Faculty Bibliography

PURPOSE: To determine vision-related quality of life (QoL) measured with the National Eye Institute Visual Function Questionnaire (NEI-VFQ) in keratoconus (KCN) patients who have undergone penetrating keratoplasty (PK) in 1 or both eyes and to compare the results of our study to those of historical controls. DESIGN: Clinical-based, cross-sectional study. METHODS: SETTING: Wills Eye Institute, Cornea Service, Thomas Jefferson University, Philadelphia, Pennsylvania. STUDY POPULATION: This study included 149 consecutive patients who had undergone PK for KCN. INTERVENTION: Between June 1, 2008 and December 31, 2008, the NEI-VFQ was administered to 149 patients. The relationship between demographic and clinical factors and NEI-VFQ subscale scores was evaluated. MAIN OUTCOME MEASURE: Vision-related quality of life. RESULTS: Eighty-three of 149 patients (55.7%) were male. Approximately half of the patients (76/149; 51.0%) had PK in both eyes. Visual acuity with current correction in the better eye was better than 20/40 in 80% of patients (119/149). Our sample had significantly lower (worse) NEI-VFQ scores compared to Collaborative Longitudinal Evaluation of Keratoconus (CLEK) historical control group for the subscales of role difficulties, dependency, driving, and peripheral vision. In general, scores of our sample were between scores of patients with age-related macular degeneration (AMD) category 3 and 4. Patients with visual acuity better than 20/40 (in the better eye) showed significantly higher scores in all subscales except color vision. There was a significant relationship between minimum time since the graft of 5 years or greater and NEI-VFQ overall score better than AMD category 3 (P = .004). CONCLUSION: Despite satisfactory results on visual outcome measures obtained after PK, vision-related QoL in KCN patients remains impaired

PURPOSE:: The purposes of this study were to report the indications, graft survival, risk factors for graft failure, and visual outcomes for third or greater penetrating keratoplasties (PKP). METHODS:: Six years of Wills Eye Institute Cornea Service charts (2000-2005) were retrospectively reviewed to identify all patients who had undergone three or more PKPs. Graft survival rates by initial diagnosis and risk factors for graft failure were analyzed. RESULTS:: Forty-five patients who had three or more PKPs were identified. The total number of grafts identified in these 45 patients was 152, including 45 third PKPs, 11 fourth, three fifth, two sixth, and one seventh. The most common indication for the initial PKP was pseudophakic bullous keratopathy in 18 of 45 patients (41%) followed by Fuchs dystrophy (seven of 45 patients [16%]) and stromal dystrophies (five of 45 patients [11%]). Approximately half of third grafts (24 of 45 [53%]) and one fourth of fourth grafts (three of 11 [27%]) survived at the last follow-up visit with a median follow up of 4.3 years for the third grafts and 8.4 years for the fourth grafts. One-, 2-, and 5-year graft survival rates were 89%, 78%, and 53% for the third grafts and 73%, 73%, and 64% for the fourth grafts, respectively. The median survival time for the third graft was 12.8 years in Fuchs dystrophy, 5.2 years in herpetic keratitis, 4.0 years in keratoconus, 3.0 years in pseudophakic bullous keratopathy, 2.3 years in iridocorneal endothelial syndrome, and 2.0 years in stromal dystrophies. There is no statistically significant difference between groups (P = 0.46). Risk factor analysis on the third grafts showed that previous glaucoma procedures and corneal neovascularization are statistically significant risk factors for graft failure (P = 0.04 and 0.02, respectively). CONCLUSION:: Over 50% of third and fourth grafts were clear at 5 years postoperatively. Outcomes of third grafts were better in patients with Fuchs dystrophy, keratoconus, and herpetic keratitis. Absence of previous glaucoma surgery and/or corneal neovascularization is associated with better outcomes of multiple PKPs

