Elvin H. Yildiz, Elisabeth J. Cohen, Ajoy S. Virdi, Kristin M. Hammersmith, Peter R Laibson, and Christopher J. Rapuano Cornea Service, Wills Eye Institute, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA. The authors have no financial interest in the subject matter of this poster Elvin H. Yildiz, Elisabeth J. Cohen, Ajoy S. Virdi, Kristin M. Hammersmith, Peter R Laibson, and Christopher J. Rapuano Cornea Service, Wills Eye Institute, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA. The authors have no financial interest in the subject matter of this poster

Background Keratoconus (KCN) is a progressive, asymmetric, noninflammatory condition of the cornea associated with corneal steeping and apical thinning that leads to decrease in vision secondary to progressive irregular astigmatism and scarring. - KCN mainly affects young adults. - A mean age of 39.2 years at the time of enrollment was reported in the large series of the Collaborative Longitudinal Evaluation of Keratoconus (CLEK) study patients. 1 A significant impairment in vision -related quality of life (QoL), with average scores comparable to category 3 and category 4 age-related macular degeneration (AMD) has been shown by CLEK study group % of these patients had a best-corrected visual acuity of 20/40 or better in both eyes Penetrating keratoplasty (PK) is a well-accepted treatment for advanced KCN. - Overall, 12-20% patients with KCN eventually require PK at a relatively young age. - The long term success rate of PK for KCN has been well described in terms of the visual acuity and graft clarity, with a 25-year graft survival rate of 90% and 20/40 or better vision in 66% to 91% patients followed more than 15 years. 2,3

Purpose To determine vision-related quality of life (QoL) measured with the National Eye Institute Visual Function Questionnaire (NEI-VFQ) in KCN patients who have undergone PK in one or both eyes. To compare the QoL in KCN patients after PK to those without PK as reported in the CLEK study.

Materials and Methods Design : Clinical-based, cross sectional studyIntervention: Between July1, 2008 and December 31, 2008, the NEI-VFQ was administered to 149 consecutive patients who had undergone PK for KCN. The relationship between demographic and clinical factors and NEI-VFQ subscale scores were evaluated

Results: Baseline Demographics 149 consecutive KCN patients *The mean age at the time of enrollment: 53.9±13.5 years (range from 24 to 87) *Slightly more males in the sample (83/149, 55.7%) *The majority of patients (122/149, 81.9%) were Caucasian *Half of the patients (76/149, 51.0%) had PKs in both eyes. *80% of patients (119/149) had a VA better than 20/40 in the better eye *60% (90/149) had a VA 20/25 or better in the better eye. * 83.2% (124/149) of the better eyes had undergone PK *Mean time since PK: 15.6±10.1 yrs (1 to 54) in the better eye (n=120) 13.9±9.9 yrs (1 to 52) in the worse eye (n=105)

Results: Comparison of NEI-VFQ Scale Scores with Historical Controls Our sample has significantly lower (worse) scores compared to the cross sectional CLEK QoL study 1 for the subscales of role difficulties, dependency, driving, and peripheral vision. Our sample group had lower (worse) scores compared to AMD category 3 scores for all NEI-VFQ scores, except color vision. When compared to cut-off scores for AMD category 4, the current sample generally showed higher (better) scores for all subscales except ocular pain (where our sample group had significantly worse scores), and peripheral vision (no significant difference) (Table 1).

Table 1: Comparison of NEI-VFQ scale scores in keratoconus patients who had undergone penetrating keratoplasty with those in historical controls Current studyCLEK study 1 ARED study 4 AMD Category 3 ARED study AMD Category 4 Scales Mean SDMeanSDP value MeanP value MeanP value General Health <.00166<.001 General vision <.001 Ocular Pain <.00187<.001 Near Activities <.00166<.001 Distance Activities < Social Function <.00185<.001 Mental Health < Role Difficulties < Dependency <.00183<.001 Driving <.00191<.00152<.001 Color vision <.001 Peripheral vision <

Results: Differences in NEI-VFQ subscale Scores by Demographics and Clinical Variables There were differences on a number of subscales by gender. In general, males had significantly higher (better) scores compared to females, including scores for general health, ocular pain, distance activities, mental health, role difficulties, dependency, driving, peripheral vision and overall score. (Table 2) There were no statistically significant differences in the mean subscale scores between different age groups and between patients with bilateral or unilateral PKs. Patients with visual acuity (in the better eye) that was better than 20/40 showed significantly higher scores in all subscales except for color vision, compared to patients with visual acuity of 20/40 or worse. Similar results were found for 20/40 visual acuity in the worse eye, although effect sizes were generally smaller. (Table 2)

Results: Differences in NEI-VFQ subscale Scores by Demographics and Clinical Variables (Cont.) Patients with visual acuity (in the better eye) that was 20/25 or better showed significantly higher overall and subscale scores except for ocular pain, mental health, and color vision, compared to patients with vision worse than 20/25. (Table 2) There were significant differences for near activities, distance activities, social function, mental health, role difficulties, dependency, peripheral vision, and overall score when stratified by minimum graft age ≥5 years versus < 5 years, with better scores with minimum graft age ≥5 years. When using a cutoff of 10 years, significant effects were found only for distance activities and peripheral vision. A cut-off of 15 years yielded significant differences only for distance activities and driving (better scores with minimum graft age <15 years ). (Table 3) Use of RGP lenses in either eye showed higher (better) scores on general health, general vision, and distance activities, and use of RGP lens in an eye with PK showed higher (better) scores on measures on general vision, distance activities, social function, dependency, peripheral vision, and overall score.

Table 2: Differences in NEI-VFQ subscale Scores by Demographics and Clinical Variables Gender Better eye visual acuity Male (n=83)Fem. (n=66)20/40 cut- off point20/25 cut-off point Scales MeanSDMeanSD >20/40 n=119 ≤20/40 n=30 ≥20/25 n=90 <20/25 n=59 General Health General vision Ocular Pain Near Activities Distance Activities Social Function Mental Health Role Difficulties Dependency Driving Color vision Peripheral vision

Table 3: Differences in NEI-VFQ subscale Scores by Demographics and Clinical Variables Minimum time since the graft of 5 year Minimum time since the graft of 10 year Minimum time since the graft of 15 year Scales ≥5yrs n=110 <5yrs n=39 ≥10yrs n=80 <10yrs n=69 ≥15yrs n=54 <15yrs n=95 General Health General vision Ocular Pain Near Activities Distance Activities Social Function Mental Health Role Difficulties Dependency Driving Color vision Peripheral vision

Conclusion Despite very good to excellent results on visual outcome measures obtained after PK for KCN, patients’ assessment of their vision related QoL remains impaired. Between 5-15 years after PK and the use of RGP contact lenses in PK eye are associated with better vision related QoL. The results of the CLEK study raised the question as to whether patients’ vision-related QoL would be better after PK, but these results do not support this. Surgery remains indicated for contact lens failures.

References 1)Kymes SM, Walline JJ, Zadnik K et al. Quality of life in keratoconus. Am J Ophthalmol 2004;138: ) Pramanik S, Musch DC, Sutphin JE, Farjo AA. Extended long-term outcomes of penetrating keratoplasty for keratoconus. Ophthalmology 2006;113: ) Zadok D, Schwarts S, Marcovich A et al. Penetrating keratoplasty for keratoconus: Long-term results. Cornea 2005;24: ) Clemons TE, Chew EY, Bressler SB et al. National eye institute visual function questionnaire in the age-related eye disease study (AREDS). AREDS report No. 10. Arch Ophthalmol 2003;121:211-7.