1. Clinical and Visual Outcomes of Children With Peters Anomaly Asim Ali, MD, FRCSC Uri Elbaz, MD Hermina Strungaru, MD, PhD Kamiar Mireskandari, MBChB, FRCSEd, FRCOphth, PhD. Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada The authors have no financial interest to disclose

2.  The purpose of this study is to investigate the visual and clinical outcomes in a large Peters anomaly cohort stratified by disease severity and selective treatment modalities in order to improve management of this disease.  Our study is the first to include the visual and clinical outcomes of different treatment modalities for both traditional subtypes of the disease.

3.  The charts of all patients diagnosed with Peters anomaly from January 2000 to December 2012 were reviewed retrospectively.  For treatment purposes, patients were classified as Peters I or II, depending on lens involvement ( please refer also to e-poster 17590 “ Characterizing the Phenotypic Spectrum of Peters Anomaly: From Mild to Severe Disease ” ). Characterizing the Phenotypic Spectrum of Peters Anomaly: From Mild to Severe Disease  Patients were further classified depending on corneal opacity location and size.

4.  Visual and clinical data were collected and stratified per treatment modality chosen.  Visual outcomes included final best corrected distance visual acuity (CDVA) and the presence of nystagmus.  Clinical outcomes included incidence of glaucoma and PKP survival in transplanted eyes.

5.  Sixty eyes of 40 patients were included in the study.  The mean patient age at presentation was 6.2±20.7 months (0.0-111.0 months) with a mean follow-up time of 75.8±52.9 months (12.1-225.3 months).

6. ≥ 20/200 n (%) <20/200-CF n (%) CF or HM n (%) Mean CDVA logMAR ± SD p-ValueLP or NLP n (%)p-Value Overall (n=59*)33 (55.9)9 (15.3)4 (6.8) 0.9±0.7 13 (22.0) Peters I (n=42)27 (64.3)8 (19.0)2 (4.8) 0.8±0.6 0.2 5 (11.9) 0.003 Peters II (n=17)6 (31.6)1 (5.3)2 (10.5) 1.2±1.1 8 (42.1) Unilateral (n=20)10 (50.0)3 (15.0) 1.1±1.0 0.16 4 (20.0) 0.78 Bilateral (n=39)23 (59.0)6 (15.4)1 (2.6) 0.8±0.5 9 (23.1) Mild Peters I (n=7)7 (100.0)0 (0.0) 0.4±0.3 0 (0.0) Moderate Peters I (n=9)8 (88.9)0 (0.0)1 (11.1) 0.7±0.7 0 (0.0) Severe Peters I (n=25)12 (48.0)8 (32.0)1 (4.0) 1.0±0.6 4 (16.0) Small opacity Type II (n=4)2 (50.0)1 (25.0)0 (0.0) 0.9±0.5 1 (25.0) Large opacity Type II (n=11)4 (36.4)0 (0.0)2 (18.2) 1.4±1.3 5 (45.5) Eyes with glaucoma (n=19)5 (26.3)7 (36.8)3 (15.8) 1.5±0.9 0.00004 4 (21.1) 0.9 Eyes without glaucoma (n=40)28 (70.0)2 (5.0)1 (2.5) 0.6±0.5 9 (22.5) Glaucoma Peters I (n=13)2 (15.4)6 (46.2)1 (7.7) 1.5±0.7 0.0002 4 (30.8) 0.01 No glaucoma Peters I (n=29)25 (86.2)2 (6.8)1 (3.4) 0.6±0.5 1 (3.4) Glaucoma Peters II (n=6)3 (50.0)1 (16.7)2 (33.3) 1.5±1.2 0.17 0 (0.0) 0.004 No glaucoma Peters II (n=11)3 (27.3)0 (0.0) 0.5±0.28 (72.7) Final visual acuity stratified by disease severity and in relation to glaucoma. *In one Peters II anomaly eye visual acuity was not obtained due to severe developmental delay. CDVA – corrected distance visual acuity

