TY - JOUR
T1 - Management of Congenital Aniridia-Associated Keratopathy
T2 - Long-Term Outcomes from a Tertiary Referral Center
AU - Yazdanpanah, Ghasem
AU - Bohm, Kelley J.
AU - Hassan, Omar M.
AU - Karas, Faris I.
AU - Elhusseiny, Abdelrahman M.
AU - Nonpassopon, Manachai
AU - Niparugs, Muanploy
AU - Tu, Elmer Y.
AU - Sugar, Joel
AU - Rosenblatt, Mark I.
AU - Cortina, Maria S.
AU - Djalilian, Ali R.
N1 - Funding Information:
Analysis of visual acuities and AAK stage-based treatments over a long follow-up period in patients with congenital aniridia led to the development of a data-supported algorithm for management of aniridia-associated keratopathy. In early stage AAK (Stages I and II), patients are best served by using medical management and follow-up. With more severe presentations (Stages III, IV, and V), LSCT and Boston KPro should be considered according to the clinical staging and evaluation of each individual eye ( Figure 3 ). After advanced corneal surgeries are performed, postoperative management is of paramount importance for long-term success. All authors have completed and submitted the ICMJE form for Disclosure of Potential Conflicts of Interest and none were reported. FUNDING/SUPPORT: This work was supported by R01 EY024349 (A.R.D.) and Vision Research Core grant EY01792 (M.I.R.) from US National Institutes of Health, United States / National Eye Institute, United States , and an unrestricted grant to the department from Research to Prevent Blindness, United States . The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. FINANCIAL DISCLOSURES: Ali Djalilian is a consultant for Novartis, Combangio, and Dompé. Mark I. Rosenblatt is a consultant for Laurel Therapeutics, Silk Tears, Roche, AbbVie, Sarentis Therapeutics, and PanOptica. Elmer Y. Tu is a consultant for Kedrion and Okogen. Maria S. Cortina is a consultant for Eversight Eye Bank and Gore. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Funding Information:
All authors have completed and submitted the ICMJE form for Disclosure of Potential Conflicts of Interest and none were reported. FUNDING/SUPPORT: This work was supported by R01 EY024349 (A.R.D.) and Vision Research Core grant EY01792 (M.I.R.) from US National Institutes of Health, United States/National Eye Institute, United States, and an unrestricted grant to the department from Research to Prevent Blindness, United States. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. FINANCIAL DISCLOSURES: Ali Djalilian is a consultant for Novartis, Combangio, and Domp?. Mark I. Rosenblatt is a consultant for Laurel Therapeutics, Silk Tears, Roche, AbbVie, Sarentis Therapeutics, and PanOptica. Elmer Y. Tu is a consultant for Kedrion and Okogen. Maria S. Cortina is a consultant for Eversight Eye Bank and Gore. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Publisher Copyright:
© 2019 Elsevier Inc.
PY - 2020/2
Y1 - 2020/2
N2 - Purpose: To report the outcomes of medical and surgical management for congenital aniridia-associated keratopathy (AAK) over a long-term follow-up period. Design: Retrospective, comparative case series. Methods: Medical records of patients diagnosed with congenital aniridia were retrospectively reviewed. Age, sex, ethnicity, follow-up time, AAK stage, noncorneal abnormalities, ocular surgeries, and complications were recorded. The visual acuity equivalent (VAE), approximate Early Treatment Diabetic Retinopathy Study (appETDRS) letter score, was calculated using recorded Snellen visual acuities. Results: A total of 92 eyes of 47 patients (31 females) with mean age of 48.0 ± 18.0 years and mean follow-up of 78.6 ± 42.2 months were included. At the initial visit, 12 eyes (13%) were classified as Stage I AAK, 33 eyes (35.9%) were Stage II, 25 eyes (27.2%) were Stage III, 17 eyes (18.5%) were Stage IV, and 5 eyes (5.4%) were Stage V. Limbal stem cell transplantation (LSCT) and Boston keratoprosthesis (KPro) were frequently performed in eyes with Stages III-V. These advanced corneal surgeries significantly improved the median (95% confidence interval [CI]) of calculated appETDRS scores from 2 (0-20) to 26 (15-41) (Snellen values, 20/20,000 to 20/300; P = 0.0004). Patients with earlier Stages (I-II) of AAK were managed medically and had stable visual acuity through their final visits (appETDRS score of 26 [20-35] to 35 [26-35]; Snellen, 20/300 to 20/200; P > 0.05). The appETDRS VAE was significantly improved from 20 (0-35) to 30 (20-55), Snellen, 20/400 to 20/250, following LSCT (P = 0.021) and from 2 (0-20) to 2 (0-41) after KPro; Snellen, 20/20,000 VAE but with improved 95% CI after follow-up (P = 0.019). Conclusions: With proper characterization and staging of AAK, individualized medical and advanced surgical interventions preserves and improves visual acuity.
AB - Purpose: To report the outcomes of medical and surgical management for congenital aniridia-associated keratopathy (AAK) over a long-term follow-up period. Design: Retrospective, comparative case series. Methods: Medical records of patients diagnosed with congenital aniridia were retrospectively reviewed. Age, sex, ethnicity, follow-up time, AAK stage, noncorneal abnormalities, ocular surgeries, and complications were recorded. The visual acuity equivalent (VAE), approximate Early Treatment Diabetic Retinopathy Study (appETDRS) letter score, was calculated using recorded Snellen visual acuities. Results: A total of 92 eyes of 47 patients (31 females) with mean age of 48.0 ± 18.0 years and mean follow-up of 78.6 ± 42.2 months were included. At the initial visit, 12 eyes (13%) were classified as Stage I AAK, 33 eyes (35.9%) were Stage II, 25 eyes (27.2%) were Stage III, 17 eyes (18.5%) were Stage IV, and 5 eyes (5.4%) were Stage V. Limbal stem cell transplantation (LSCT) and Boston keratoprosthesis (KPro) were frequently performed in eyes with Stages III-V. These advanced corneal surgeries significantly improved the median (95% confidence interval [CI]) of calculated appETDRS scores from 2 (0-20) to 26 (15-41) (Snellen values, 20/20,000 to 20/300; P = 0.0004). Patients with earlier Stages (I-II) of AAK were managed medically and had stable visual acuity through their final visits (appETDRS score of 26 [20-35] to 35 [26-35]; Snellen, 20/300 to 20/200; P > 0.05). The appETDRS VAE was significantly improved from 20 (0-35) to 30 (20-55), Snellen, 20/400 to 20/250, following LSCT (P = 0.021) and from 2 (0-20) to 2 (0-41) after KPro; Snellen, 20/20,000 VAE but with improved 95% CI after follow-up (P = 0.019). Conclusions: With proper characterization and staging of AAK, individualized medical and advanced surgical interventions preserves and improves visual acuity.
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U2 - 10.1016/j.ajo.2019.11.003
DO - 10.1016/j.ajo.2019.11.003
M3 - Article
C2 - 31730836
AN - SCOPUS:85076852505
VL - 210
SP - 8
EP - 18
JO - American Journal of Ophthalmology
JF - American Journal of Ophthalmology
SN - 0002-9394
ER -