PURPOSE: This study was designed to determine the demographics of patients aged 50 years or more with a diagnosis of keratoconus, who were seen on the Cornea Service at Wills Eye Hospital. METHODS: A retrospective chart review of all patients with the diagnosis of keratoconus was undertaken to identify demographics characteristics between January 1, 2005 and December 31, 2005. RESULTS: A total of 697 patients were identified. Two hundred seventy-nine (40.0%) patients were age 50 years or more. The age ranged from 50 to 93 years (average 60.2 +/- 8.2 years). Keratoconus was diagnosed as a bilateral disease in 266 (95.3%) and as a unilateral disease in 13 patients (4.7%). In 186 patients (66.6% of the total), the average age at the time of diagnosis was 31.7 +/- 10.9 years (range from 13 to 70 years). Of the 279 patients, 167 had surgery (59.8%). In the group of 112 patients that did not have surgery, 25 (9.0%) were treated with glasses in both eyes, 85 (30.5%) with contact lenses in both eyes, and 2 (0.7%) with glasses in one eye and contact lenses in the other eye. The average time of follow-up was 13.6 +/- 10.5 years (range from 0.1 to 38.7 years). CONCLUSIONS: In a corneal referral practice, the number of patients older than 50 years with keratoconus is higher than previously reported in the literature. We believe that this is because our practice has a strong interest in both contact lenses and corneal surgery with the ability to follow up patients on a long-term basis

OBJECTIVE: To report the concomitant presentation of keratoconus and corneal dystrophies at Wills Eye Hospital for the 10-year period from January 1, 1997, to December 31, 2006. METHODS: Patients with concomitant keratoconus and corneal dystrophies were identified using a computer database. Complete ophthalmologic examination, keratometry, pachymetry, and computerized videokeratography were performed in all patients. When present, cornea guttata were confirmed by clinical examination and specular microscopy. Histopathologic examination with special stains of excised corneal buttons was performed. RESULTS: Fifty-one patients manifested typical signs and topographic evidence of keratoconus associated with another corneal dystrophy. Fuchs dystrophy was the most common association accounting for 27 cases (52.9%), followed by anterior basement membrane dystrophy with 13 cases (25.5%) and posterior polymorphous dystrophy with 7 cases (13.8%). A bilateral combination of Fuchs dystrophy and anterior basement membrane dystrophy with keratoconus was seen in 3 cases (5.8%). Finally, there was 1 bilateral case (2%) of granular dystrophy. Histopathologic studies in cases that underwent penetrating keratoplasty confirmed the clinical diagnoses. CONCLUSION: To our knowledge, this is the largest report of such a concurrence in the English literature and could lead to further studies on the possible pathophysiologic or genetic links between these entities, although a chance association cannot be excluded

OBJECTIVES: To assess the impact of the different types of contact lenses (CLs) on quality of life (QoL) in patients with keratoconus based on self-reported results from The Contact Lens Impact on Quality of Life (CLIQ) Questionnaire. METHODS: Consecutive keratoconus patients who wore CLs (rigid gas permeable [RGP], hybrid or soft toric) at least in one eye were asked to complete the CLIQ questionnaire on the Cornea Service, Wills Eye Institute. RESULTS: A total of 71 patients with a mean age of 42.6 +/- 13.1 year were included in the study. One eye of each patient was included in the study. Of these, 40 eyes used rigid gas-permeable lenses, 20 eyes used hybrid lenses and 11 eyes used soft toric lenses. The mean CLIQperson measure was 45.5 +/- 8.2 in RGP group, 45.4 +/- 7.5 in hybrid group and 48.4 +/- 10.5 in soft toric group. There was no significant difference among the three groups in self-reported results from the CLIQ questionnaire (P = 0.8). CONCLUSIONS: Subjects with keratoconus who wear RGP, hybrid or soft toric CLs, reported similar contact lens impact on their QoL

PURPOSE: To evaluate the outcomes of the Boston Keratoprosthesis (KPro) at our institution. METHODS: A computerized database retrospectively identified all patients who received a Boston KPro from August 2005 to October 2007. RESULTS: There were 37 patients, 24 (65%) were male. Mean age was 66.3 years (median 69 years, range: 24-93 years; SD = 18.1 years). Mean follow-up was 16 months (range: 6-28 months; SD = 6.0 months). There were 36 type 1 (97%) and 1 type 2 (3%) KPros. The primary indication was failed penetrating keratoplasty in 29 patients (78%)-22 (59%) from multiple failures. Preoperative comorbidities included glaucoma [27 patients (73%)] and limbal stem cell deficiency [13 patients (35%)]. No intraoperative complications occurred. Postoperative complications included retroprosthetic membrane [24 patients (65%)], increased intraocular pressure [14 patients (38%)], glaucoma progression [5 patients (13.5%)], and endophthalmitis [4 patients (11%)-3 who discontinued prophylactic antibiotics secondary to compliance]. Thirty-six KPros (97%) were retained-1 type 2 KPro (3%) in a patient with ocular cicatricial pemphigoid extruded and was replaced. Mean best-corrected visual acuities were counting fingers preoperatively (range: light perception to 20/100) and 20/90 at last follow-up (range: light perception to 20/25). At last follow-up, 31 patients (84%) improved 2 lines or greater-3 patients (8%) had worse vision. CONCLUSIONS: Retention rates were excellent, and best-corrected visual acuities improved in the vast majority of patients. Complications can occur and require frequent follow-up to monitor and treat glaucoma progression, endophthalmitis, and inflammation. Patient compliance is of paramount importance. Despite the potential complications, the Boston KPro provides visual improvement in patients with an otherwise poor prognosis