7. ≥ 20/200 n (%) <20/200-CF n (%) CF or HM n (%) Mean CDVA logMAR ± SD p-Value LP or NLP n (%) p-Value Nystagmus n (%) Obseravation (n=7)7 (100.0)0 (0.0) 0.4±0.3 0 (0.0) 2 (28.6) Optical management (n=13)10 (76.9)1 (7.7) 0.7±0.6 1 (7.7) 4 (30.8) Peters I (n=9)8 (88.9)0 (0.0)1 (11.1)0.7±0.7 0.8 0 (0.0) 0.12 2 (22.2) Peters II (n=4)2 (50.0)1 (25.0)0 (0.0)0.9±0.51 (25.0) 2 (50.0) PKP (n=36)16 (44.4)8 (22.2)3 (8.3)1.1±0.8 9 (25.0) 30 (81.1) Peters I (n=25)12 (48.0)8 (32.0)1 (4.0)1.0±0.6 0.4 4 (16.0) 0.06 18 (72) Peters II (n=11*)4 (36.4)0 (0.0)2 (18.2)1.4±1.35 (45.5) 12 (100) No treatment (n=3)0 (0.0) NA 3 (100.0) 2 (66.7) Final visual acuity and nystagmus prevalence stratified by treatment modality Causes of graft failure in 27 transplants of 12 eyes (12 primary and 15 repeat transplants) CauseNo of transplants (%) Rejection 7 (25.9) Limbal stem cell deficiency 5 (18.5) Corneal ulcer1 (4.0) Uncontrolled glaucoma 4 (14.8) Silicone oil2 (7.4) Retrocorneal membrane 4 (14.8) Unknown 4 (14.8) *In one Peters II anomaly eye in the PKP group visual acuity was not obtained due to severe developmental delay. Nystagmus data was available in all eyes. CDVA – corrected distance visual acuity. NA – not applicable.

8. Risk factorHazarad ratio95% CIp - Value Laterality0.930.26 – 3.380.91 Iridocorneal adhesion1.450.18 - 11.600.73 Keratolenticular adhesion2.910.81 – 10.400.13 Vascularization3.220.92 – 11.340.07 Microphthalmos3.691.06 – 12.820.04 Donor size (>6mm)2.440.08 – 1.330.19 Donor age (> 40 years)1.320.26 – 4.930.68 Glaucoma3.180.82 – 12.290.09 Glaucoma surgery after 1 st PKP1.810.53 – 6.210.35 Lens surgery5.62 1.21 – 26.160.03 lens surgery along with1 st PKP1.05 0.13 – 8.540.97 Lens surgery after 1 st PKP 4.771.26 – 18.070.02 Peters Type I0.27 0.08 – 0.90 0.03 Univariate analysis of preoperative and intraoperative variables affecting primary PKP survival. Risk factorOdds ratio95% CIp - Value Laterality0.82 0.24 - 2.84 0.75 Iridocorneal adhesion0.510.09 - 2.920.45 Keratolenticular adhesion0.330.09 - 1.170.09 Posterior corneal defect1.440.31 - 6.600.64 Vascularization 0.290.10 – 0.810.02 Microphthalmos 0.190.05 – 0.680.01 Glaucoma0.23 0.08 – 0.670.007 Glaucoma surgery 0.160.04 – 0.67 0.01 Lens surgery 0.270.08 – 0.89 0.03 PKP 0.45 0.13 – 1.54 0.21 Optical iridectomy 0.980.17 – 5.710.98 Optical management 2.530.60 – 10.72 0.21 Peters type I 3.281.0 - 10.20.04 Univariate analysis of preoperative and intraoperative variables that associated with final visual acuity of 20/200 or better.

9. Kaplan Meier survival curves showing overall PKP survival probability (A), PKP survival probability stratified per disease type (B), and by the presence of glaucoma (C). A B C

10. Peters anomaly Severe disease Moderate disease Mild disease Observe n=7 Optical management n=9 PKP n=25 Peters type I Weigh risk against benefits. Poor prognosis n=3 (1 Peters I & 2 Peters II eyes) Peters type II Keratolenticular touch and/or cataract Aphakia/lens remnant Associated severe systemic/ocular abnormalities Large axial opacity (>3mm) Small axial opacity (≤3mm) PKP n=12 (n=4) Optical management Peripheral opacity Paraxial opacity Small axial opacity (≤3mm) Large axial opacity (>3mm) ± Viscodissection ± Cataract extraction n=3  0.9 ± 0.5 n=1  LP 0.4 ± 0.3 0.7 ± 0.7 n=21  1.0 ± 0.6 n=2  LP n=2  NLP n=6  1.4 ± 1.3 n=2  LP n=3  NLP n=3  LP Vision is expressed as mean ± standard deviation (logMAR units).

11.  In accordance to previous studies, our study shows that mild disease has better visual outcomes than more severe disease and that there is no difference in the visual outcomes between unilateral and bilateral disease.  Our study also shows that in eyes that have undergone PKP, development of glaucoma is a major hurdle for graft survival and for good visual outcome.  Despite being considered the severe form of the disease, patients with Peters type II can still attain good visual outcome especially when optical management is feasible.  In our series, all patients who received optical management in both peters I and II groups had CDVA of 1.3 logMAR (20/400) or better with 8 patients having 0.5 logMAR (20/70) vision or better.

12.  Visual rehabilitation in Peters anomaly remains a great challenge.  Cases necessitating observation or minor surgical intervention achieve good vision.  When PKP is indicated poorer outcomes are obtained, nevertheless patients can still attain functional visual acuity with encouraging graft longevity.