complications of black diaphragm Aniridia intraocular lens (IOL) implant in traumatic aniridaia and to investigate the causes and precautionary measures.Methods This is a retrospective consecutive case study.Five traumatic Aniridia cases undertaken black diaphragm Aniridia IOL implantation in Shandong Eye Institute and Hospital and developed severe complications during long-term follow-up were analyzed, including 4 males and 1 female, averaged 26.8ears old.The follow-up time varied from 42 months to 108 months.Two cases had implantation of a secondary black diaphragm IOL after pars plana vitrectomy.Two cases had implantation of a black diaphragm IOL together with cataract extraction.One case implanted a black diaphragm IOL only.Results All patients felt well within a short period after the surgery, symptoms of glare and photophobia were improved.A better visual acuity was obtained in a short-term period.However, severely secondary glaucoma and bullous keratopathy occurred in the long-term follow up.Visual acuity decreased to counting finger or hand motion.All cases received penetrating keratoplasty and IOL explantation.Conclusions Black diaphragm Aniridia intraocular lens implantation may induce severe long-term complications.The indications should be selected seriously and closely follow-up is important.

    Key words:
    Aniridia;  Black diaphragm intraocular lens;  Eye injuries

  • black diaphragm intraocular lens implantation in aphakic eyes with traumatic Aniridia and previous pars plana vitrectomy
    Journal of Cataract and Refractive Surgery, 2003
    Co-Authors: Xiao-guang Dong, Lixin Xie


    Purpose: To evaluate the efficacy of secondary black diaphragm intraocular lens (IOL) implantation in aphakic eyes with traumatic Aniridia and previous pars plana vitrectomy (PPV).

    Setting: Shandong Eye Institute and Hospital, Qingdao, China.

    Methods: This retrospective study comprised 15 aphakic eyes with Aniridia and no vitreous that had implantation of a secondary black diaphragm IOL 6 to 72 months after PPV. The PPV was performed as a result of trauma to the posterior segment. Before implantation of the IOL, all patients had reduced visual acuity from aphakia and intolerable glare from Aniridia. Eyes were aphakic as a result of previous extracapsular cataract extraction (1 eye), lens extrusion during trauma (3 eyes), or simultaneous cataract lensectomy during PPV (11 eyes). Significant iris defects were present, with 9 eyes being aniridic after the injury. The mean follow-up was 17 months (range 3 to 34 months). The postoperative visual acuity, intra- ocular pressure (IOP), endothelial cell density, IOL centration, and intraocular inflammation were monitored.

    Results: All 15 eyes had improved visual acuity and marked glare reduction after IOL implantation. No major IOL decentration was seen. Five patients had increased IOP 3 to 5 days after IOL implantation; 3 were known to have secondary glaucoma from trauma before surgery and their IOP was controlled preoperatively (< 21 mm Hg) with timolol 0.5% eyedrops. The other 2 patients had normal- appearing angles, and 1 was diagnosed with hemolytic glaucoma after IOL insertion. Postoperative elevated IOP was controlled by timolol eyedrops (4 eyes) or selective laser trabeculoplasty (1 eye). Hyphema was seen in 1 patient on the first day after IOL implantation, but no further bleeding was noted. Vitreous hemorrhage was seen the first day after IOL insertion in 2 patients. One resolved without sequelae; the other required vitreous washout. Retinal detachment and cystoid macular edema did not occur in any patient. Conclusions: Black diaphragm IOL implantation can be considered in eyes with coexisting aphakia and Aniridia and without vitreous. Intraoperative IOP regulation is crucial in vitrectomized eyes to prevent complications such as expulsive hemorrhage. In general, visual function was better after IOL insertion as a result of better visual acuity and glare reduction. Although this IOL appears to be safe, long-term results must be assessed in studies with a longer follow-up and a larger study group.