Search Our Scientific Publications & Authors

Publications
find publications by category +
Translate page:

Clinical outcomes and visual prognostic factors in congenital aniridia.

Authors:
Adam Jacobson Shahzad I Mian Brenda L Bohnsack

BMC Ophthalmol 2022 May 25;22(1):235. Epub 2022 May 25.

Division of Ophthalmology, Ann & Robert H. Lurie Children's Hospital of Chicago, 225 E. Chicago Ave, Chicago, IL, 60611, USA.

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.

Download full-text PDF

 Source
http://dx.doi.org/10.1186/s12886-022-02460-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9131660PMC
May 2022

Publication Analysis

Top Keywords

congenital aniridia
16
final follow-up
12
prognostic factors
12
factors congenital
8
patients congenital
8
patients
5
eyes
5
congenital
5
surgery
5
rates 10-years
4
surgery average
4
survival rates
4
average 17 ± 23
4
longest survival
4
managing congenital
4
17 ± 23 surgeries/eye
4
final
4
surgeries/eye final
4
follow-up
4
follow-up median
4

Keyword Occurance

Similar Publications

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

Authors:
Andreas Viberg André Vicente Branka Samolov Jesper Hjortdal Berit Byström

Acta Ophthalmol 2022 Aug 9. Epub 2022 Aug 9.

Department of Clinical Sciences, Ophthalmology, Umeå University, Umeå, Sweden.

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. Read More

View Article and Full-Text PDF
August 2022
Similar Publications

[Implantation of an aniridia ring for complicated cataract and iris defects].

Authors:
Debora Scharf Lizaveta Chychko Victor A Augustin Ramin Khoramnia Gerd U Auffarth

Ophthalmologie 2022 Jun 29. Epub 2022 Jun 29.

Universitätsaugenklinik Heidelberg, Im Neuenheimer Feld 400, 69120, Heidelberg, Deutschland.

View Article and Full-Text PDF
June 2022
Similar Publications

[Autologous serum eye drops for therapy-resistant epithelial defects of the cornea : Impact of underlying disease and simultaneous amniotic transplantation in 990 applications].

Authors:
Laura Weischnur Kassandra Xanthopoulou Cristian Munteanu Marie Leonhard Loay Daas Berthold Seitz

Ophthalmologie 2022 Jun 23. Epub 2022 Jun 23.

Klinik für Augenheilkunde, Universitätsklinikum des Saarlandes (UKS), Kirrberger Str. 100, 66424, Homburg/Saar, Deutschland.

Background: The aim of the study was to evaluate the impact of simultaneous amniotic membrane transplantation (AMT), status of the cornea (own cornea vs. graft) and underlying disease on the success and recurrence rates of autologous serum (AS) in therapy-resistant epithelial defects.

Patients And Methods: Between 2007 and 2019, 990 treatments with AS in 703 eyes of 645 patients were retrospectively examined. Read More

View Article and Full-Text PDF
June 2022
Similar Publications

EPHA2 biallelic disruption causes syndromic complex microphthalmia with iris hypoplasia.

Authors:
Cécile Courdier Anna Gemahling Damien Guindolet Amandine Barjol Claire Scaramouche Laurence Bouneau Patrick Calvas Gilles Martin Nicolas Chassaing Julie Plaisancié

Eur J Med Genet 2022 Jul 31;65(10):104574. Epub 2022 Jul 31.

Service de Génétique Médicale, Hôpital Purpan, CHU de Toulouse, France; Centre de Référence pour les Affections Rares en Génétique Ophtalmologique (CARGO), CHU de Toulouse, France; Unité Inserm 1214 ToNIC, Hôpital Purpan, Toulouse, France. Electronic address:

Disruption of any of the ocular development steps can result in ocular defects such as microphthalmia, coloboma and anterior segment dysgeneses including aniridia and cataract. All of these anomalies can be isolated or seen in association with each other. Except for aniridia (almost exclusively due to PAX6 mutations), most of these congenital ocular malformations are related to a wide genetic heterogeneity, as hundreds of genes are implied in ocular development. Read More

View Article and Full-Text PDF
July 2022
Similar Publications

Response to: HCE-T cells express cornea-specific differentiation marker, PAX6 protein.

Authors:
Lorenz Latta Tanja Stachon Berthold Seitz Nóra Szentmáry

Graefes Arch Clin Exp Ophthalmol 2022 Jul 25. Epub 2022 Jul 25.

Dr. Rolf M. Schwiete Center for Limbal Stem Cell and Aniridia Research, Saarland University, Kirrberger Str. 100, 66424, Homburg, Saar, Germany.

View Article and Full-Text PDF
July 2022
Similar Publications
}