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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

Clinical and Visual Outcomes of Children With Peters Anomaly Asim Ali, MD, FRCSC Uri Elbaz, MD Hermina Strungaru, MD, PhD Kamiar Mireskandari, MBChB, FRCSEd,

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Clinical and Visual Outcomes of Children With Peters AnomalyAsim Ali, MD, FRCSCUri Elbaz, MD Hermina Strungaru, MD, PhDKamiar Mireskandari, MBChB, FRCSEd, FRCOphth, PhD.

Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, CanadaThe authors have no financial interest to disclose Purpose 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.

METHODS 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).

Patients were further classified depending on corneal opacity location and size.

METHODS 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.

RESULTSSixty eyes of 40 patients were included in the study.

The mean patient age at presentation was 6.220.7 months (0.0-111.0 months) with a mean follow-up time of 75.852.9 months (12.1-225.3 months).

20/200n (%) 40 years)1.320.26 4.930.68Glaucoma3.180.82 12.290.09Glaucoma surgery after 1st PKP 1.810.53 6.210.35Lens surgery 5.621.21 26.160.03lens surgery along with1st PKP 1.050.13 8.540.97Lens surgery after 1st PKP 4.771.26 18.070.02Peters Type I0.270.08 0.900.03Univariate analysis of preoperative and intraoperative variablesaffecting primary PKP survival. Risk factorOdds ratio95% CIp - Value Laterality0.820.24 - 2.840.75Iridocorneal adhesion0.510.09 - 2.92 0.45Keratolenticular adhesion0.330.09 - 1.17 0.09Posterior corneal defect 1.440.31 - 6.60 0.64Vascularization0.290.10 0.810.02Microphthalmos0.190.05 0.680.01Glaucoma0.230.08 0.670.007Glaucoma surgery 0.160.04 0.670.01Lens surgery 0.270.08 0.890.03PKP 0.450.13 1.540.21Optical iridectomy0.980.17 5.710.98Optical management 2.530.60 10.720.21Peters type I3.281.0 - 10.20.04Univariate analysis of preoperative and intraoperative variables that associated with final visual acuity of 20/200 or better.

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). ABCPeters anomaly Severe disease Moderate diseaseMild disease Observen=7 Optical managementn=9 PKPn=25Peters type IWeigh risk against benefits. Poor prognosisn=3 (1 Peters I & 2 Peters II eyes) Peters type IIKeratolenticular touch and/or cataract Aphakia/lens remnant Associated severe systemic/ocular abnormalitiesLarge 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 0.30.7 0.7n=21 1.0 0.6 n=2LPn=2NLPn=6 1.4 1.3n=2LP n=3 NLPn=3LPVision is expressed as mean standard deviation (logMAR units).10DISCUSSION 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.CONCLUSION 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.