PURPOSE: The aim of the present study was to evaluate the frequency of steroid-induced intraocular pressure (IOP) elevation and/or glaucoma in patients with keratoconus (KCN) compared with patients with Fuchs endothelial dystrophy after penetrating keratoplasty (PK). METHODS: A retrospective review of the medical records of patients with KCN or Fuchs dystrophy, who underwent PK and were examined on the Cornea Service, Wills Eye Institute, was performed. IOP measurements were recorded preoperatively; postoperative first month and maximal IOP measurements between 1 and 3 months, 4 and 6 months, 7 and 12 months, 1 and 2 years, 2 and 3 years, and 3 and 4 years were noted. Steroid-induced IOP elevation and/or glaucoma were grouped into 5 different categories; an increase in IOP of at least 5 or 10 mm Hg over the preoperative baseline and also IOP > or =22, 30, and 40 mm Hg. Addition of glaucoma medications and/or characteristic glaucomatous optic disc and visual field changes were also assessed. RESULTS: A total of 100 patients with KCN and 58 patients with Fuchs dystrophy were included in this study. The overall frequency of steroid-induced IOP elevation after PK was 73% in the KCN group and 60.3% in the Fuchs dystrophy group. The frequency of IOP elevation of at least 5 or 10 mm Hg over the preoperative baseline were 72% and 24% in KCN group and 56.9% and 20.7% in the Fuchs dystrophy group, respectively. The frequency of IOP elevation > or =22 or > or =30 mm Hg was 22% and 6% in the KCN group and 29.3% and 1.7% in the Fuchs dystrophy group, respectively. There was one patient in the KCN group who had IOP >40 mm Hg. There was no difference between the groups in terms of frequency of IOP elevation (P > 0.05 for all). Glaucomatous visual field defect was detected in 4 patients in the KCN group and only one patient in the Fuchs dystrophy group. Despite the maximum medical therapy, 2 patients in the KCN group underwent glaucoma surgery and none in the Fuchs dystrophy group. CONCLUSION: Steroid-induced IOP elevation or glaucoma after PK is not unusual in eyes with KCN or Fuchs dystrophy. Careful and ongoing observation of IOP throughout the prolonged follow-up period is recommended for these individuals with prompt attention to IOP treatment as indicated

PURPOSE: To compare the incidence and characteristics of graft rejection in keratoconus patients with and without self-reported atopy. METHODS: From November 2007 to February 2008, 75 consecutive keratoconus patients who had undergone penetrating keratoplasty completed a questionnaire regarding personal history of atopy. The questionnaires were reviewed without knowledge of history of graft rejection. The patients were divided into three groups: nonatopic (group 1), mild atopic (group 2), and severe atopic (group 3). Charts were retrospectively reviewed with regard to the incidence and characteristics of graft rejection episodes for each patient. RESULTS: There were 13 patients in group 1, 36 patients in group 2, and 26 patients in group 3. The incidence of graft rejection (rejection episodes per 100 person-years) in groups 1, 2, and 3 was 7.3, 15.8, and 13.4, respectively. Although the mild and severe atopic groups had roughly twice the rate of rejection of the nonatopic group, there was no statistically significant difference among the groups (P = 0.25). The mean age of 37.1 +/- 8.9 years at the time of surgery was significantly lower (P = 0.004) and the rate of bilateral penetrating keratoplasty was significantly higher (40/55 eyes 73%) (P = 0.01) in group 2 than in the two other groups. The majority of first graft rejection episodes were mild or moderate in atopic patients (69% in group 2 and 94% in group 3) and severe (50%) in nonatopic patients; however, no significant difference was found among the groups (P = 0.13). CONCLUSIONS: Atopic conditions may increase the incidence of graft rejection in keratoconus patients, and the characteristics of graft rejection in atopic patients may differ from those in nonatopics because of the immune alteration, but differences in this study were not statistically significant